Pregnancy following treatment of endodermal sinus tumor of the ovary with combination chemotherapy, including cis-platinum

A 16-year-old woman with endodermal sinus tumor of the ovary underwent conservative surgical debulking and combination chemotherapy and had a recurrence documented by a rise in alpha-fetoprotein. She had a complete response to a second chemotherapy regimen, including cis-platinum. Subsequently, she became pregnant. The case emphasizes the management of endodermal sinus tumor, including conservative surgery, combination chemotherapy, and monitoring serum alpha-fetoprotein levels. This is the second report of pregnancy following cis-platinum administration.     

Primary yolk sac tumor (endodermal sinus tumor) of the vulva: case report and review of the literature

INTRODUCTION: Endodermal sinus tumor (EST) or primary yolk sac tumor (YST) of the vulva is extremely rare and a highly malignant germ cell tumor. Only nine cases of vulvar YST have been reported to the world literature to date. We present the tenth case of endodermal sinus tumor of the vulva. CASE: A 32-year-old white virgin presented with a 3.5 cm right labial mass without any other signs or symptoms. Excisional biopsy showed YST with a predominantly solid pattern. Unilateral hemivulvectomy with bilateral inguinal lymphadenectomy was performed. Six months after surgery there was a recurrence. She was treated with three courses of the BEP regimen (bleomycin, etoposide, cisplatin). The patient refused to take any further treatment including radiotherapy. The serum alpha-fetoprotein (AFP) was not elevated at the initial diagnosis however it was elevated during recurrence. The patient is alive with the disease 42 months after the first appearance of the vulvar mass.     

Combination chemotherapy in the management of ovarian germ cell malignancies

Twenty-four patients with ovarian germ cell malignancies received combination chemotherapy in the present series. Fifteen patients received intensive vincristine, actinomycin-D, and cyclophosphamide therapy for 12 to 18 courses, and 13 are alive and free of disease 34 to 86 months later. One patient with a stage III pure endodermal sinus tumor and one patient with a stage III mixed germ cell tumor composed predominantly of endodermal sinus tumor elements failed vincristine, actinomycin-D, and cyclophosphamide therapy, but each transiently responded to cis-diamminedichloroplatinum, vinblastine, and bleomycin therapy. Nine patients subsequently were treated on a new protocol that used the intensive vincristine, actinomycin-D, and cyclophosphamide regimen for five to six courses for all stage I ovarian germ cell malignancies and cis-diamminedichloroplatinum, vinblastine, and bleomycin therapy for advanced stage tumors containing endodermal sinus tumor. Each patient on the new protocol is alive and free of disease 14 to 26 months later. Short-term intensive vincristine, actinomycin-D, and cyclophosphamide therapy is recommended for all stage I ovarian germ cell malignancies requiring adjuvant chemotherapy. Preservation of ovarian and reproductive function is appropriate in the present group of patients. Vincristine, actinomycin-D, and cyclophosphamide therapy is also recommended for biomarker negative advanced stage ovarian germ cell malignancies. Cis-diamminedichloroplatinum, vinblastine, and bleomycin therapy is recommended for advanced stage biomarker positive ovarian germ cell malignancies. Serial alpha-fetoprotein (AFP) titers accurately reflect the status of endodermal sinus tumor elements and may be used as a guide to discontinue treatment for patients with pure endodermal sinus tumor malignancies, obviating the need for second-look surgery.     

Ovarian endodermal sinus tumor associated with pregnancy--a case report

An ovarian endodermal sinus tumor was found in a 29-years old patient with a 32-week intrauterine gestation. She received caesarean section, conservative surgery and three courses of combination chemotherapy. She died 6 months after the diagnosis.     

