Article

Polyps and Masses of the Pediatric Urinary Bladder: A 21-Year Pathology Review

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Abstract

Although not uncommon in adults, bladder tumors are rare in children. In addition, the histologic types of tumors seen in the pediatric population differ from those seen in adults. Although rhabdomyosarcoma is the most common pediatric bladder tumor, many other benign, malignant, and reactive lesions can be encountered. All may present clinically as a mass or polyp in the bladder. This study was designed to describe the pathology and patient demographics of pediatric bladder masses, because there are few studies describing these entities. Retrospectively reviewing our experience over a 21-year period, we identified 98 specimens from 65 patients with polyps or masses in the urinary bladder. As expected, the most frequent diagnosis was rhabdomyosarcoma. This was followed by fibroepithelial polyp and a variety of additional nonurothelial tumors. Only 7 urothelial tumors were identified, including 1 low-grade papillary urothelial carcinoma. Inflammatory lesions, such as cystitis cystica and nephrogenic adenoma, were invariably associated with an irritating factor when a history was provided. Our findings emphasize that diagnoses made in the pediatric urinary bladder are distinct from those in adults, although a wide variety of lesions may still be seen.

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... In the newborn period, bladder masses are extremely rare, including rhabdomyosarcomas (RMS), 2-4 inflammatory pseudotumor of the bladder, 1 hemangioma, 5 nonspecific/reactive lesion, 6 and polypoid cystitis/inflammatory pseudopolyp. 6 The neonatal presentation of RMS has an incidence of approximately 4 in 1,000,000 live births. ...
... In the newborn period, bladder masses are extremely rare, including rhabdomyosarcomas (RMS), 2-4 inflammatory pseudotumor of the bladder, 1 hemangioma, 5 nonspecific/reactive lesion, 6 and polypoid cystitis/inflammatory pseudopolyp. 6 The neonatal presentation of RMS has an incidence of approximately 4 in 1,000,000 live births. 7 The radiologic features of bladder RMS are not specific and benign mural masses of the bladder are difficult to differentiate from RMS. 4 A precise diagnosis can demand additional clinical and pathologic examination. ...
... The newborn case reported here had a polypoid cystitis, only the second described case in English literature in neonatal age. 6 It was not sonographically or cystoscopically distinguishable from other localized lesions. All reported neonatal cases of bladder mass are summarized in Table 1. ...
Article
Benign proliferative lesions of the bladder are exceptional in neonates. We describe a case of a 3 day-old neonate, presenting with bloody meconium and sonographic diagnosis of bladder mass. Cystoscopic biopsies were performed and a diagnosis of polypoid cystitis was made. The patient was treated conservatively and the lesion healed during follow-up. We review the literature of other cases of neonatal bladder masses.
... The histologic types of tumors seen in the pediatric population differ from those seen in adults. Nonepithelial tumors are more common than epithelial ones and, although rhabdomyosarcoma is the most common pediatric bladder tumor, many other benign, malignant, and reactive lesions can be encountered [1,2]. ...
... Neurofibromatosis type 1 (NF1) is a common autosomal dominant syndrome affecting 1/3,500 individuals worldwide [1]. Clinical features of the disease are pigmentary anomalies, skinfold freckling and Lisch nodules [2], learning disabilities, orthopedic problems, and benign and malignant tumors [3,4]. NF1 patients develop neural tumors more frequently than normal population. ...
... PUNLMP usually affects adults older than 20 years and is extremely rare in children younger than 10 years. A case of a 9-year-old child with bladder PUNLMP has been reported by Gao and coworkers, while Stanton and collaborators found one PULNMP and two LGPUC, low-grade papillary urothelial carcinoma [102,103] From 65 children with polyps or masses in the urinary bladder only 7 urothelial tumors were identified, including 1 lowgrade papillary urothelial carcinoma, by Huppmann and collaborators [2]. Nonetheless, in the Surveillance, Epidemiology and End Results (SEER) database (1973 to 2003), of 140 bladder tumours affecting patients younger than 18 years old, 50.7% were diagnosed as PUNLMP. ...
Article
Full-text available
Bladder masses are represented by either benign or malignant entities. Malignant bladder tumors are frequent causes of disease and death in western countries. However, in children they are less common. Additionally, different features are found in childhood, in which non epithelial tumors are more common than epithelial ones. Rhabdomyosarcoma is the most common pediatric bladder tumor, but many other types of lesions may be found, such as malignant rhabdoid tumor (MRT), inflammatory myofibroblastic tumor and neuroblastoma. Other rarer tumors described in literature include urothelial carcinoma and other epithelial neoplasms. Rhabdomyosarcoma is associated to a variety of genetic syndromes and many genes are involved in tumor development. PAX3-FKHR and PAX7-FKHR (P-F) fusion state has important implications in the pathogenesis and biology of RMS, and different genes alterations are involved in the pathogenesis of P-F negative and embryonal RMS, which are the subsets of tumors most frequently affecting the bladder. These genes include p53, MEF2, MYOG, Ptch1, Gli1, Gli3, Myf5, MyoD1, NF1, NRAS, KRAS, HRAS, FGFR4, PIK3CA, CTNNB1, FBXW7, IGF1R, PDGFRA, ERBB2/4, MET, BCOR. Malignant rhabdoid tumor (MRT) usually shows SMARCB1/INI1 alterations. Anaplastic lymphoma kinase (ALK) gene translocations are the most frequently associated alterations in inflammatory myofibroblastic tumor (IMT). Few genes alterations in urothelial neoplasms have been reported in the paediatric population, which are mainly related to deletion of p16/lnk4, overexpression of CK20 and overexpression of p53. Here, we reviewed available literature to identify genes associated to bladder malignancies in children and discussed their possible relationships with these tumors.
... Pediatric neoplasms in the urinary system are rare due to the relatively low incidence of epithelial tumors. Childhood bladder hemangiomas, among them, are seldom reported due to their extreme rarity [2]. In the present case, we describe multiple urinary bladder hemangiomas as a cause of pediatric gross hematuria focused on ultrasonographic findings. ...
... Leiomyoma arises from the submucosal or muscular layer of the bladder and presents as a well-marginated homogenous solid mass on ultrasonography [10]. Bladder involvement of neurofibromatosis is rare and usually manifests as a generalized neurofibromatosis rather than an isolated form [2]. It usually shows diffuse and irregular wall thickening with or without nodules. ...
... Pseudotumoral cystitis, an uncommon form of cystitis, presents with hypoechoic or hyperechoic mass-like elevations of the mucosal surface, mimicking a bladder neoplasm on ultrasonography. Although these various benign and malignant tumors may appear as intraluminal polypoid bladder masses on imaging studies [2], hemangioma should be considered in children when the mass has a benign-looking appearance such as a well-defined margin and no extravesical extension, while showing vascularity despite its small size and multiplicity. ...
