[Show abstract][Hide abstract] ABSTRACT: We report a case of pleuropulmonary blastoma (PPB) occurring in a very unusual location, extrapulmonary lung tissue, as possible extralobar pulmonary sequestration. The patient was a girl aged two years and six months with respiratory symptoms. Infection of a congenital pulmonary malformation was suspected based on computed tomography findings. The operative findings revealed a partly adherent mass located between the upper and middle right lobes, and a feeding vessel. Histological analysis showed the mass to be PPB. A small portion of normal-looking pulmonary tissues was confirmed in the subcapsular area of the mass, suggesting that the tumor originated in extrapulmonary lung tissue. The patient underwent postoperative chemotherapy. No recurrence or metastasis of the PPB has occurred during the 10-year follow-up. When considering the treatment options for an extralobar pulmonary lesion, the possibility of PPB should be considered, although its occurrence may be extremely rare.
Journal of Pediatric Surgery Case Reports. 07/2014;
[Show abstract][Hide abstract] ABSTRACT: Fetal lung interstitial tumor, a newly recognized lung lesion in infants, was first reported in 2010. Here, we report the first Japanese case of fetal lung interstitial tumor which was originally diagnosed as atypical congenital cystic adenomatoid malformation/congenital pulmonary airway malformation type 3. A 7-day-old girl was referred to our hospital with respiratory distress and a left lung mass and she subsequently underwent left lower lobectomy. The specimen showed a 5 cm solid mass with a fibrous capsule. Histological examination revealed immature airspaces and interstitium, containing bronchioles and cartilage. The epithelial and interstitial cells contained abundant glycogen granules. Immunohistochemistry showed nuclear/cytoplasmic expression of β-catenin in the epithelial and interstitial cells. β-catenin gene mutations and trisomy 8 were not detected, so a neoplastic origin could not be confirmed. The histological findings were partly consistent with normal fetal lung at the canalicular stage, pulmonary interstitial glycogenosis, and congenital cystic adenomatoid malformation/congenital pulmonary airway malformation type 3. In this report, we compare the above conditions and discuss the pathogenesis of fetal lung interstitial tumor.
Pathology International 10/2013; 63(10):506-9. · 1.72 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: We evaluated the clinicopathological characteristics of pediatric sacrococcygeal germ cell tumors (SGCTs) and yolk sac tumors (YSTs) developing after sacrococcygeal teratoma (SCT) resection, and discussed the pathogenesis of sacrococcygeal YST.
We retrospectively analyzed pediatric SGCT patients attending 10 Japanese institutions.
A total of 289 patients were eligible, of which 74.6% were girls. The mean age at surgery was 7.1months. There were 194 mature and 47 immature teratomas, and 48 YSTs. YST developed after SCT resection in 13 patients (5.4% of SCTs), and was detected between 5 and 30months after resection. At initial surgery, 9 of these 13 patients were neonates, 12 underwent gross complete resection with coccygectomy, and 9 had histologically mature teratoma without microscopic YST foci. Postoperative serum alpha-fetoprotein (AFP) levels were regularly examined in 11 patients. Intervals of AFP measurement≤4months helped to detect subclinical localized YSTs for resection.
The characteristics of SGCT in Japanese children were similar with those reported in Europe or the United States. YST developed after SCT resection not only in patients with previously reported risk factors. We recommend that patients undergo serum AFP monitoring every 3months for≥3years after SCT resection.
Journal of Pediatric Surgery 04/2013; 48(4):776-81. · 1.38 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The authors report on a case of histiocytic sarcoma (HS) in a pediatric patient presenting with a solitary tumor in the cerebellum, with the aim of providing insight into primary HS in the CNS, which is especially rare. A 17-month-old Japanese girl presented with a 2-week history of progressive gait disturbance. Brain MRI revealed a 4.7 × 4.3 × 4.3-cm well-demarcated solitary mass in the right hemisphere of the cerebellum, initially suggestive of medulloblastoma, ependymoma, or anaplastic astrocytoma. On intraoperative inspection the cerebellar tumor showed intensive dural attachment and was subtotally removed. Histological and immunohistochemical findings were consistent with HS. The patient subsequently received chemotherapy, and her preoperative neurological symptoms improved. Primary HS in the CNS usually demonstrates an aggressive clinical course and is currently considered to have a poor prognosis. The possibility of this rare tumor should be included in the differential diagnosis of localized cerebellar tumors in the pediatric age group.