Fertility-preserving treatment for patients with malignant germ cell tumors of the ovary

AIM: The aim of this study was to investigate whether fertility preservation influences the clinical outcome in patients with malignant germ cell tumors of the ovary (MGCTO). METHODS: A case study analysis was performed on patients with MGCTO treated at Kurume University Hospital between 1986 and 2004. Thirty-five patients were included in the study, 14 with immature teratoma, 11 with dysgerminoma, eight with endodermal sinus tumor, and two with mixed germ cell tumor. Twenty-three patients had International Federation of Gynecology and Obstetrics stage I (Ia, 11; Ib, 2; Ic, 10), one had stage II, seven had stage III, and four had stage IV disease. RESULTS: Five patients with stage III or IV disease received radical surgery. Thirty patients underwent conservative surgery. As the adjuvant treatment, 30 patients received chemotherapy, while five patients did not receive any chemotherapy. The overall survival rate was 97.1%. One patient died of the disease. She was 13 years old with a stage IV endodermal sinus tumor. Twelve have attempted conception, and eight have achieved at least one pregnancy (66.7%). CONCLUSIONS: Irrespective of the stage of the disease, conservative surgery and adjuvant chemotherapy for MGCTO can achieve a favorable outcome in terms of survival and fertility.     

Endodermal sinus (yolk sac) tumor of the vulva in a pregnant female

An unusual case of endodermal sinus (yolk sac) tumor arising in the vulva and presenting during pregnancy is reported. The tumor recurred within 4 months of surgery. Despite subsequent wide excision and chemotherapy, the patient succumbed to her illness due to widespread pulmonary and pleural involvement within 11 months of onset of symptoms.     

Recent management of malignant ovarian germ cell tumors: a study of 34 cases.

OBJECTIVE: To review the outcome of the treatment in patients with malignant ovarian Germ cell tumors with respect to survival and surgical management at a single institution during 1990-1996. METHODS: Thirty-four patients with malignant ovarian Germ cell tumors were studied retrospectively for their surgical management. Fourteen patients had pure dysgerminoma, 11 endodermal sinus tumor, 6 immature teratoma, and 3 mixed Germ cell tumors. Nine patients had stage IA, 8 stage IC, 2 stage IIC, 8 stage III, 3 stage IV, and 4 referred patients with recurrent diseases. RESULTS: Nineteen patients underwent primary conservative surgery, 11 had primary nonconservative surgery. Twenty-two patients were treated with chemotherapy (BEP or EP or PVB regimen). Five patients with pure dysgerminoma received adjuvant radiotherapy. Persistent remission was achieved in 26 patients. Two patients (7.4%) had recurrence after remission. Seven patients had died of the diseases. Patients with complete clinical remission did not undergo second-look surgery. The overall survival was 78.8%, 100% for immature teratoma, 84.6% for pure dysgerminoma, 72.8% for endodermal sinus tumor, and 33.3% for mixed Germ cell tumors, with median follow-up time 31 (3-93) months. CONCLUSION: Patients with limited diseases regardless of histologic types can be safely managed by unilateral salpingo-oophorectomy followed by, if indicated, 3-4 courses of cisplatin-based chemotherapy. For advanced diseases, conservative surgery is advisable in patients with endodermal sinus tumor.     

Female adnexal tumor of probable wolffian origin: a clinicopathological case report and a possible new treatment

Female adnexal tumors of probable wolffian origin (FATWOs) are rare tumors arising in the broad ligament from the remnants of the mesonephric duct. We report a case of recurrent disease. A 15-year-old girl who presented with a painful pelvic mass underwent a laparotomy with tumor resection. Pathology findings confirmed a FATWO. The tumor recurred within 2 years and was treated with multiple chemotherapy regimens, including a platinum-based drug, and surgery for progressive disease. The tumor was positive for c-kit oncogene (CD 117). Gleevac therapy, a tyrosine kinase inhibitor, was prescribed, and she developed severe persistent lower abdominal pain 2 months later. She underwent a hysterectomy and debulking of retroperitoneal masses. Pathology showed evidence of tumor necrosis, suggesting a possible beneficial effect, and she was recommenced on Gleevac in an effort to prevent recurrences. She is currently asymptomatic, without evidence of disease 10 months after surgery, continuing on Gleevac therapy. FATWOs are very rare tumors. Most cases are benign but have the potential to recur and metastasize. There is limited knowledge about the optimal treatment for this neoplasm. Our patient's favorable response to Gleevac therapy supports the concept of targeted molecular therapy in patients with c-kit-positive FATWO tumors.     