Article
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We report a case of multiple hemangiomas involving the urinary bladder in a 4-year-old boy who presented with recurrent episodes of gross hematuria. On ultrasonography, compared with the bladder wall, the lesions presented as multiple isoechoic polypoid intraluminal masses with mildly increased vascularity on color Doppler exam. Cavernous hemangioma was confirmed by cold-cup biopsy, and the all lesions were coagulated with a Holmium laser. Despite their rarity, bladder hemangiomas should be included in the differential diagnosis of multiple intravesical masses in children with gross hematuria.
... The most common pediatric bladder tumor is rhabdomyosarcoma, followed by fibroepithelial polyps. 1 Bladder hemangiomas, however, are exceedingly rare in both adults and children, and therefore relatively few reports of them exist in the literature. 1,2 Although there is a known association with cutaneous vascular lesions and vascular malformation syndromes (namely, Sturge-Weber syndrome and Klippel-Trenaunay-Weber syndrome), bladder hemangiomas may also occur in the absence of these conditions. ...
... The most common pediatric bladder tumor is rhabdomyosarcoma, followed by fibroepithelial polyps. 1 Bladder hemangiomas, however, are exceedingly rare in both adults and children, and therefore relatively few reports of them exist in the literature. 1,2 Although there is a known association with cutaneous vascular lesions and vascular malformation syndromes (namely, Sturge-Weber syndrome and Klippel-Trenaunay-Weber syndrome), bladder hemangiomas may also occur in the absence of these conditions. 3 Here, we present a case of histologically proven hemangioma of the urinary bladder in an otherwise healthy 2-year-old Caucasian female. ...
Article
Full-text available
We report a case of a bladder hemangioma in a pediatric patient. A 2-year-old Caucasian female presented with intermittent gross hematuria and protrusion of beefy red tissue near the vaginal introitus when straining. On cystoscopy, we discovered a wide-based vermiform mass. Transurethral resection of the bladder mass was performed. Based on the histological findings of the tissue resected, a diagnosis of capillary hemangioma of the bladder was made. Despite their rarity, bladder hemangiomas should be considered in the differential in children with gross hematuria.
... Rhabdomyosarcoma is the most common pediatric bladder tumor. [1] Other lesions such as malignant rhabdoid tumor, inflammatory myofibroblastic tumor, and neuroblastoma may be found. Urothelial carcinomas are very unusual. ...
... Many benign, malignant, and reactive bladder lesions are encountered in childhood. [1] Standard treatment in children is transurethral resection of the tumor. Usually, no postoperative intravesical chemotherapy or immunotherapy is needed in children. ...
Article
Full-text available
A 12-year-old male child, during evaluation of chronic constipation, was incidentally diagnosed to have a fronded bladder growth. Transurethral en bloc excision of the tumor was achieved using holmium laser. Histopathology confirmed it to be an inflammatory pseudotumor. This case is reported for its unusual presentation and management by holmium laser.
... Malignant lesions include rhabdomyosarcomas (RMS), leiomyosarcomas, urothelial carcinomas, and secondary involvement such as lymphomas, with RMS being by far the most common [9,11,12]. ...
... In a study of 65 pediatric patients with polyps and masses in the urinary bladder, reactive/inflammatory masses comprised 20 (31%), RMS comprised 15 (23%), and other neoplasms (benign and malignant) 22 (33%) [12]. ...
Article
Full-text available
We report a case of eosinophilic cystitis in a 7-year-old boy with a history of atopic symptoms, with focus on the radiological findings. He presented with hematuria and dysuria and ultrasonography (US) showed irregular bladder wall thickening resembling a bladder mass. CT urography did not characterize the lesion any further and showed no local or distant spread. Biopsies revealed eosinophilic cystitis, a benign inflammatory condition. We found that US characterized the lesion at least as well as CT and should be the first choice of imaging. When staging is considered before biopsy, MRI should be preferred to CT. There are no specific radiological signs of eosinophilic cystitis. On follow-up, US was a safe, cost-effective imaging modality, but findings should be interpreted in a clinical context. In a child with hematuria and a bladder mass, eosinophilic cystitis is a relevant but rare differential diagnosis, especially when there is a known atopic history.
... 8,9 In children they have been documented antenatally, 10 and in many cases are thought to be congenital; 11 however, they may occur in multiple sites in the lower urinary tract in children post-pyeloplasty. 12,13 These polypoid or cauliflower-like masses have a median size of 2.5 cm (maximal size reported is 8 cm 14 ), and usually involve the ventral glans penis. 1,15,16 Clinically, the differential diagnosis includes condyloma acuminatum, giant condylomas (called Buschke-Löwenstein tumors), verrucous carcinoma, squamous cell carcinoma, urethral carcinoma and angiomyxoma. ...
Article
Fibroepithelial polyps are rare benign tumours of the glans penis; there are only a few reported cases. The pathogenesis is unknown. However, they have been linked with chronic condom catheter use or prior penile surgery. We report a case of a 62-year-old man with a large fibroepithelial polyp of the glans penis of 11 years duration, which was not associated with condom catheter use or prior surgery. The mass was large, measuring 7 × 5 × 3 cm. Fibroepithelial polyps have been reported in a range of genito-urinary sites in males and females, adults and children, and in rare cases may be associated with malignant transformation. They should be considered in the differential diagnosis of both cutaneous and mucosal genitourinary lesions.
... On the contrary, bladder hemangiomas are typically characterized by proliferated of vessel walls with distinct borders and spreading between the normal vasculature, and which lack distinct endothelial atypia or multilayering and with favorable prognosis [23] . The differential diagnosis of a polypoid bladder mass detected in children with painless gross hematuria includes not only hemangioma but also rhabdomyosarcoma, other vascular tumors, in ammatory pseudotumor, leiomyoma, neuro bromatosis, pheochromocytoma, transitional cell papilloma, transitional cell carcinoma, and pseudo tumoral cystitis [25][26] . ...
Preprint
Full-text available
Background: Although hemangioma in the genitourinary system occurs relatively infrequently, bladder hemangioma has arisen during medical research. We describe two rare cases of urinary bladder hemangioma (UBH), in which was confirmed histopathology followed by Immunohistochemistry (IHC) and review the literature on the diagnosis and treatment of patients with this disease to raise awareness of urinary bladder hemangioma and appropriate management. Case presentation: A 41-year-old Chinese female presented with a sudden onset of painless gross hematuria for one month, Multi-Slice spiral computed tomography urography in the urological system showed the anterior and superior wall thickening of the bladder, multiple nodules and masses, exhibited multiple punctate calcifications and marked uneven enhancement. The second case is a 30-year-old female who was asymptomatic and incidentally founded a large bladder tumor during a routine examination from outside hospital, magnetic resonance imaging scan confirmed a 6.2cm x 6.9cm x 5.2cm soft tissue mass arising from the right anterior and superior wall of the bladder, which suggested the possibility of a benign bladder tumor. Postoperative pathology confirmed the diagnosis of urinary bladder hemangioma. The radical cystectomy was performed with open-methods surgery associated with an abdominal wall ostomy of the ileal outlet tract for case 1. And case 2 finally underwent a Laparoscopic partial cystectomy. Hematuria resolved postoperatively and no evidence of tumor recurrence in three year follow-ups for case 1. Postoperative urinary and pelvic ultrasonography showed no signs of recurrence in three months follow-ups for case 2. Conclusion: Urinary bladder hemangioma is a benign non-urothelial tumor that rarely occurred in pediatric and adolescent patients . Clinical and radiological examinations are not adequate for an accurate diagnosis. Careful histopathological and immunohistochemical studies are required to establish the correct diagnosis. There is no ‘gold standard’ treatment for UBH, treatment options are varied for individuals with favorable follow-ups.
