1/197. Ovarian endometrioid-like yolk sac tumor treated by surgery alone, with recurrence at 12 years. We describe the case of a stage Ia endometrioid-like yolk sac tumor (YST) of the ovary, which was originally misdiagnosed as a malignant struma ovarii and not treated with adjuvant chemotherapy. After 12 years, a contralateral dermoid cyst was excised along with a small omental nodule of partially necrotic and calcified endometrioid-like YST. No tumor was detected in several other biopsy specimens, and a peritoneal lavage was negative for tumor cells. Since there was no evidence of remaining tumor and the serum alpha-fetoprotein (AFP) level was normal after the second operation, the patient was followed. Serial serum AFP levels remained normal for 4 months. At a second-look laparotomy after 4 months, a small tumor nodule was removed from the cul-de-sac. Postoperatively, the patient received three cycles of BEP chemotherapy. The long disease-free interval after the first operation in spite of the presence of occult spread to the omentum and to the pouch of Douglas in this case indicates that some endometrioid-like YSTs may have an indolent course. The present case underscores the importance of careful surgical staging and of long-term follow-up in cases of primitive germ cell tumors of the ovary. ( info) |
| endodermal sinus tumor (EST) is a rare neoplasm which usually arises in the testis or ovary. But extragonadal EST is well recognized and may arise in a wide array of sites such as the mediastinum, vagina, and brain. We report a case of primary EST of the omentum and to our knowledge it is the first case of omental EST in the literature. A 45-year-old woman with a history of abdominal distension was treated with total abdominal hysterectomy with bilateral salpingo-oophorectomy and infracolic omentectomy, followed by four cycles of BEP (bleomycin, etoposide, cisplatin) combination chemotherapy. Microscopically, the tumor exhibited typical patterns of EST and stained for alpha-fetoprotein and cytokeratin. She has remained free of disease for 10 months of follow-up. According to previous studies, debulking surgery and BEP chemotherapy were used as primary therapy with good results. The subject of extragonadal EST is reviewed, and the possible histogenesis of this tumor at such a rare site is discussed. ( info) |
3/197. Primary yolk sac tumour of the liver in adulthood. Primary yolk sac tumour of the liver is exceedingly rare. A 28 year old woman presented with a cystic liver mass and a markedly raised serum alpha-fetoprotein concentration. She underwent a partial hepatectomy for a suspected hepatocellular carcinoma but histological examination of the tumour revealed the classical morphological and immunohistochemical features of a yolk sac tumour. There was no evidence of an extrahepatic primary source. review of this case, together with the six previously reported adult cases of primary yolk sac tumours of the liver, revealed several features of the tumour that may aid differentiation from hepatocellular carcinoma, with potential therapeutic implications. ( info) |
4/197. Intrapericardial yolk sac tumor associated with acute myocarditis. An occult intrapericardial yolk sac tumor occurred in a 3-year-old girl with a fatal outcome. At autopsy, a 5.5-cm mass surrounded the base of the heart and compressed the left atrium posteriorly. Histologically, the tumor was a pure yolk sac tumor. Postmortem chemical analyses of the blood revealed an alpha-fetoprotein level greater than 7000 microg/L. Acute myocarditis of both ventricles was also found. ( info) |
5/197. Mixed malignant germ cell tumor of the fallopian tube. Immature teratomas of the fallopian tube are exceedingly rare with only three reported cases in the English literature. Reported here is a case of primary mixed malignant germ cell tumor of the fallopian tube composed of immature teratoma and yolk sac tumor. ( info) |
6/197. Intracranial germ cell tumors in children with and without down syndrome. PURPOSE: Two Chinese children with down syndrome affected by intracranial germ cell tumors are described. Because they represent two of eight affected patients in the current series from 1990 to 1996, it is postulated that such occurrence may be more than a coincidental event. patients AND methods: Two children with down syndrome developed germ cell tumors in atypical intracranial sites that affected basal ganglion and cerebellum. The pathology showed germinoma and yolk sac tumor, respectively. These were treated by radical surgical resection and chemotherapy with cisplatin, etoposide, and bleomycin, but without radiotherapy. RESULTS: One patient survived 3 years without radiologic evidence of tumor. The other died from infective complications caused by severe myelosuppression after chemotherapy. CONCLUSIONS: Subtle neurologic manifestations in developmentally handicapped patients with intracranial space-occupying lesions could result in delayed diagnosis. Children with down syndrome suffering from brain tumors may have a higher chance for germ cell tumors. Assay for alpha-fetoprotein and beta-human chorionic gonadotrophin could hasten diagnosis in some cases. This observation and review of literature suggest an increased risk of developing intracranial germ cell tumors in subjects with down syndrome. ( info) |