Endodermal sinus tumor of the vulva: successfully treated with high-dose chemotherapy

Endodermal sinus tumors (EST) of the lower female genital tract are uncommon malignancies. Most of these tumors are found in the vagina or in the cervix. Only seven cases of EST involving the vulva are reported. We report an eighth case of vulvar EST. The patient recurred and was successfully treated with high-dose chemotherapy and peripheral blood cell transplantation. Because of their location, an early diagnosis of vulvar EST should be possible. Resection of the lesion by using a modified radical vulvectomy followed by cisplatin-based chemotherapy seems the best treatment for these tumors today. Our patient was initially treated with the help of local surgery and adjuvant chemotherapy consisting of three courses of etoposide and cisplatin. Sixteen months after the first diagnosis, pleural and lung metastases were diagnosed. Second-line chemotherapy followed by high-dose chemotherapy with autologous peripheral blood stem cell support was administered after resection of the lung metastases and biopsy of the pleural metastases. There is no evidence of disease 40 months after the diagnosis of the lung and pleural metastases, and 56 months after the primary diagnosis.     

Ovarian gonodoblastoma with yolk sac tumor in a young 46, XX female: case report

BACKGROUND: Gonodoblastomas with ovarian germ cell tumors (OGCTs) other than dysgerminoma are very unusual. This is the first case report of a pure endodermal sinus tumor of the ovary with gonodoblastoma in a 46 XX, female. CASE: A 19-year-old female was admitted to our hospital with an abdominal mass and pain. She had elevated levels of CA-125 and AFP. Fertility sparing surgery was undertaken and revealed a Stage IA endodermal sinus tumor and gonodoblastoma in the same ovary. The patient refused chemotherapy and was followed-up for six months without disease. CONCLUSION: Gonodoblastomas may occur in conjunction with OGCTs. Tumor markers and immunohistochemical examination may help in the diagnosis of these OGCTs with gonodoblastoma.     

阴道内胚窦瘤的诊断和治疗——附二例报告

目的 探讨阴道内胚窦瘤的临床特点及化疗效果。方法 对２例阴道内胚窦瘤进行临床报告及分析。结果 阴道内胚窦瘤主要发生在儿童,临床表现主要为阴道出血和息肉状易脆的阴道肿物。血清甲胎蛋白（α－ＦＰ水平化疗前均异常升高,病理诊断主要依靠Ｓｃｈｉｌｅｒ－Ｄｕｒａｌ小体和α－ＦＰ染色阳性。２例口才分别对顺铂、长春新碱、博莱霉素（ＰＶＢ）方案和顺铂、鬼臼乙叉甙、博莱霉素（ＰＥＢ）方案化疗均有良好反应,２￣３个疗     

Second-look laparotomy in the management of malignant germ cell tumors of the ovary

From 1970 to 1985, 53 patients with malignant nondysgerminomatous germ cell tumors of the ovary underwent second-look laparotomy after initial surgery and combination chemotherapy. Twenty-two patients had immature teratoma, 15 had endodermal sinus tumor, 15 had mixed germ cell tumor, and one patient had embryonal carcinoma. Thirty-one of the neoplasms were stage I, four were stage II, 17 were stage III, and one was stage IV. Two patients received a combination of actinomycin-D, 5-fluorouracil, and cyclophosphamide; four patients received vinblastine, bleomycin, and cisplatin; 44 patients received vincristine, actinomycin-D, and cyclophosphamide; and three patients received a combination of the last two regimens. Second-look findings were negative in 52 patients and positive in one patient who was subsequently salvaged with further chemotherapy. One patient with stage I endodermal sinus tumor relapsed nine months after a negative second-look laparotomy and died. Two patients with negative findings subsequently died of leukemia. Of 53 patients undergoing second-look laparotomy, three are dead (one of cancer and two of leukemia), and 50 patients are surviving without disease. Although the precise role of second-look laparotomy in patients with malignant germ cell tumors is yet to be established, possible indications are discussed.     