... Transitional cell carcinoma of the bladder in children is very rare, with about 150 patients younger than 20 years presented in the literature, only 20% of them in the first 10 years of age. It predominates in white children with a male-to-female ratio of 3:1 (46)(47)(48). ...
Article
Full-text available
Tumors of the kidney, bladder, prostate, testis, and adrenal represent a large part of the adult urologic practice, but are relatively infrequent in children. The natural history and management of these tumors in the pediatric age is different from that of the adults. As result of the successful work of several clinical trial groups in recent decades, there has been a significant improvement in their cure rates. The aim of this article is to review their most significant clinical aspects, as well as to present an update in their management.
... Malignant vascular tumours, such as angiosarcoma, have highly aggressive potential with the features of infiltrative growth, clear cytological atypia, high cellularity, and poor prognosis. By contrast, bladder haemangiomas are typically characterized by the proliferation of vessel walls with a clear boundary and spreading between the normal vasculature, which lack typical endothelial atypia or multilayering and have a satisfying prognosis [24]. The differential diagnosis of a polypoid bladder mass found in children with painless gross haematuria includes haemangioma, rhabdomyosarcoma, other vascular tumours, inflammatory pseudotumours, leiomyoma, neurofibromatosis, pheochromocytoma, transitional cell papilloma, transitional cell carcinoma, and pseudotumoural cystitis [25,26]. ...
... Frequent causes of hematuria such as stone, cyst, and tumor must be excluded in patients suspected with HS(6). In 21 years experience of Huppman et al.; although mass in the bladder is occasional in pediatric population, the most common one is rhabdomyosarcoma and masses of children in the bladder has a very wide variation (7). Although underlying the use of chemotherapy, bladder mass detected after macroscopic hematuria complicates the differential diagnosis in this case of children. ...
... 7/M + F <16 Low-grade TURBT NR after 18 months [9] ...
Article
Full-text available
Urothelial bladder tumour in childhood is extremely rare, and almost all the reported cases have been low-grade tumours with a favourable outcome. Here we review 57 reports comprising 127 cases, and we report two new cases.
... The most frequently it affects the bladder trigone, followed by the dome localization, posterior wall and lateral wall. Rarely affects children and is characterized by the presence of intestinal type of epithelium (1,5). The GC can coexist with the IM, as was the case of our patient, that had a rare localization of the tumor, mostly inside the cavity but also attached to the bladder dome. ...
Article
Benign tumoral bladder tumors in children are extremely rare. The inflammatory condition determined by the presence of glandular cystitis and intestinal metaplasia, coexisting or separately, can be a determinant factor for the development of these bladder masses. Both entities have been suggested to represent a precursor for bladder adenocarcinoma. We present the case of an autistic adolescent that presented with a polypoid bladder mass and urinary tract infection. We performed a cystoscopic examination and biopsy of the tumor and due to the large dimensions and preliminary histological results we decided to perform a laparotomy and excision of the mass and bladder wall that was attached to. Post operatory evolution and follow-up showed no recurrence or progression of the dysplasia towards adenocarcinoma.
... A clinical history for only one of these patients was available in this retrospective study, and it remarked on a prior bladder exstrophy repair. 3 A small number of individual cases have been reported in children over the years. [4][5][6] An association has been documented with bladder exstrophy, pelvic lipomatosis and recurrent urinary tract infections. ...
Article
Full-text available
The macroscopic appearances of florid cystitis cystica et glandularis can be mistaken for malignancy, and it is therefore important to perform a prompt resection to confirm the histological diagnosis and exclude sinister pathology.
... Un análisis retrospectivo de masas vesicales entre 1988 -2009 describe al rabdomiosarcoma como la neoplasia vesical más frecuente, seguido de lesiones reactivas e inflamatorias, entre las que se mencionan la cistitis polipoide (4%), cistitis quística glandular (3%) y adenoma nefrogénico (2%). Más infrecuentes aún, las neoplasias de origen urotelial incluyen entidades como la Atipia/Displasia Urotelial (5%), Papiloma Urotelial (2%), Neoplasia Urotelial Papilar de bajo potencial maligno (PUNLMP) (2%), Carcinoma Papilar de Bajo y Alto Grado (1%) y el Papiloma Invertido (< 1%) 1,4 . ...
Article
Full-text available
Introducción: El papiloma urotelial invertido es una neoplasia vesical inusual en la población pediátrica. Debido a su baja incidencia y al reporte de componentes atípicos, no existe consenso respecto a su manejo. Objetivo: reportar una adolescente con diagnóstico de papiloma invertido, y revisar la aproximación diagnóstica, terapéutica y plan de seguimiento de esta entidad. Caso Clínico: Adolescente de 13 años con hallazgo ecográfico incidental de lesión polipoídea vesical localizada en pared póstero-inferior, durante estudio de dismenorrea. Se realizó resección cistoscópica y el informe histopatológico confir mó un papiloma urotelial invertido. La paciente evolucionó asintomática, sin evidencia de recurren cia tras 3 años de seguimiento ecográfico posterior a su resección. Conclusiones: El caso reportado soporta el comportamiento benigno del papiloma invertido, destacando la importancia del correcto diagnóstico histopatológico para la decisión de realizar un seguimiento no invasivo ecográfico.
... Urothelial carcinoma may occur as a secondary primary tumor in young adult survivors of retinoblastoma or in the setting of other childhood cancer-predisposing syndromes, such as Costello syndrome and hereditary nonpolyposis colorectal cancer syndrome (10). Patients with a history of augmentation cystoplasty have an increased risk of developing urothelial carcinoma, which in this setting occurs more frequently at bladder-intestine anastomosis sites and is more likely to be of high grade and have an aggressive clinical course (57). ...
Article
Urinary bladder masses are rare in children, and the associated histologic features and prognoses in this population are different from those in adults. Most children with urinary bladder masses present with lower urinary tract symptoms, which may include hematuria, dysuria, frequent urination, and urgency to urinate. However, some of these masses may be identified incidentally or involve generic symptoms such as abdominal distention. In general, pediatric bladder tumors can be divided into those that originate from the bladder epithelium, known as urothelial neoplasms, and mesenchymal bladder neoplasms, which are more prevalent. The most common bladder malignancy in children is a rhabdomyosarcoma, whereas the most common benign bladder lesion in the pediatric population is a papillary urothelial neoplasm of low malignant potential (PUNLMP). The first-line imaging tool for assessing bladder lesions is ultrasonography, which may be followed by a cross-sectional imaging examination such as computed tomography or magnetic resonance imaging if the origin of the mass is unclear or if distant spread is suspected. Although imaging may enable the radiologist to suggest a differential diagnosis based on lesion location and patient age, tissue biopsy generally is required to identify the exact pathologic entity. This is usually performed at cystoscopy and may be curative in cases in which the lesion is small and has low recurrence potential. Knowledge of the clinical, histopathologic, and imaging features of common bladder neoplasms is essential, as it can aid in preventing imaging pitfalls. These may include the misinterpretation of either a pelvic mass as arising from the bladder or a bladder mass as arising from the pelvis, and interpreting an inflammatory mass or bladder detritus as a neoplasm.