Journal of Neurosurgery Pediatrics 06/2012; 10(2):126-9. · 1.63 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: In this study, we report 2 pediatric cases of nuclear protein of the testis (NUT) midline carcinoma (NMC) suggestive of pulmonary origin: case 1 was a 14-year-old Japanese boy and case 2 was a 7-year-old Japanese girl. Initial symptoms of both cases were prolonged cough and chest pain, and the case 2 patient also complained of lumbago and lumbar mass due to bone metastases. Imaging studies revealed that pulmonary tumors from both patients were located at the hilar region of the lower lobe. Biopsies of the tumors showed undifferentiated carcinoma in case 1 and combined undifferentiated and squamous cell carcinoma in case 2. Despite intensive treatment with chemotherapy and radiation, progression of neither tumor was controlled, and both patients died of the tumors at 1 year (case 1) and 4 months (case 2) after onset of disease. Both tumors were diffusely positive for p63 and NUT expression and were partially positive for various cytokeratins. Reverse transcription polymerase chain reaction analysis and subsequent direct sequencing revealed that the bromodomain-containing protein 4-NUT chimeric gene was present in tumor tissue of both patients, leading to a diagnosis of NMC. The tumor cells of case 1 were also positive for thyroid transcription factor-1 expression, but those of case 2 were negative. Histologic examination of the surgically removed lung tumor of case 1 indicated that the origin of the tumor was basal cells of the bronchiolar epithelia.
The American journal of surgical pathology 03/2012; 36(3):381-8. · 4.06 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: We report a case of malignant steroidogenic tumor arising from a sacrococcygeal teratoma in a 5-year-old girl. A congenital gluteal mass and a 7-month history of precocious puberty had been noted, and a large estrogen-producing tumor in the sacrococcygeal area was found. After a biopsy, chemotherapy and tumor resection were performed, and no recurrence has been observed. The biopsy specimen showed small clusters of atypical round cells adjacent to a mature teratoma. They had large round nuclei with prominent nucleoli and abundant eosinophilic cytoplasms and were positive for vimentin, steroidogenic factor-1, inhibin α, and melan-A. Increased mitoses, vascular invasion, and necrosis were noted. The tumor was diagnosed as sacrococcygeal mature teratoma, with malignant steroidogenic tumor as somatic malignant transformation. Although several kinds of somatic malignant transformation of sacrococcygeal teratoma have been reported, to the best of our knowledge, this is the first case of malignant steroidogenic tumor arising from sacrococcygeal teratoma.
Human pathology 04/2011; 42(10):1568-72. · 3.03 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: We report a case of papillary carcinoma (PC) with extensive squamous metaplasia arising from a thyroglossal duct cyst (TDC) that required differential diagnosis from squamous cell carcinoma (SCC). An 11-year-old Japanese girl presented with a 9-month history of an anterior-midline neck mass that was clinically diagnosed as TDC. Open neck biopsy revealed nested proliferation of atypical squamous cells within the cystic structures, and SCC arising from TDC was initially suspected. Further examination, however, including immunohistochemistry, revealed the tumor to be of thyroid cell origin. The patient underwent wide local resection of the thyroglossal duct carcinoma by Sistrunk procedure and cervical lymph node dissection. Microscopically, the diagnosis was of PC with extensive squamous metaplasia and metastasis to the medial submandibular lymph node. Distinction of squamous metaplasia in PC from SCC is sometimes difficult, but has a significant effect on postoperative management.
Journal of Pediatric Surgery 04/2011; 46(4):e1-4. · 1.38 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Sialolipoma is a new variant of salivary gland lipoma that was first proposed by Nagao et al (Histopathology 2001;38:30-36). Three cases of submandibular gland sialolipoma have been previously reported in the English literature, all of which were in adults. We report an unusual case of sialolipoma of the submandibular gland in a 3-year-old boy. This is the first reported case of submandibular gland sialolipoma in a child.
Journal of Pediatric Surgery 02/2011; 46(2):408-10. · 1.38 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: There are numerous techniques of minimally invasive surgery for pediatric inguinal hernia. However, laparoscopic percutaneous extraperitoneal closure (LPEC) is one of the most simple and reliable methods. Single-incision laparoscopic surgery has recently been developed to reduce the invasiveness of traditional laparoscopy. This study aimed to assess the safety and feasibility of the single-incision approach for LPEC.
