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Purpose: 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., Methods: We retrospectively analyzed pediatric SGCT patients attending 10 Japanese institutions., Results: 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., Conclusions: 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., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Objective: Prepubertal testicular tumors are rare and fundamentally distinct from adult testicular tumors. We reviewed our 11-year experience in a single medical center of China., Material and Methods: This study reports the clinical characteristics, histopathologic diagnosis, treatment methods, and outcome in a series of 63 prepubertal boys who were treated between 1997 and 2008., Results: A total of 63 primary prepubertal testicular and paratesticular tumors were identified. The median age at presentation was 11 months. Of these tumors, 27 (42.9%) were mature teratomas, 5 (7.9%) were immature teratomas, 21 (33.3%) were yolk sac tumors, 4 (6.3%) were epidermoid cyst, 2 (3.2%) were Leydig cell tumors, 1 (1.6%) was a mixed malignant germ cell tumor, and 3 (4.8%) were paratesticular tumors. The most common clinical presentation (95.2%) was a painless scrotal mass or swelling. Forty-eight tumors were treated with radical inguinal orchiectomy, and 15, with a testis-sparing procedure. Follow-up was available in 59 cases, range from 4 to 128 months (median, 50 months). One patient with yolk sac tumor had recurrence and progression to metastasis at the end of 4 months after surgery. Other patients were disease free at last follow-up., Conclusions: Most of the prepubertal testicular lesions were benign, and the most common histologic subtype was teratoma. Our experience with testis-sparing procedures supports the current trends that less invasive treatment should be performed for benign lesions. This study confirms the excellent cure rates obtained in children with prepubertal testicular tumors., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Yolk sac tumor (YST) is a germ cell neoplasm usually arising from the gonads. Extragonadal YST has been reported in the mediastinum, retroperitoneum, liver, and pelvis. Primary YST of the common bile duct (CBD) has not been previously reported. We describe an 11-month-old girl with a YST arising from the CBD who presented with obstructive jaundice. Imaging studies revealed an intraluminal CBD mass with intrahepatic biliary dilatation and extraluminal infiltration of the head of the pancreas. A complete resection was carried out, and postoperative chemotherapy with cisplatin, etoposide and bleomycin was administered., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Yolk sac tumors (YSTs) represent 3% of malignancies in childhood and most commonly arise in the gonads. Hepatic YSTs are rare and previous reported cases were treated with resection and/or chemotherapy with mixed results. We present the first case of an unresectable hepatic YST in a 2-year-old boy treated successfully with liver transplantation.

Intestinal obstruction in a 2-year-old girl with a histologically proven diagnosis of retroperitoneal yolk sac carcinoma developed after the second course of anticancer chemotherapy. Nonoperative treatment was not effective. Because the patient had fallen into a state of chemotherapy-induced myelosuppression, surgery was ruled out. Thus, hyperbaric oxygen therapy was the next treatment of choice. It was performed twice under hyperbaric oxygen conditions at 2.8 atmospheric pressure for 111 minutes. After the procedure, her general status recovered well. The air-fluid level disappeared on the radiograph, and no adverse effects were observed. Later, a surgical removal of the primary tumor was performed successfully, but an intestinal resection was not required. This is the first instance in which we performed hyperbaric oxygen therapy on a child in the management of an intestinal obstruction. Based on the successful outcome in this case, hyperbaric oxygen therapy is suggested to be a useful adjunct to nonoperative therapy for intestinal obstruction when a patient's overall state does not allow operative intervention.

Two cases of vaginal endodermal sinus tumor (EST), a rare pediatric malignancy, were managed with conservative surgery followed by adjuvant chemotherapy. The first case involved a 9-month-old girl with EST of the vagina, who was treated with a partial vaginectomy and VAC regimen (vincristine, actinomycin D, cyclophosphamide) during a 2-year period. The serum alpha-fetoprotein (AFP) level returned to normal after excision of the tumor, and it remained normal throughout the treatment period. There was no evidence of disease 30 months after diagnosis. The second case involved an 8-month-old girl with EST of the vagina, who was treated with local excision of the mass through a vaginotomy. The VAC regimen was administered, but the serum AFP level remained elevated. A follow-up abdominopelvic computed tomography scan, taken 4 months after the operation, showed local recurrence of the tumor. The VAC regimen was then changed to a BEP regimen (bleomycin, etoposide, cisplatin). The serum AFP level returned to normal after 2 courses of the new regimen, and no tumor was visible on the follow-up magnetic resonance imaging study. For vaginal EST, primary conservative surgery and adjuvant chemotherapy are attractive measures to preserve both reproductive and sexual function. The extent of conservative surgery requires at least a partial vaginectomy. Simple tumor excision may not be adequate to achieve cure or to prevent local recurrence, even with adjuvant chemotherapy. The serum AFP level is useful for diagnosing and monitoring vaginal EST in the infant.