... Bladder tumours are rare in the paediatric population, with rhabdomyosarcoma being the most common constituting 32% of bladder tumours. 6 The classical presentation of bladder tumour is painless haematuria. Some patients can present with obstructive symptoms such as urgency, frequency and dysuria. ...
Article
Full-text available
Paragangliomas of the urinary bladder are very rare tumours representing less than 1% of bladder tumours. Preoperative diagnosis is essential to avoid perioperative complications related to catecholamine release. A high index of suspicion should be maintained when the classical symptoms of voiding-related paroxysms of headache, palpitation and dizziness are present. We present a rare case of malignant paraganglioma of the urinary bladder in a 10-year-old boy. The patient had the classic presentation. We review the radiological diagnostic tools and findings of this rare entity.
Article
ABSTRACT Similar to nephrogenic adenomas in adults, those in children are rare benign lesions that often occur in the setting of previous surgery or chronic irritation of the urinary tract. These lesions often present with hematuria and/or as polypoid or papillary lesions on cystoscopy, which may indicate malignancy. We sought to evaluate the various patterns of nephrogenic adenoma occurring in the pediatric population and better characterize the immunophenotype of these lesions. We reviewed 21 cases of nephrogenic adenomas from urinary bladder biopsies of 16 patients. Most patients had a history of urinary bladder augmentation with recurrent urinary stones and urinary tract infections. Many cases presented as a papillary or polypoid mass on cystoscopy. The most common morphologic patterns are papillary, tubulocystic, and a mixed pattern of papillary and tubulocystic, followed by cystic and tubular. On immunostaining, PAX-2, PAX-8, CK7, and MUC-1 provided the most diffuse and intense positive reactivity for nephrogenic adenoma, whereas CD10 and P504S were focal and lesser in intensity when positive. p63 and PAX-5 were consistently negative. We conclude that, although rare in children, nephrogenic adenoma should be included in the differential diagnosis of papillary/polypoid lesions in the urinary tract, especially in the context of previous surgery, chronic irritation from recurrent urinary tract infections, or stones. The immunohistochemistry profile of nephrogenic adenomas in our study also provides evidence that these are derived from distal renal tubular cells. In difficult cases, an immunohistochemical panel consisting of cytokeratin 7, PAX-2, PAX-8, and MUC-1 may be useful.
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Both benign and malignant pelvic masses are encountered in the pediatric population. Although ultrasonography remains the modality of choice for initial evaluation of a pediatric pelvic mass, in selected cases magnetic resonance (MR) imaging can add important diagnostic information. MR imaging has several advantages over ultrasonography and computed tomography, including superior contrast resolution and an ability to characterize abnormalities based on unique tissue characteristics. MR evaluation assists in lesion characterization, presurgical planning, and staging when a malignancy is suspected. MR imaging also offers a nonionizing imaging modality for long-term follow-up of patients undergoing therapy for malignant pelvic masses.
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Bladder chronic inflammation is associated with the pathogenesis of bladder cancer; the underlying mechanism is unclear. The PT53 gene is an important anticancer gene in the body, which is suppressed in cancer. The ubiquitin E3 ligase A20 (A20) plays a role in regulating the activities of epithelial cells. This study was designed to investigate the correlation between A20 and the pathogenesis of bladder cancer. The biopsy tissues of human bladder cancer, bladder polypoid cystitis, and chronic inflammation were collected; the levels of A20 and p53 were analyzed by quantitative real-time RT-PCR, Western blotting, and immune precipitation. HEK293 cells were employed to test the role of overexpression of A20 in the suppression of the p53 gene in the cells. Fifty-six patients with bladder cancer, 48 patients with bladder polypoid cystitis, and 16 patients with bladder chronic inflammation were recruited into this study. Human bladder cancer tissue and the polypoid tissue showed high levels of A20, which had a positive correlation with the tumorigenesis in the bladder; 12 out of 46 (26.1 %) patients with bladder polypoid cystitis were diagnosed as bladder cancer. A20 bound to p53 to form complexes in bladder cancer tissue and bladder polypoid tissue. The overexpression of A20 suppresses p53 protein levels in HEK293 cells. A20 has a positive correlation in the tumorigenesis of bladder polypoid disorders.
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Introduction: Clear cell sarcoma of the kidney (CCSK) is childhood neoplasm with its own distinctive pattern of metastasis and may appear after a disease free interval of 5 years or more. Materials and methods: Histopathology and immunohistochemistry were available from the radical nephrectomy and the later partial cystectomy, which was performed after a seven disease-free interval. Results: The pathologic features of the primary tumor were those of a classic CCSK with a monotypic pattern of uniform rounded to ovoid tumor cells with a background network of delicate blood vessels. By contrast, the bladder recurrence had a myxoid hypocellular appearance (one of the known variant patterns of CCSK). Both tumors displayed immunopositivity for Cyclin-D1 and CD117 with a less intense reaction in the bladder metastasis. Conclusions: This case demonstrates that CCSK has the potential to metastasize after a prolonged disease-free interval and may have deceptively bland histopathologic features.
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Urothelial tumors are very rare in children (to date, only about 150 cases have been reported worlwide). Only 20 % occur before the age of ten. The aim of this study is to specify the clinicopathologic features of urothelial tumor in young patients, which require a slightly different approach to treatment. On the basis of the WHO/ISUP (World Health Organisation/International Society of Urological Pathology) consensus classification report, these lesions are usually low-grade lesions, non invasive, and rarely recurrent. The sex ratio is three boys to one girl. These tumors are located preferentially in the low urinary tract, especially in the bladder. The main symptom is the macroscopic hematuria, which requires ultrasound examination in all cases. Cystoscopy is indicated in case of lesion of the bladder wall, or in case of persistent or recurrent hematuria, to obtain definitive diagnosis and biopsies. The tumors are mainly located on the posterior or lateral bladder wall above the trigone or near the ureteral orifices. Treatment is based on the transurethral resection of the lesion. The subsequent monitoring is sparsely codified, due to the exceptional occurrence of these tumors in the paediatric age group. These patients are likely to have better outcome than older patients, but it is due to the predominance of noninvasive papillary urothelial tumors. Tumor recurrences are not uncommon. In case of invasive, high-grade urothelial carcinomas, metastases or even lethal outcome may occur in rare cases. Copyright © 2016 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.
