Juvenile Xanthogranuloma Presenting as a Testicular Mass in Infancy: A Clinical and Pathologic Study of Three Cases
ABSTRACT Juvenile xanthogranulomas (JXG) is a histiocytic disorder whose most common clinical presentation is a solitary cutaneous nodule in a child under 5 years of age, but it has come to be recognized that solitary extracutaneous lesions may present in a number of sites, including the soft tissues and various organs. Involvement of the genitourinary tract has been documented in children with multifocal or systemic JXG. The current report describes our experience with 3 cases of JXG presenting as a solitary mass in the testicle of infants between 2.5 and 13 months without manifestations of JXG elsewhere. The masses were intratesticular and had an infiltrative pattern of interstitial growth by mononuclear, histiocyte-like cells with replacement of seminiferous tubules and involvement of the epididymis whose pattern resembled leukemic and lymphomatous involvement of the testis. Because extracutaneous lesions of JXG may have few or no Touton giant cells, immunohistochemistry is an important adjunct to the histopathologic diagnosis. One of the previous 2 cases in the literature of JXG of the testis recurred after partial resection without an orchiectomy. Neither of the 2 youngest patients has experienced a local recurrence of JXG elsewhere in excess of 1 year since the original orchiectomy. The oldest patient, who was treated with partial orchiectomy, remains free of recurrence after 3 months of follow-up. Though uncommon, JXG joins several other distinctive neoplasms presenting in the infantile testis.
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ABSTRACT: Fibrohistiocytic tumors (FHTs) in children and adolescents range from the benign fibrous histiocytoma, or dermatofibroma, to a variety of intermediate and malignant neoplasms, such as dermatofibrosarcoma protruberans and high-grade undifferentiated pleomorphic sarcoma (malignant fibrous histiocytoma). Those tumors as a group are comprised of fibroblasts, myofibroblasts, and histiocytes-dendritic cells with a variably prominent inflammatory infiltrate consisting of lymphocytes and eosinophils. Dendritic cells are also a major constituent of another group of neoplasms that include Langerhans cell histiocytosis, follicular and interdigitating cell sarcomas, and juvenile xanthogranuloma. These latter tumors are considered in this discussion for the sake of differential diagnosis and their possible histogenetic relationship to FHTs. Recent studies have suggested that the relationship between the fibroblast and histiocyte in the FHTs may reflect the intrinsic capacity to transdifferentiate from one to the other morphologic and functional state. The so-called "facultative fibroblast," as a cell with fibroblastic and histiocytic properties, was discussed in the context of the fibrous xanthoma 50 years ago. Possibly the entire histogenetic concept of FHTs should be reconsidered in light of current studies.Pediatric and Developmental Pathology 01/2012; 15(1 Suppl):181-210. DOI:10.2350/11-03-1001-PB.1 · 0.86 Impact Factor
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ABSTRACT: The spectrum of testicular tumors and tumor-like lesions that affect young patients (defined for purposes of this essay as less than 20 years old) differs significantly from that in an older age group. Although germ cell tumors remain the single largest category, they are a smaller proportion than in adults. Furthermore the pathogenesis and behavior of comparably named germ cell tumors differ depending on whether or not they have developed in prepubertal or postpubertal patients. This is most apparent for the teratomas, which are almost uniformly benign in children but, with some notable exceptions, malignant in the older patients. But even the most common malignant tumor of the testis in children, the yolk sac tumor, despite its identical morphology, lacks the association with other germ cell tumor types, including intratubular germ cell neoplasia, and more aggressive behavior that typify the adult tumors. Among the sex cord-stromal tumors, the juvenile granulosa cell tumor predominates in children, mostly occurs in those under 1 year old, and, for all intents and purposes, is not seen in the postpubertal period. It has a distinctive morphology and, to date, a uniformly benign outcome. There are additional tumors in the sex cord-stromal group that are mostly seen in young patients, including the large cell calcifying Sertoli cell tumor and intratubular large cell hyalinizing Sertoli cell neoplasia. The former is sometimes associated with the Carney syndrome and, to date, all of the latter with the Peutz-Jeghers syndrome. The subtypes of lymphomas and leukemias that involve the testis in children are rare in older patients, and similar remarks pertain to the metastatic tumors, where neuroblastoma (especially) and Wilms’ tumor are most common but may be mimicked by primary tumors originating in the paratestis. The pseudoneoplastic lesion, the testicular “tumor” of the adrenogenital syndrome, is usually found in young patients and bears a strong resemblance to the Leydig cell tumor, although there are features that allow its distinction, which is important given its frequently bilateral nature and amenability to medical management through glucocorticoid administration. One of the preferential sites for embryonal rhabdomyosarcoma is the paratestis of young patients, where the spindle cell variant predominates. The melanotic neuroectodermal tumor (retinal anlage tumor) usually occurs in the first year of life, typically involves the epididymis and uncommonly metastasizes. Occasional cases of the desmoplastic small round cell tumor present in the paratestis of teenagers, and some distinctive tumor-like lesions of the paratestis may also be seen, including meconium perirorchitis and splenic-gonadal fusion (occupying both testis and paratestis). These tumors and tumor-like lesions and many others are discussed in this review with the hope it will provide the diagnostic pathologist aid in recognizing the lesions and providing some insight into their clinical significance.Seminars in Diagnostic Pathology 07/2014; 31(5). DOI:10.1053/j.semdp.2014.07.003 · 1.80 Impact Factor
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ABSTRACT: The goals of this study are to define the spectrum, incidence, and relative frequency of testicular and paratesticular lesions in a typical North American population of 6 million based on the analysis of pathology specimens. Twenty years of pathology reports from 1990 to 2009 of all testicular and paratesticular specimens were retrieved and analyzed from computerized databases of the single major pediatric hospital in the state of Indiana. We showed that the three most common benign lesions were vanishing testis, atrophy/cryptorchidism, and testis torsion, all showed left-sided predominance. The incidence and trends of the major pathology entities were also studied. Neoplasms represented roughly 10% of the total, with leukemia, rhabdomyosarcoma, teratoma, and mixed germ cell tumor occurring in decreasing frequency. Many of the neoplasms occurred at characteristic narrow age ranges, with overall interesting bimodal distribution or some with unique genetic background.Pathology - Research and Practice 04/2013; 209(7). DOI:10.1016/j.prp.2013.04.002 · 1.56 Impact Factor