A phase II trial of bevacizumab and rucaparib in recurrent carcinoma of the cervix or endometrium.

OBJECTIVE: The aim of this study was to examine the tolerability and efficacy of combination bevacizumab rucaparib therapy in patients with recurrent cervical or endometrial cancer. PATIENTS & METHODS: Thirty-three patients with recurrent cervical or endometrial cancer were enrolled. Patients were required to have tumor progression after first line treatment for metastatic, or recurrent disease. Rucaparib was given at 600 mg BID twice daily for each 21-day cycle. Bevacizumab was given at 15 mg/kg on day 1 of each 21-day cycle. The primary endpoint was efficacy as determined by objective response rate or 6-month progression free survival. RESULTS: Of the 33 patients enrolled, 28 were evaluable. Patients with endometrial cancer had a response rate of 17% while patients with cervical cancer had a response rate of 14%. Median progression free survival was 3.8 months (95% C·I 2.5 to 5.7 months), and median overall survival was 10.1 months (95% C·I 7.0 to 15.1 months). Patients with ARID1A mutations displayed a better response rate (33%) and 6-month progression free survival (PFS6) rate (67%) than the entire study population. Observed toxicity was similar to that of previous studies with bevacizumab and rucaparib. CONCLUSIONS: The combination of bevacizumab with rucaparib did not show significantly increased anti-tumor activity in all patients with recurrent cervical or endometrial cancer. However, patients with ARID1A mutations had a higher response rate and PFS6 suggesting this subgroup may benefit from the combination of bevacizumab and rucaparib. Further study is needed to confirm this observation. No new safety signals were seen.

Unilateral inguinofemoral lymphadenectomy in patients with early-stage vulvar squamous cell carcinoma and a unilateral metastatic sentinel lymph node is safe.

OBJECTIVE: Optimal management of the contralateral groin in patients with early-stage vulvar squamous cell carcinoma (VSCC) and a metastatic unilateral inguinal sentinel lymph node (SN) is unclear. We analyzed patients who participated in GROINSS-V I or II to determine whether treatment of the contralateral groin can safely be omitted in patients with a unilateral metastatic SN. METHODS: We selected the patients with a unilateral metastatic SN from the GROINSS-V I and II databases. We determined the incidence of contralateral additional non-SN metastases in patients with unilateral SN-metastasis who underwent bilateral inguinofemoral lymphadenectomy (IFL). In those who underwent only ipsilateral groin treatment or no further treatment, we determined the incidence of contralateral groin recurrences during follow-up. RESULTS: Of 1912 patients with early-stage VSCC, 366 had a unilateral metastatic SN. Subsequently, 244 had an IFL or no treatment of the contralateral groin. In seven patients (7/244; 2.9% [95% CI: 1.4%-5.8%]) disease was diagnosed in the contralateral groin: five had contralateral non-SN metastasis at IFL and two developed an isolated contralateral groin recurrence after no further treatment. Five of them had a primary tumor ≥30 mm. Bilateral radiotherapy was administered in 122 patients, of whom one (1/122; 0.8% [95% CI: 0.1%-4.5%]) had a contralateral groin recurrence. CONCLUSION: The risk of contralateral lymph node metastases in patients with early-stage VSCC and a unilateral metastatic SN is low. It appears safe to limit groin treatment to unilateral IFL or inguinofemoral radiotherapy in these cases.

A phase 2, randomized, double-blind, placebo- controlled study of chemo-immunotherapy combination using motolimod with pegylated liposomal doxorubicin in recurrent or persistent ovarian cancer: a Gynecologic Oncology Group partners study.