Chapter
Nephroblastoma or Wilms’ tumor (WT) is the most common renal neoplasm in children accounting for 90 % of pediatric renal tumors (Pastore et al. 2006). It is a tumor with a good prognosis and with well-established treatment strategies. Other rare malignant renal tumors, such as clear cell sarcoma and rhabdoid tumor of the kidney, have a poor prognosis despite aggressive treatment. Renal cell carcinoma occurs in older children, while mesoblastic nephroma is the most frequent renal tumor in the neonate. Hematological malignancies, the most frequent neoplasms in children, may also involve the kidney, most often as part of a multi-organ involvement. Renal infections and malformations are much more common in children than renal tumors and may show a pseudotumoral pattern mimicking a renal tumor. In all cases, close collaboration among radiologists, pediatricians, and pathologists is essential so as to avoid diagnostic pitfalls due to atypical presentations.
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Haematuria in paediatric population is common yet alarming. It warrants a thorough physical examination and other investigations. Of late, a number of extremely rare aetiology of childhood haematuria have come to forefront. One such uncommon cause is benign fibroepithelial urinary bladder polyp. The presentation is of a child with intermittent haematuria exacerbated by physical/sports activity associated with or without suprapubic pain. Diagnosis is usually made by ultrasonography and cystoscopy and confirmed by histopathological examination. Treatment is surgical and involves cystoscopic transurethral resection of the mass. The exact aetiology of benign fibroepithelial polyp is uncertain with no clear guidelines on long-term surveillance. However, these cases should be subjected to cystourethroscopy if haematuria recurs. Treatment is surgical with good long-term prognosis. Not much is written in literature about benign fibroepithelial bladder polyp.
Chapter
Urinary bladder tumors are not common in children and adolescents. They are either urothelial or non-urothelial (Fig. 65.1). The commonest is rhabdomyosarcoma (RMS) of the bladder or prostate/vagina followed by papillary urothelial tumors (Table 65.1) [1]. RMS, being the commonest childhood soft tissue sarcoma, ranks third commonest pediatric solid tumor (5–15%) [2, 3]. A recent analysis of 57 reports of pediatric urothelial bladder tumors which included 127 cases reported patients younger than 20 years old, of which only 21 patients (16.5%) were below 10 years old [4]. Both tumor categories exhibit a 3–9:1 male-to-female preponderance [5].
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There are a number of benign epithelial proliferations in the bladder that may be difficult to distinguish from carcinomas, including urothelial carcinoma and its variants, squamous cell carcinoma and adenocarcinoma. If misdiagnosed, there is the potential for over treatment, with its attendant risk of complications, as well as errors relating to prognostic assessment. In the case of the misdiagnosis of high grade proliferative lesions that mimic invasive carcinoma, unnecessary radical surgery, chemotherapy and radiotherapy may result. Similarly, the misdiagnosis of lesions that have the appearance of low grade carcinoma can prompt a lifetime of radiological investigation and cystoscopies. In this review, we discuss a variety of entities that may be diagnostically challenging and emphasise the importance of identifying key morphological features that have diagnostic utility. We also highlight the importance of relevant clinical information and the clinical settings in which these lesions may occur. In this review we have divided the lesions on the basis of morphology in order to facilitate discussion relating to the differential diagnosis. The architectural patterns we discuss include papillary lesions (polypoid/papillary cystitis and papillary urothelial hyperplasia), pseudocarcinomatous proliferations (pseudocarcinomatous urothelial hyperplasia, florid proliferation of von Brunn nests and fibroepithelial polyps), glandular lesions (intestinal metaplasia and müllerianosis) and lesions with several different patterns (prostatic type urethral polyps and nephrogenic adenoma or metaplasia).
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The urinary tract obstruction is a common problem in pediatrics. When a defect in the urinary tract blocks the normal flow of urine, the urine backs up and causes hydroureters and hydronephrosis. Such abnormalities of the urinary tract may be identified before or after the baby is born. In this chapter some anomalies of the urinary tract as well the pathology of the male genital system are discussed.
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Ultrasound is the imaging modality of choice for assessment of the urinary tract in children, particularly in the workup of hydronephrosis, urinary tract infection, kidney masses, and bladder abnormalities. Other important applications include the evaluation of stone disease, kidney failure, renovascular disease, and kidney transplants. This chapter reviews the development and anatomy of the urinary tract followed by an overview of ultrasound imaging features of urinary tract disorders in children. Brief discussions of clinical presentation and treatment approach are also included.
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Specimens from the prostate and bladder are commonly encountered by the general surgical pathologist. Emphasis is usually placed on neoplasms of the bladder and prostate, particularly if malignant, owing to their therapeutic consequences. A good command of benign lesions occurring in the bladder and prostate, and knowledge of their preneoplastic potential will help pathologists confidently diagnose malignancy versus its benign mimickers and guide the urologists in choosing the appropriate therapy and follow-up for the patient. To present a mixture of benign entities, and discuss their histologic and clinical characteristics, hoping to provide a practical review for the general surgical pathologist. An extensive review of the literature on the entities discussed was performed. A wide variety of benign entities are present in the prostate and bladder. Benign lesions in the prostate can be age related, such as prostatic atrophy and benign prostatic hyperplasia; transition zone associated, such as basal cell hyperplasia, adenosis, and sclerosing adenosis; or prostatic urethra associated. Benign lesions of the bladder encompass a wide variety of reactive changes that can occur in the urothelium, as well as hyperplastic lesions or reactive proliferations that could be misdiagnosed as malignant. The bladder responds to chronic irritation through several reactive/metaplastic lesions such as cystitis cystica/glandularis, keratinizing squamous metaplasia, or nephrogenic metaplasia. The urothelium can also give rise to hyperplastic/proliferative lesions, in particular von Brunn nest hyperplasia, papillary polypoid cystitis, and pseudocarcinomatous proliferation, which should be distinguished from malignant processes. Ectopic tissue, such as prostatic or mullerian, can also be seen.
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Fibroepithelial polyps of the urothelium are rare but frequently mistaken for transitional cell carcinoma. To better define the demographics, urothelial distribution, and typical gross anatomic and radiologic appearances, we reviewed 41 pathologically proven cases. We reviewed 41 cases of fibroepithelial polyps from the archives of the Armed Forces of Pathology. Data were collected from radiographic studies, gross anatomic pathology, and pathology and radiology reports and categorized by age, sex, clinical presentation, lesion size, location, and morphology. The mean patient age was 21 years, and 58% were male. Most presented with hematuria and/or flank pain (68%). Most polyps were located in the upper ureter or renal pelvis (87%). Posterior urethral and bladder polyps were present in children. Most polyps were single or bilobed (73%) and 1-6 cm. Because most urothelial tumors are malignant epithelial tumors, fibroepithelial polyps are commonly mistaken for transitional cell carcinomas. However, because fibroepithelial polyps and malignant urothelial tumors typically present in different patient populations, different locations in the urinary tract, and appear different radiographically, distinguishing features between these entities is helpful in determining the differential diagnosis of a urothelial mass. In the appropriate clinical setting, fibroepithelial polyps should be considered in the differential diagnosis, which will affect surgical treatment.