7/197. Intracardiac yolk sac tumor in an infant girl. We report about a two and a half year old girl with a primary yolk sac tumor (YST) of the heart, located in the interventricular septum. The girl was admitted to the hospital with a systolic cardiac murmur. echocardiography revealed a smoothly surfaced tumor which based on the interventricular septum below the aortic valve and filled out more than half of the left ventricular cavity. Diagnosis of a yolk sac tumor was established on a frozen section of a biopsy taken at surgery and confirmed by the examination of the resected material. Excessively elevated serum alpha-fetoprotein (AFP) levels contributed to this diagnosis. Follow up examinations of the patient including computed tomography (CT) of head, thorax and abdomen, did not indicate an extracardiac tumor manifestation. We found no previous report of a primary intracardial yolk sac tumor in the literature. ( info) |
| BACKGROUND: Extragonadal yolk sac tumors of the gastrointestinal tract are extremely rare neoplasms. Their greater rarity compared with other extragonadal yolk sac tumors suggests that different pathogenetic mechanisms could be involved according to the site of origin. This report describes a case of a combined yolk sac tumor and adenocarcinoma that arose in a gastric stump in a man age 61 years 43 years after he underwent distal gastric resection and gastrojejunostomy (Billroth II operation) for a benign duodenal ulcer. The coexistence of an adenocarcinomatous component with the yolk sac component suggests that the two histologic patterns may represent distinct phenotypes arising from a common mucosal epithelial cell. methods: Immunohistochemical and molecular techniques were used to define the mutation pattern of p53 in both components of the tumor. RESULTS: Single-strand conformation polymorphism and sequencing analyses demonstrated the same pattern of p53 mutation in the adenocarcinomatous and yolk sac tumor components. CONCLUSIONS: This finding suggests that the two tumors could have been derived from the same cellular clone and supports the hypothesis that the two components represented a heterogeneous differentiation of the same tumor. ( info) |
9/197. Postchemotherapy retroperitoneal residual mass in infantile yolk sac tumor. BACKGROUND: Persistent postchemotherapy retroperitoneal residual mass with normalization of alpha-fetoprotein (AFP) in infantile yolk sac tumor is rare. methods/RESULTS: A 38-month-old boy with recurrent yolk sac tumor was treated with cisplatin-based combination chemotherapy. After chemotherapy, the retroperitoneal lymph node metastasis, 7 x 6 cm in size, decreased to 2 x 2 cm. serum AFP levels returned to normal. The retroperitoneal residual mass was resected and histologically showed complete necrosis without viable cancer cells. CONCLUSION: The patient has remained free of disease for 36 months after operation. ( info) |
10/197. Ovarian hepatoid yolk sac tumours: morphological, immunohistochemical and ultrastructural features. AIM: The clinicopathological, immunohistochemical and ultrastructural features of two ovarian hepatoid yolk sac tumours (H-YST) from our files are reviewed. methods AND RESULTS: Using avidin-biotin-peroxidase complex technique, the immunoprofile of these tumours was compared to that of a classic yolk sac tumour and to that previously reported for hepatocellular carcinomas. The clinicopathological and morphological features of our cases are similar to the seven previously reported ovarian cases. This rare germ cell tumour occurs in young females (mean age = 17.6 years) and presents most commonly with abdominal pain and a large ovarian mass (average size = 140 mm). Histologically, the tumours display a striking resemblance to hepatocellular carcinoma. The absence of an associated typical pattern of yolk sac tumour or other germ cell neoplasm may make it difficult to recognize the germ cell origin of this lesion. Our cases demonstrated positive staining for alpha-fetoprotein and alpha-1-antitrypsin. In addition, there was immunoreactivity with polyclonal carcinoembryonic antigen (CEA) antiserum in a canalicular pattern, focal staining for inhibin, oestrogen and progesterone receptors and absence of immunoreactivity for CK7 that contrasts with the immunophenotype of a usual yolk sac tumour. CONCLUSIONS: Ovarian H-YST and hepatocellular carcinoma share a similar immunoprofile. Ovarian H-YST is a highly aggressive tumour, most patients exhibit recurrence or die of disease within 2 years of diagnosis. ( info) |