Endodermal sinus tumor of the ovary: a paradoxical response to chemotherapy

A patient with ovarian endodermal sinus tumor metastatic to the liver responded unusually to chemotherapy. She experienced histologically documented regression of all intraperitoneal disease with the exception of the liver metastases when given vinblastine, bleomycin, and cisplatin. She eventually died of liver failure. A review of patients with ovarian endodermal sinus tumor who either presented with initial liver metastases or had recurrent disease in the liver leads us to conclude that the liver is a preferential site for spread, that liver metastases carry an ominous prognosis, and that failure to see prompt liver metastatic tumor response after systemic chemotherapy should signal a need for a more aggressive therapeutic approach to the liver lesions.     

Bleomycin, vinblastine, and cis-platinum in the treatment of advanced endodermal sinus tumor

The endodermal sinus tumor has traditionally been associated with an exceedingly poor prognosis. Three women with advanced pure endodermal sinus tumors were treated with a combination of bleomycin, vinblastine, and cis-platinum (VBC). Serum alpha-fetoprotein levels were monitored for all patients during and after therapy, and in each case the alpha-fetoprotein returned to normal range, correlating with complete clinical remission. Second-look laparotomy was negative for tumor in each case. Two patients have had no overt sign of recurrence 12 months after cessation of therapy. The third patient had post-treatment elevation of alpha-fetoprotein levels, and eventually was found to have recurrent tumor 6 months after chemotherapy was stopped. The VBC combination, previously found effective in testicular germ cell tumors, is also effective in ovarian germ cell tumors.     

Tumor del seno endodérmico ovárico

The endodermal sinus ovarian tumor is a malignant tumor derived from germinal cells. Usually one appears in young women and with very fast growth. Characteristically it makes debut with elevated levels of alfafetoprotein (AFP). Its classic badly prognosis at the present time, has improved and is possible to obtain complete remissions after radical surgery an chemotherapy. This is the case of a younger patient with an endodermic sinus tumour ovarian that was presented how an acute abdominal pain because of ovarian torsion, first treated with conservative surgery and after the recurrence with multi-agent chemotherapy and radical surgery. Actually the patient is live and free of disease.     

Endodermal sinus tumor of the infant vagina

A case of endodermal sinus tumor of the infant vagina is reported with long-term survival after successful therapy by surgery, chemotherapy, and radiation. The previous 26 reported cases are reviewed and the problems of therapy and long-term management are discussed. Therapy of these tumors should be monitored by alpha-fetoprotein radioimmunoassay.     

Clear Cell Carcinoma of the Ovary: A Distinct Histologic Type with Poor Prognosis and Resistance to Platinum-Based Chemotherapy in Stage III Disease

Between 1982 and 1992, 24 women with Stage III clear cell ovarian cancer were identified from the tumor registry. Thirty-four women with Stage III papillary serous tumors treated between 1987 and 1989 were used as a comparison. All patients underwent cytoreductive surgery followed by conventional platinum-based chemotherapy. In the women with clear cell histology, nine (37.5%) had endometriosis in the surgical specimen compared with one (3%) in the papillary serous group (P= 0.002). Ten women (42%) with clear cell histology experienced a thromboembolic event during the course of treatment, compared to six (18%) in the papillary serous group (P= 0.05). In the group with clear cell histology, overall, 70% of women had progressive disease. Fifty-two percent experienced clinical progression while receiving platinum-based chemotherapy. In addition, four patients were found to have progressive disease at second-look laparotomy. Only two patients had a pathologic complete response. In the group with papillary serous histology, 29% overall had progressive disease while on chemotherapy (P= 0.005). The median survival for the women with clear cell histology was 12 months compared to 22 months for those with papillary serous (P= 0.02). For women with clear cell histology, univariate analysis was used to evaluate prognostic factors. Age less than 50 was a poor prognostic factor (P= 0.045). The presence of endometriosis, thromboembolic event, or optimal cytoreduction were not prognostic factors (P= 0.67,P= 0.34,P= 0.39). Patients with advanced clear cell ovarian cancer have a poor response to conventional platinum-based chemotherapy and overall prognosis is poor.     