Article
Context.—The differential diagnoses of prostatic carcinoma and bladder epithelial neoplasms include several histologic mimics that should be known to avoid misdiagnosis. Objective.—To discuss pseudoneoplastic lesions of the prostate and bladder that could potentially be confused with prostatic carcinoma and bladder epithelial neoplasms, respectively, with specific focus on their distinguishing histopathologic features. Data Sources.—Relevant published literature and authors' experience. Conclusions.—Pseudoneoplastic lesions in the prostate include those of prostatic epithelial origin, the most common being atrophy, adenosis (atypical adenomatous hyperplasia), basal cell hyperplasia, and crowded benign glands, as well as those of nonprostatic origin, such as seminal vesicle epithelium. Such lesions often mimic lower-grade prostatic adenocarcinoma, whereas others, such as clear cell cribriform hyperplasia and granulomatous prostatitis, for example, are in the differential diagnosis of Gleason adenocarcinoma, Gleason grade 4 or 5. Pseudoneoplastic lesions of the urinary bladder include lesions that could potentially be confused with urothelial carcinoma in situ, such as reactive urothelial atypia, and others, such as polypoid/papillary cystitis, where papillary urothelial neoplasms are the main differential diagnostic concern. Several lesions can mimic invasive urothelial carcinoma, including pseudocarcinomatous hyperplasia, von Brunn nests, and nephrogenic adenoma. Diagnostic awareness of the salient histomorphologic and relevant immunohistochemical features of these prostatic and urinary bladder pseudoneoplasms is critical to avoid rendering false-positive diagnoses of malignancy.
Article
BACKGROUND Neurofibroma of the urinary bladder is rare. Only isolated case reports have appeared. Information regarding the long term follow-up of patients with neurofibroma is limited.METHODS The authors studied 4 cases of neurofibroma of the bladder diagnosed at Mayo Clinic from 1965 through 1990. Immunostains for S-100 protein, neurofilament protein, epithelial membrane antigen (EMA), cytokeratin (CAM 5.2; AE 1/3), Type IV collagen, MIB-1, and p53 protein were performed in all cases, as was Alcian blue at pH 2.5. The mean follow-up was 9.6 years (range, 2–18 years).RESULTSThe mean age at diagnosis was 17 years (range, 7–28 years); the male-to-female ratio was 1:1. All four patients exhibited physical stigmata of neurofibromatosis type 1. Clinical presentations included hematuria (one patient), irritative symptoms (two patients), and pelvic mass (one patient). Long term urinary complications included bladder atony (two patients), neurogenic bladder (one patient), and recurrent urinary tract infection with hematuria (one patient). Subsequently, two patients underwent partial cystectomy and one a complete cystectomy. Involvement of the bladder was generalized in all four cases. Three tumors were transmural, showing a diffuse and plexiform pattern of growth; in the fourth case, a superficial biopsy showed only diffuse submucosal growth with conspicuous pseudo-Meissnerian corpuscle formation. An Alcian blue positive, variably collagenized matrix was present in all cases. Tumor cells displayed immunoreactivity for S-100 protein and Type IV collagen in all cases. Neurofilament protein positive axons were evident in three cases; all other immunostains were negative. The mean MIB-1 labeling index was 3.2% (range, 0.9–7.3%). No malignant transformation was observed during a mean follow-up of 9.6 years.CONCLUSIONS Neurofibroma of the bladder presents early in life, is of the plexiform type with a diffuse component, and usually occurs in the setting of generalized neurofibromatosis type 1 rather than as isolated visceral neurofibromatosis. Malignant transformation did not occur in any of these 4 patients during a mean follow-up time of 9.6 years. Cancer 1999;86:505–13. © 1999 American Cancer Society.
Article
As an initial manifestation of neurofibromatosis type 1, isolated neurofibroma of the urinary bladder occurs only rarely in the first 2 decades of life. We report a case of a 6-year-old African-American girl who presented in this manner and was found to have a plexiform neurofibroma as well as diffuse ganglioneuromatosis of the urinary bladder. We describe the clinical presentation, diagnostic procedures, and pathologic features encountered. In addition, we review the literature with respect to etiology, current treatment strategies, and the issue of surveillance for this complex lesion occurring in the pediatric population. Neurofibroma of the bladder should be considered in the differential diagnosis of painless hematuria in childhood. Its presentation in such patients warrants a complete evaluation to establish the diagnosis of neurofibromatosis type 1 and begin long-term surveillance for its associated manifestations. At present, there are no established criteria for the treatment of this rare lesion.
Article
The differential diagnoses of prostatic carcinoma and bladder epithelial neoplasms include several histologic mimics that should be known to avoid misdiagnosis. To discuss pseudoneoplastic lesions of the prostate and bladder that could potentially be confused with prostatic carcinoma and bladder epithelial neoplasms, respectively, with specific focus on their distinguishing histopathologic features. Relevant published literature and authors' experience. Pseudoneoplastic lesions in the prostate include those of prostatic epithelial origin, the most common being atrophy, adenosis (atypical adenomatous hyperplasia), basal cell hyperplasia, and crowded benign glands, as well as those of nonprostatic origin, such as seminal vesicle epithelium. Such lesions often mimic lower-grade prostatic adenocarcinoma, whereas others, such as clear cell cribriform hyperplasia and granulomatous prostatitis, for example, are in the differential diagnosis of Gleason adenocarcinoma, Gleason grade 4 or 5. Pseudoneoplastic lesions of the urinary bladder include lesions that could potentially be confused with urothelial carcinoma in situ, such as reactive urothelial atypia, and others, such as polypoid/papillary cystitis, where papillary urothelial neoplasms are the main differential diagnostic concern. Several lesions can mimic invasive urothelial carcinoma, including pseudocarcinomatous hyperplasia, von Brunn nests, and nephrogenic adenoma. Diagnostic awareness of the salient histomorphologic and relevant immunohistochemical features of these prostatic and urinary bladder pseudoneoplasms is critical to avoid rendering false-positive diagnoses of malignancy.
Article
To estimate the risk of a second primary tumour (SPT) of the bladder in a cohort of childhood cancer survivors, investigate factors associated with a bladder SPT developing, and compare the risk observed with that expected from the general population. The analysis included 17981 individuals diagnosed with childhood cancer, between 1940 and 1991 in Britain, and surviving for ≥5 years. Ascertainment of a bladder SPT was primarily through the National Health Service Central Registers (NHSCR). From the NHSCR, 17 bladder SPTs were ascertained; this corresponded to four times (95% confidence interval 2.5-6.4) the expected number of bladder tumours. Standardized incidence ratios (SIRs) varied significantly (P < 0.05) by first primary tumour (FPT) type, follow-up period, attained age and chemotherapy. The highest SIRs were in those: with heritable retinoblastoma (31.4); treated with chemotherapy (12.0); 0-9 years of follow-up (10.8); and aged 0-19 years (9.3). The absolute excess risk (AER) for a bladder SPT was 3.7 cases/100000 survivors per year. The AER varied significantly by FPT type, follow-up period, attained age and gender. The highest AERs were in those: diagnosed with heritable retinoblastoma (34.0); 20-29 years of follow-up (14.2); aged 40-49 years (13.0); and male (5.8). Using multivariable Cox regression, FPT and chemotherapy were significantly associated with the risk of a bladder SPT developing. By the age of 55 years, 0.4% of survivors developed a bladder SPT. Although the absolute risk of a bladder tumour within childhood cancer survivors was low, the risk was four times that expected from the general population. Specific groups, e.g. survivors of heritable retinoblastoma and those treated with chemotherapy, were at the highest risk.