BACKGROUND: A phase 2, randomized, placebo-controlled trial was conducted in women with recurrent epithelial ovarian carcinoma to evaluate the efficacy and safety of motolimod-a Toll-like receptor 8 (TLR8) agonist that stimulates robust innate immune responses-combined with pegylated liposomal doxorubicin (PLD), a chemotherapeutic that induces immunogenic cell death. PATIENTS AND METHODS: Women with ovarian, fallopian tube, or primary peritoneal carcinoma were randomized 1 : 1 to receive PLD in combination with blinded motolimod or placebo. Randomization was stratified by platinum-free interval (≤6 versus >6-12 months) and Gynecologic Oncology Group (GOG) performance status (0 versus 1). Treatment cycles were repeated every 28 days until disease progression. RESULTS: The addition of motolimod to PLD did not significantly improve overall survival (OS; log rank one-sided P = 0.923, HR = 1.22) or progression-free survival (PFS; log rank one-sided P = 0.943, HR = 1.21). The combination was well tolerated, with no synergistic or unexpected serious toxicity. Most patients experienced adverse events of fatigue, anemia, nausea, decreased white blood cells, and constipation. In pre-specified subgroup analyses, motolimod-treated patients who experienced injection site reactions (ISR) had a lower risk of death compared with those who did not experience ISR. Additionally, pre-treatment in vitro responses of immune biomarkers to TLR8 stimulation predicted OS outcomes in patients receiving motolimod on study. Immune score (tumor infiltrating lymphocytes; TIL), TLR8 single-nucleotide polymorphisms, mutational status in BRCA and other DNA repair genes, and autoantibody biomarkers did not correlate with OS or PFS. CONCLUSIONS: The addition of motolimod to PLD did not improve clinical outcomes compared with placebo. However, subset analyses identified statistically significant differences in the OS of motolimod-treated patients on the basis of ISR and in vitro immune responses. Collectively, these data may provide important clues for identifying patients for treatment with immunomodulatory agents in novel combinations and/or delivery approaches. TRIAL REGISTRATION: Clinicaltrials.gov, NCT 01666444.

A phase I trial of paclitaxel, cisplatin, and veliparib in the treatment of persistent or recurrent carcinoma of the cervix: an NRG Oncology Study (NCT#01281852).

Background: Preclinical studies demonstrate poly(ADP-ribose) polymerase (PARP) inhibition augments apoptotic response and sensitizes cervical cancer cells to the effects of cisplatin. Given the use of cisplatin and paclitaxel as first-line treatment for persistent or recurrent cervical cancer, we aimed to estimate the maximum tolerated dose (MTD) of the PARP inhibitor veliparib when added to chemotherapy. Patients and methods: Women with persistent or recurrent cervical carcinoma not amenable to curative therapy were enrolled. Patients had to have received concurrent chemotherapy and radiation as well as possible consolidation chemotherapy; have adequate organ function. The trial utilized a standard 3 + 3 phase I dose escalation with patients receiving paclitaxel 175 mg/m2 on day 1, cisplatin 50 mg/m2 on day 2, and escalating doses of veliparib ranging from 50 to 400 mg orally two times daily on days 1-7. Cycles occurred every 21 days until progression. Dose-limiting toxicities (DLTs) were assessed at first cycle. Fanconi anemia complementation group D2 (FANCD2) foci was evaluated in tissue specimens as a biomarker of response. Results: Thirty-four patients received treatment. DLTs (n = 1) were a grade 4 dyspnea, a grade 3 neutropenia lasting ≥3 weeks, and febrile neutropenia. At 400 mg dose level (DL), one of the six patients had a DLT, so the MTD was not reached. Across DLs, the objective response rate (RR) for 29 patients with measurable disease was 34% [95% confidence interval (CI), 20%-53%]; at 400 mg DL, the RR was 60% (n = 3/5; 95% CI, 23%-88%). Median progression-free survival was 6.2 months (95% CI, 2.9-10.1), and overall survival was 14.5 months (95% CI, 8.2-19.4). FANCD2 foci was negative or heterogeneous in 31% of patients and present in 69%. Objective RR were not associated with FANCD2 foci (P = 0.53). Conclusions: Combining veliparib with paclitaxel and cisplatin as first-line treatment for persistent or recurrent cervical cancer patients is safe and feasible. Clinical trial information: NCT01281852.

A phase II evaluation of motesanib (AMG 706) in the treatment of persistent or recurrent ovarian, fallopian tube and primary peritoneal carcinomas: a Gynecologic Oncology Group study.