Between June 2009 and March 2010, 117 conventional LPEC and 60 single-incision LPEC (SILPEC) procedures were performed. A laparoscope was placed through a transumbilical incision. A 3-mm grasping forceps was inserted in the lower abdomen in LPEC and through the same transumbilical incision with a different entrance in SILPEC. Using the LPEC needle, the hernia sac was closed extraperitoneally. We compared the short-term outcomes of 177 children operated on using either technique.
Visualization and tissue manipulation were good in both methods. There were no complications or evidence of early recurrence. Cosmetically, SILPEC proved to be superior to LPEC because the scar resulting from surgical incision is hidden within the umbilicus.
Single-incision LPEC proved to be as successful an operative procedure as LPEC and produced excellent cosmetic results. Single-incision LPEC for inguinal hernia in children is safe and feasible.
Journal of Pediatric Surgery 12/2010; 45(12):2386-9. · 1.38 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Laparoscopic spleen-preserving distal pancreatectomy (LSDP) with conservation of the splenic vessels is gaining acceptance as a reliable treatment for selected patients with low-grade malignant tumors of the pancreas in adults. The operation requires advanced laparoscopic skills to safely divide the fine branches of the splenic vessels. Laparoscopic spleen-preserving distal pancreatectomy with conservation of splenic vessels is rarely reported in children. We describe a 12-year-old girl with solid pseudopapillary tumor in the body of the pancreas that was successfully treated with LSDP, preserving the splenic vessels. The postoperative course was uneventful, and the functional and aesthetic results were satisfactory. Laparoscopic spleen-preserving distal pancreatectomy with conservation of splenic vessels may be a safe and feasible treatment option for children with pancreatic disease.
Journal of Pediatric Surgery 07/2010; 45(7):1525-9. · 1.38 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Choledochal cyst causes liver fibrosis, the extent of which varies with each case. Liver damage seldom progresses to cirrhosis, but when it does, it is generally irreversible. We report an infantile case of liver cirrhosis associated with choledochal cyst in which complete clinical resolution was achieved by surgery. Pancytopenia caused by splenomegaly, massive ascites, hypoalbuminemia, and coagulation abnormality that were observed during the early postoperative period had disappeared within 4 months after surgery. Needle liver biopsy performed at 1 year after surgery revealed marked improvement in liver fibrosis.
Journal of Pediatric Surgery 05/2010; 45(5):e11-4. · 1.38 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: We report 2 cases of growth spurt-related recurrence after Nuss procedure. Each of the 2 cases underwent bar insertion at the age of 6 and 11 years, respectively. The support bar was removed 2 years later followed by severe redepression during the growth spurt. One patient underwent redo Nuss procedure elsewhere. The other patient was diagnosed as idiopathic precocious puberty and is in treatment. The possibility of growth spurt-related recurrence must be explained to those who undergo early correction before surgery, and follow-up is mandatory at least until patients' puberty is over.
Journal of Pediatric Surgery 09/2009; 44(8):E13-6. · 1.38 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Although various urologic complications have been reported after abdominoperineal pull-through and posterior sagittal anorectoplasty for the treatment of high-type imperforate anus, reports regarding complications after laparoscopically assisted anorectoplasty (LAARP) are surprisingly rare. Here, we discuss the potential complications of LAARP.
A retrospective study was conducted of 24 patients treated with LAARP from 2000 to 2006. The clinical and operative records were reviewed. Of the 24 patients, 18 were evaluated postoperatively by screening magnetic resonance imaging (MRI).
The 24 participants are composed of patients with rectoprostatic urethral fistula (n = 15), rectal agenesis (n = 3), rectovesical fistula (n = 2), rectovaginal fistula (n = 2), and cloaca (n = 2). Defecatory function after LAARP was satisfactory. None of the patients had dysuria or urinary infection postoperatively. Cystic formations posterior to the urethra were demonstrated in 9 of the 18 cases examined by MRI. Postoperative voiding cystourethrography failed to demonstrate the lesion in 6 of 9 patients. The types of imperforate anus in this subgroup were rectoprostatic urethral fistula (n = 7), rectovesical fistula (n = 1), and rectal agenesis without fistula (n = 1). Average cyst diameter was 22 +/- 19 mm. Two patients with large cysts (62 and 42 mm) underwent surgical resection.
Although satisfactory fecal continence could be achieved by LAARP, we experienced 2 cases with a large residual fistula that required surgical resection. In addition, screening MRI demonstrated the presence of cystic formations in 9 of 18 patients. We recommend that MRI be performed routinely during follow-up of patients treated with LAARP.
Journal of Pediatric Surgery 02/2009; 44(1):278-81. · 1.38 Impact Factor