Article
Tumor-like lesions of the urinary bladder are reviewed emphasizing those that are most diagnostically challenging for the pathologist and may result in serious errors in patient care if misinterpreted. The first category considered, pseudocarcinomatous proliferations, represents an area of bladder pathology only recently appreciated as being particularly treacherous because of the extent to which irregular islands of benign epithelial cells may seemingly penetrate the lamina propria and cause confusion with carcinoma. Somewhat orderly proliferations of this type have been known for years, von Brunn's nests, and are rarely a challenge for the experienced, but proliferations of an irregular nature such as may be seen most often as a result of prior radiation therapy, but sometimes due to chemotherapy or even ischemia, represent a challenging interpretation. The clinical history may be very important in arriving at the correct diagnosis as is the appreciation that the morphology, although architecturally problematic, is different from that of any of the familiar patterns of invasive carcinoma. Florid epithelial proliferations in fibroepithelial polyps are also briefly noted. Within the category of glandular proliferations, emphasis is placed on the wide spectrum of morphology of nephrogenic adenoma including its pseudoinfiltrative pattern and occasional propensity for tiny tubules to be misconstrued as signet-ring cells. The spectrum of müllerian glandular lesions including the relatively recently described mucinous variant, endocervicosis, is reviewed. The reactive papillary proliferation, papillary-polypoid cystitis, is then discussed. This entity has long been known but has recently been re-emphasized. Other non-neoplastic papillary lesions include florid papillary forms of nephrogenic adenoma. The past 25 years has seen a great expansion of knowledge concerning non-neoplastic spindle cell proliferations, including those related to a prior procedure, the postoperative spindle cell nodule and those without such a history, variously designated inflammatory pseudotumor, pseudosarcomatous myofibroblastic proliferation, or even other terms. The morphologic spectrum is explored and it is recommended that the two categories be retained.
Article
This WHO/ISUP system is an attempt to develop as broad a consensus as possible in the classification of urothelial neoplasms, building upon earlier works and classification systems. It is meant to serve as a springboard for future studies that will help refine this classification, thus enabling us to provide better correlation of these lesions with their biologic behavior using uniform terminology.
Article
We report on a 12-year-old boy with Costello syndrome born to consanguineous (first cousins once removed) parents, supporting the hypothesis of recessive transmission of this syndrome. At age 11 years, the patient developed a bladder carcinoma, a rare pediatric tumor not previously described in Costello syndrome. This suggests that an increased risk of malignancy may be part of this condition.
Article
We report five new cases of rhabdomyosarcoma (RMS) in Costello syndrome. These cases, combined with those previously reported, increase the number of solid tumors to 17 (10 RMSs, 3 neuroblastomas, 2 bladder carcinomas, 1 vestibular schwannoma, 1 epithelioma), in at least 100 known Costello syndrome patients. Despite possible ascertainment bias, and the incomplete identification of all Costello syndrome patients, the tumor frequency could be as high as 17%. This is comparable to the 7-21% frequency of solid tumors in Beckwith-Wiedemann syndrome (BWS), and may justify tumor screening. Based on the recommendations for screening BWS patients, we propose a screening protocol consisting of ultrasound examination of the abdomen and pelvis every 3-6 months until age 8-10 years for RMS and abdominal neuroblastoma; urine catecholamine metabolite analysis every 6-12 months until age 5 years for neuroblastoma; and urinalysis for hematuria annually for bladder carcinoma after age 10 years. These recommendations may need to be modified, as new information becomes available. Potential criticism of the tumor screening protocol concerns the lack of evidence for improved outcome, and possible overestimation of the tumor risk. The ability of RMSs to occur at various sites complicates tumor screening, but 8 of the 10 RMSs in Costello syndrome patients originated from the abdomen, pelvis and urogenital area. Prior diagnosis of Costello syndrome is a prerequisite for the implementation of any screening protocol. The diagnosis of Costello syndrome should also be considered in individuals with RMS and physical findings suggestive of Costello syndrome.
Article
Bladder epithelial tumors are uncommon in children. The Costello syndrome is a rare disorder that involves multiple congenital abnormalities, and affected patients may be at increased risk for tumor development. We report recurrent and multiple transitional cell carcinomas of the bladder in a girl with the Costello syndrome. To our knowledge this case represents the third report of transitional cell carcinoma associated with the Costello syndrome.
Article
Fibroepithelial polyps of the urinary tract are rare with most cases reported in children. We report the clinicopathologic features of 12 fibroepithelial polyps of the lower urinary tract in adults. There were 9 males, 2 females, and 1 patient where the gender was unknown (median age, 44 years; range, 17-70 years). Chief clinical symptoms were hematuria, urinary urgency, and hesitancy. Five patients were asymptomatic, where the lesions were discovered incidentally. Most lesions were located near the verumontanum or the bladder neck. Ten patients were treated by transurethral resection. Of the 10 patients with follow-up information, none showed recurrence (mean, 20 months; median, 17 months). Histologically, all of the fibroepithelial polyps were lined by normal-appearing urothelium, with in one lesion the additional finding of a columnar epithelial lining. There were three overall architectural patterns seen within fibroepithelial polyps. The most common pattern (Pattern 1) seen in 5 cases consisted of a polypoid mass with club-like projections resembling a cloverleaf with florid cystitis cystica et glandularis of the nonintestinal type in the stalk. The second pattern (Pattern 2) seen in 4 cases consisted of a papillary tumor composed of numerous small, rounded fibrovascular cores containing dense fibrous tissue. The last morphologic pattern (Pattern 3) consisted of a polypoid lesion with secondary tall finger-like projections, which was seen in 3 cases. All lesions lacked prominent edema and inflammation seen in polypoid cystitis. Fibroepithelial polyps contained broader stalks with dense fibrous tissue, in contrast to the thin delicate loose fibroconnective tissue seen in the stalk of papillomas. One lesion contained atypical degenerative-appearing stromal cells. Although fibroepithelial polyps have been considered to be congenital, we think that some of these polyps could develop after birth because all of our patients first showed clinical symptoms in adulthood. Because fibroepithelial polyps in adults are rare, some of these cases can be misdiagnosed as urothelial neoplasms or reactive conditions. Recognition of the precise histologic features of fibroepithelial polyp can facilitate its correct diagnosis.