OBJECTIVES: Vascular endothelial growth factors (VEGF) and their receptors have a critical role in stimulating the growth of ovarian cancer cells. Motesanib is a small molecule inhibitor of multiple receptor tyrosine kinases including VEGF receptors 1-3, as well as c-KIT and platelet-derived growth factor which are related to the VEGF family. PATIENTS AND METHODS: Twenty-two eligible patients with recurrent ovarian, fallopian tube or primary peritoneal carcinoma were treated with an oral daily dose of 125 mg of motesanib. Peripheral blood was analyzed for circulating tumor cells (CTC) and circulating endothelial cells/circulating endothelial progenitors (CEC/CEP), VEGF levels and cell-free circulating DNA (cfDNA). RESULTS: The study was abruptly halted after four patients developed posterior reversible encephalopathy syndrome. One patient had a partial response and seven patients had stable disease at the time they were removed from study treatment. Twelve of the 22 patients (50%) had indeterminate responses at trial closure. Early closure without clinical efficacy data precludes meaningful correlative studies. CONCLUSIONS: The serious central nervous system toxicity observed in patients with recurrent ovarian cancer precluded full examination of this agent in this population. There were no clear cut explanations for the high incidence of this known class effect in the study population compared with patients with other cancers.

Do uterine risk factors or lymph node metastasis more significantly affect recurrence in patients with endometrioid adenocarcinoma?

OBJECTIVES: Controversy continues over the importance of lymph node (LN) status in treating and predicting recurrence in endometrial cancer. Several predictive models are available which use uterine factors to stratify risk groups. Our objective was to determine how LN status affects recurrence and survival compared to uterine factors alone. METHODS: A retrospective review was performed of patients undergoing complete surgical staging for clinical stage 1 endometrioid adenocarcinoma of the uterus. Patients were assessed based on PORTEC 1 high intermediate risk (H-IR) criteria (2 factors : age>60, grade 3, >50% DOI), GOG-99 H-IR criteria (age >70+1 factor, age 50-70+2 factors, any age +3 factors: grade 2 or 3, LVSI, >50% DOI), and PORTEC 2 criteria. Rates of nodal involvement, recurrence rates, PFS, and OS were compared. RESULTS: We identified 352 clinical stage I patients with positive LN in 24% (87). 175 patients met PORTEC 1 eligibility and 66 met H-IR criteria. Rates of LN positivity were similar among groups (18.4% vs 19.7%, p=0.83) but recurrence rates were dissimilar (7.4% vs 27.3%, p=0.0004). Only 93 met PORTEC 2 criteria for treatment with no association between LN status, recurrence, and eligibility. 188 patients met H-IR eligibility criteria for GOG-99 with LN positive and recurrence rates higher in the H-IR group compared to GOG-99 eligible (34.6% vs 16.3%, p=0.0004, 28.3% vs. 10.6%, p=0.0002). CONCLUSIONS: Patients with H-IR disease based on uterine characteristics alone have substantial risk of nodal involvement. Knowledge of LN status may better define risk, prognosis, and postoperative treatment.

Cellular fibroma masquerading as ovarian carcinoma.

The presence of a benign pelvic mass, ascites, and an elevated CA 125 tumor marker level may mimic the presentation of ovarian adenocarcinoma. Two cases of ovarian cellular fibromas are reported to illustrate that the presence of ascites in association with an elevated CA 125 serum level may mislead physicians into a diagnosis of ovarian carcinoma. Although CA 125 is helpful for following patients after ovarian carcinoma has been diagnosed histologically, it has limited value as a screening test. Exploratory laparotomy with excision of the pelvic mass remains the only reliable and acceptable method available to achieve an accurate diagnosis.

Indiana pouch continent urinary reservoir in patients with previous pelvic irradiation.

Little information exists on the use of continent urinary reservoirs in patients with previous pelvic irradiation. We report the use of the Indiana pouch urinary reservoir in ten women with a history of pelvic irradiation for cervical cancer, of whom eight underwent a total pelvic exenteration for recurrent pelvic tumor and two had diversion for radiation-induced vesicovaginal fistula. All ten women achieved daytime continence, with a median time between catheterizations of 4.5 hours and a median pouch capacity of 500 mL. There was no evidence of leakage from the reservoir or significant ureteral reflux or obstruction on postoperative radiographic evaluation. No patient has required reoperation or had significant postoperative complications with the technique described.

Comparison of clinical versus surgical staging systems in vulvar cancer.