Nondysgerminomatous tumors of the ovary treated with cisplatin, vinblastine, and bleomycin: long-term results.

Twenty-four consecutive patients with nondysgerminomatous germ cell tumor of the ovary were treated after surgery with cisplatin, vinblastine, and bleomycin (PVB regimen). The cycle was repeated every 3 weeks for three to five courses. Fourteen patients had endodermal sinus tumor, and 10 had mixed germ cell tumors. Stage of disease (FIGO, 1986) was as follows: stage I, 6; stage IIc-IV, 17; and recurrence, 1 patient. All patients were monitored by alpha-fetoprotein and human chorionic gonadotropin. Only 1 patient had received previous chemotherapy. All 5 patients without residual disease and with negative marker levels in which PVB was used as adjuvant treatment were free of disease for a median duration of 59 months from the start of PVB. Of the 19 patients with measurable disease (evident disease or positive marker levels), complete remission was obtained in 16 (84%), but 5 of these relapsed. Therefore, treatment with PVB failed in 8 out of 19 patients (42%) with measurable disease. Toxicity was evident, but no patient died of it. Menses were regular in 11 patients whose initial surgery was conservative. PVB regimen is an effective but not a satisfactory treatment. The considerable failure rate of PVB treatment suggests the investigation of other regimens.     

Malignant fibrous histiocytoma of the vulva: a case report

Background: Primary sarcomas of the vulva are rare tumors that account for 1.8–3% of all vulvar malignancies. Malignant fibrous histiocytoma occurs infrequently on the vulva but nonetheless is the second most frequent sarcoma of this region. The purpose of this report is to review the diagnosis and therapy of this exceedingly rare tumor. Case: A 72-year-old woman was presented with a vulvar mass that was ultimately found to be a vulvar malignant fibrous histiocytoma. After surgical excision and 9 months of initial diagnosis, she returned with rapid localized progression and pulmonary metastases. Conclusion: Malignant fibrous histiocytoma arising in the vulva represents an example of vulvar sarcomas. Since the diagnostic criteria and natural history of this tumor still remain poorly defined, further case studies may be helpful to elucidate these issues as well as the optimal therapeutic approach of this heterogeneous group of malignancies.     

Endodermal sinus tumor of the ovary: the Hacettepe University experience

OBJECTIVE: The purpose of this study was to evaluate the treatment regimens used for patients with endodermal sinus tumors (EST), and also to examine the prognostic value of surgicopathological variables. STUDY DESIGN: Twenty-two patients treated for pure EST, and seven patients who had mixed germ cell tumors with EST components were included. Initial surgery consisting of surgical staging to achieve optimal debulking was the principal mode of therapy. Data were obtained from patients' files, a special gynecologic oncology database, and pathology records. RESULTS: The median age at the time of diagnosis was 18 (range 8-45). Sixteen patients had stage I (55%), and 13 (45%) had stage II-IV disease. As an adjuvant therapy 18 patients (62%) received platin-based chemotherapy, three patients (10%) had non-platin-based chemotherapy, four patients (14%) had radiotherapy, and four patients (14%) had combined radiotherapy plus non-platin-based chemotherapy. The 5-year disease-free and overall survival rates were 47 and 48%, respectively. Survival rates were dismal in patients with stage II-IV disease (P < 0.001). Platin-based chemotherapy achieved significant survival benefit (P = 0.03 and P < 0.001, respectively). Fertility-saving surgery had an overall survival no worse than those who underwent more extensive surgery. There was no significant survival difference with respect to age, histology, and tumor size. CONCLUSION: Fertility-sparing surgery with a postoperative platin-based combination chemotherapy should be the selected mode of treatment.