Article
Urothelial neoplasms in patients younger than 20 years are rare, with conflicting data regarding clinical outcomes. We identified 23 patients 4 to 20 years old with urothelial neoplasms, reclassified the microscopic diagnoses using the 2004 WHO/International Society of Urologic Pathology grading classification and collected data on presentation, risk factors and outcomes. Pathological grading revealed 2 urothelial papillomas, 10 papillary urothelial neoplasms of low malignant potential (PUNLMPs), and 8 low grade and 3 high grade papillary urothelial cancers, all without invasion. Mean patient age was 13.2 years (range 4 to 20), 19 patients were male and 19 presented with gross hematuria. All lesions were solitary and measured 0.1 to 6 cm. One patient had a history of smoking and 1 had parents who smoked. Three patients (13%) had recurrences classified as either urothelial papilloma (1) or PUNLMP (2). All patients were alive with no evidence of disease after a mean followup of 4.5 years (range 6 months to 13 years). Urothelial neoplasms in individuals younger than 20 years more commonly occur in males and are predominantly low grade with a favorable clinical outcome. Before the current classification system the 10 patients with a diagnosis of PUNLMP would have been classified as having papillary carcinoma. Thus, the diagnostic category of PUNLMP allowed 43.5% of patients in this series to avoid being labeled with "cancer" at a young age.
Article
Pseudosarcomatous myofibroblastic proliferation of the genitourinary tract is rare and may develop after trauma or spontaneously. The aim of this study was to characterize further the clinicopathological features of these lesions and to examine their relationship to inflammatory myofibroblastic tumour (IMT). Twenty-seven cases of pseudosarcomatous myofibroblastic proliferation were analysed. There were seven males and 20 females; median age was 37 years (range 16-88). Most lesions were from the bladder (n = 21), while others were in the urethra, vulva, vagina, rectum and retrovesical space. Median tumour size was 30 mm (range 6-120 mm). Seven cases (25%) had a history of prior trauma or surgery. Three cases recurred locally but not destructively. The tumours had fasciitis-like features including bland spindle cells with evenly distributed chromatin, admixed inflammatory cells (mainly lymphocytes) and often a myxoid stroma. Immunohistochemistry showed positivity for smooth muscle actin in 14/20 cases, keratin in 8/19, desmin in 7/20 and anaplastic lymphoma kinase (ALK) in 10/21 cases. Fluorescent in situ hybridization was performed in six ALK+ cases; all were negative for ALK gene rearrangement. Pseudosarcomatous myofibroblastic proliferations of the genitourinary tract may show ALK immunopositivity but do not show consistent ALK rearrangement. Given subtle morphological differences and more consistently benign behaviour, their relationship to inflammatory myofibroblastic tumour at other sites remains uncertain.
Article
Inflammatory pseudotumor or pseudosarcomatous fibromyxoid tumor and postoperative spindle cell nodule of the bladder are unusual lesions of uncertain pathogenesis which share overlapping, if not identical, histologic features. We present our experience with 42 cases, the largest series to date, to study the etio-pathogenesis, histologic features, biologic behavior and relationship to "inflammatory myofibroblastic tumor" of childhood. Patients ranged in age from 7 to 77 years (mean 47 y) and males predominated (3.2:1). Most patients presented with hematuria (31/42). Common associations were smoking (10/30) and previous instrumentation or surgery (9/42). The clinicopathologic features of patients having or not having prior instrumentation were identical. Grossly the lesions were polypoid or nodular and involved any portion of bladder wall, most commonly the dome (9/27) and measured 1 to 10 cm (mean 4 cm). They were composed of spindled and stellate cells arranged in a myxoid background with numerous inflammatory cells. Myxoid hypocellular areas were more pronounced near the mucosal surface with greater cellularity and a fascicular arrangement in the deep aspect of the lesion. "Atypical" features included mitotic activity (0 to 20/10 HPF; mean 2/10 HPF; median 1/10 HPF; none atypical), necrosis (22/42), and extension into muscularis propria (28/32) or perivesicular fat (3/8). Lesions were positive for cytokeratin (31/33), SMA (23/34), desmin (21/35), and Alk-1 protein (12/26). FISH confirmed the Alk-1 translocation in 4/6 cases. Treatment included transurethral resection (30/42), partial cystectomy (9/42), and total cystectomy (3/42). Initial diagnostic error resulted in radiotherapy and chemotherapy in 3 patients. Follow-up was available in 28 patients. (range 3 to 93 mo; median 25 mo). Three patients developed recurrences, but none had metastases. Because the clinicopathologic features of lesions associated with and without instrumentation were similar and inseparable, we believe they are essentially the same entity, and propose the term pseudosarcomatous myofibroblastic proliferation. The preponderance of evidence which includes the extravesical growth, local recurrence, and Alk-1 gene translocation in some cases suggests perhaps a neoplastic process with limited growth potential. Even in the face of atypical histologic features (muscle invasion and necrosis) the prognosis is excellent. Despite the Alk-1 gene translocation, there continues to be sufficient evidence for regarding these as distinct from the so-called inflammatory myofibroblastic tumor of childhood.
Article
Benign nerve sheath tumors (schwannoma and neurofibroma) involving the urinary bladders are rare with only case reports and limited series. We identified 6 neurofibromas and 2 schwannomas involving the urinary bladder. Of the 8 cases, 7 were sent to one of the authors in consultation and the last came for treatment to The Johns Hopkins Hospital. Patients (3 males, 5 females) ranged in age from 3 to 69 years old (average 36 y old). Presenting symptoms included recurrent infection in 6 patients, hematuria in 3 patients, and irritative symptoms in 3 patients (some patients with 2 symptoms). Three patients had a pelvic mass on physical examination. Three patients (3/8) had a solitary lesion in the bladder, 4 patients (4/8) had multifocal lesions, and 1 patient had no information available. Five patients underwent local resection. One patient, who was 3 years old, had multiple plexiform neurofibromas within the bladder and did not have surgical treatment and another patient is also undergoing surveillance. One patient had no treatment information available. Seven patients had clinical follow-up information available. The length of follow-up varied from 2 to 124 months (average 47 mo). Three out of the seven patients with follow-up information had neurofibromas in other sites, including the skin, uterus, mesentery, and ureter. One patient was known to have other stigmata of neurofibromatosis. Both cases with schwannoma had only bladder involvement. No family history of neurofibromatosis and no genetic studies were performed on any of the patients. Three neurofibromas were of the diffuse type, with 2 cases plexiform, and 1 case could not be subtyped. Neither recurrence nor malignant transformation was demonstrated on follow-up. The importance of accurately diagnosing plexiform neurofibromas of the bladder is that a subset harbors neurofibromatosis. If the lesion is focal, conservative excision is the choice of treatment with a low risk of recurrence. Diffuse neurofibromas can be difficult to diagnose leading to delay of treatment and potentially the need for a more extensive excision. Once recognized as a neurofibroma, it is important to identify it as a diffuse neurofibroma, given its lack of relationship to neurofibromatosis.
  • Gripp KW
  • Urakami S