OBJECTIVE: To compare prognostic information from the new surgical staging system of the International Federation of Gynecology and Obstetrics (FIGO) with the old clinical staging system for vulvar cancer. METHODS: One hundred six women with previously untreated squamous cell carcinoma of the vulva who underwent radical vulvectomies and inguinal lymph node dissections at the University of Oklahoma from 1971-1990 were considered eligible for this study. A retrospective chart review was conducted to assign surgical stage. The clinical and pathologic factors analyzed for survival included the clinical and surgical stage of disease, nodal status, tumor size, and lesion location. RESULTS: Overall 5-year survival was 64%. Forty-three patients had inguinal and femoral node metastasis with a 5-year survival of 38%, versus 87% for patients without nodal metastasis (P < .00001). An increased number of positive groin lymph nodes was associated with a poorer prognosis. Thirty-one patients had tumors of 2 cm or less in maximum diameter with no recurrences, versus 52% 5-year survival in the remaining patients (P < .001). Perineal involvement was identified in 24 patients, but did not significantly influence survival. CONCLUSION: Overall, the new classification system revised by FIGO for vulvar cancer staging places patients into more accurate risk categories.

Surgical treatment of unexpected invasive cervical cancer found at total hysterectomy.

OBJECTIVE: To determine the proper management of patients found to have invasive cancer of the cervix on pathologic examination of a uterus removed for benign indications. METHODS: We report 18 patients undergoing hysterectomy who were found to have cervical cancer with invasion deeper than 3 mm and/or lymph-vascular space involvement. None had gross residual tumor following simple hysterectomy. All patients underwent a second operation. Seventeen women underwent a radical parametrectomy, upper vaginectomy, and pelvic lymphadenectomy; one had pelvic and periaortic lymphadenectomy alone because of bilateral grossly positive obturator nodes. RESULTS: Median follow-up was 72 months. One of the 15 women without residual disease or nodal involvement at second operation had pelvic recurrence 66 months after therapy. Three patients with disease identified at radical surgery underwent tailored postoperative pelvic radiation, and two of these had pelvic recurrence. The overall actuarial 5-year survival for the 18 patients was 89%. Operative morbidity was comparable to that of patients undergoing primary radical hysterectomy. CONCLUSION: This study confirms that patients with unexpected invasive cervical cancer found at total hysterectomy can undergo radical re-operation with low morbidity and excellent cure rates.

The influence of endometriosis on the staging of cervical cancer.

Pelvic endometriosis often presents as nodularity of the uterosacral and broad ligaments. Therefore, the presence of unrecognized pelvic endometriosis in cervical cancer patients can masquerade as parametrial spread of tumor, preventing correct assessment of stage of disease. A review of three cases in which these disease processes coexisted demonstrates that endometriosis can easily be mistaken for tumor extension on pelvic examination, computed tomography, and even gross inspection at laparotomy, resulting in incorrect staging and inappropriate management decisions. Because clinical staging often is inaccurate in cervical cancer patients with a history of endometriosis, pre-treatment operative assessment should be considered and frozen sections are recommended to assist with intraoperative management whenever the diagnosis of endometriosis could be entertained.

Management of endometrial cancer with suspected cervical involvement.

The 1989 International Federation of Gynecology and Obstetrics (FIGO) staging system for endometrial cancer cells for operative assessment of the extent of uterine disease, grade, and sites of metastasis before assigning a stage to the cancer. In the current study, 70 endometrial cancer patients with suspected cervical involvement based on a positive endocervical curettage or punch biopsy were treated with initial surgery followed by tailored radiation or chemotherapy. Only 37% of the patients had operative findings consistent with the preoperative suspicion of stage II disease. Postoperative therapy was determined by the extent of cervical involvement, depth of myometrial invasion, cell type, tumor grade, and the presence and location of extra-uterine disease. Based upon these parameters, 21 patients were believed to have low risk for pelvic recurrence and received no adjuvant therapy (90% 5-year survival); 38 patients received postoperative pelvic radiation because of high-risk factors for pelvic recurrence or pelvic nodal involvement (65% 5-year survival); and 11 patients received chemotherapy and/or extended radiation because of extrapelvic disease (no 5-year survivors). The approach outlined supports initial surgery for cases of endometrial cancer with suspected cervical involvement. This approach permits accurate surgical staging under the new FIGO system, avoids radiotherapy in many patients whose disease is less extensive than suspected preoperatively, and can accomplish good local control with limited morbidity.

Incidence and location of para-aortic lymph node metastases in gynecologic malignancies.

BACKGROUND: We sought to determine the location of metastases to para-aortic lymph nodes in patients with gynecologic malignancies. STUDY DESIGN: A retrospective chart review was performed for all cases of endometrial, ovarian, and cervical carcinoma in which right and left para-aortic lymph node dissection was done at our institution from 1985 to 1993. Records were assessed for tumor type as well as for presence and location of metastases to para-aortic lymph nodes. RESULTS: A total of 315 patients had bilateral para-aortic lymphadenectomy performed at the time of laparotomy as part of staging or therapy for their gynecologic malignancies. A total of 47 patients (15 percent) had metastasis to the para-aortic lymph nodes. Para-aortic metastasis were identified in 22 (30 percent) of 73 patients with ovarian carcinoma, 11 (8 percent) of 141 patients with cervical carcinoma, and 14 (14 percent) of 101 patients sampled. Unilateral left-sided para-aortic node involvement was observed in 13 patients, unilateral right-sided involvement was present in 14 patients, and bilateral involvement occurred in 20 patients. Regarding tumor type or origin, no significant difference was noted in right-sided compared with left-sided para-aortic metastases. CONCLUSIONS: Our data suggest no difference in the incidence of metastases to right-sided compared with left para-aortic lymph nodes in patients with gynecologic malignancies, emphasizing the need for bilateral evaluation of the para-aortic lymph nodes. This information is important in the clinical staging of gynecologic malignancies and in establishing protocols requiring para-aortic lymph node dissection.

Cost analysis of Hickman catheter insertion at bedside in gynecologic oncology patients.

BACKGROUND: Cost-containment issues are becoming increasingly important in medicine. The current study compares bedside versus operating room insertion of Hickman catheters from a cost and safety standpoint. STUDY DESIGN: A prospective chart review of all patients undergoing Hickman catheter insertion during the study period was performed to determine location of the procedure, rate of successful catheter placement, complications, and cost. RESULTS: Ninety-six patients underwent placement of 108 Hickman catheters during a seven year period. Fifty-three catheters were inserted at bedside while 55 catheters were placed in the operating room. The complication rate was 8 percent for the bedside and 9 percent for the operating room group. Due to anesthesia standby, operating room time, and fluoroscopy, cost analysis revealed a substantial savings of $1,545 per patient if bedside insertion was utilized. CONCLUSIONS: The data indicate that, in select patients, percutaneous insertion of Hickman catheters at bedside is a safe, cost-effective procedure.

Human immunodeficiency virus testing in patients with invasive cervical carcinoma: a prospective trial of the gynecologic oncology group.

To determine the frequency of positive human immunodeficiency virus (HIV) serostatus among North American women 50 years of age or younger with invasive cervical cancer and to define their tolerance to treatment. Consenting patients with newly diagnosed invasive cervical cancer, age 50 or younger were tested by enzyme-linked immunosorbent assay. The study design anticipated that approximately 3% of patients would be HIV positive. After the accrual of 913 eligible and evaluable patients, interim analysis revealed that only 9/913 ( approximately 1%) patients were HIV seropositive, indicating that it would not be feasible to achieve the study objective. The study was closed to further accrual. Between 1994 and 1997, the frequency of positive HIV serostatus among North American women with newly diagnosed cervical cancer was quite low. As a consequence, no evaluation of response to treatment or treatment tolerance can be made.

Cisplatin and pentoxifylline in advanced or recurrent squamous cell carcinoma of the cervix: a phase II trial of the Gynecologic Oncology Group.

OBJECTIVE: The aim of this study was to determine the antitumor activity and toxicity of cisplatin and pentoxifylline in previously treated patients with squamous cell carcinoma of the cervix. METHODS: A Gynecologic Oncology Group (GOG) Phase II trial of recurrent squamous cell cervical cancer using standard GOG response and toxicity criteria was performed. RESULTS: A total of 47 patients with advanced or recurrent squamous cell carcinoma of the cervix were entered. The starting dose was 75 mg/m(2) of cisplatin every 21 days and 1600 mg of pentoxifylline PO every 8 h for nine doses during each course. Forty patients were evaluable for response and 44 were evaluable for toxicity. Of the 40 evaluable patients, 37 had received prior radiotherapy and 35 had received prior chemotherapy. A median of three courses were given (range: 1-7). Among evaluable patients, 1 had a complete response (2.5%) and 3 had a partial response (7.5%) for an overall objective response rate of 10%. The complete responder had not previously had chemotherapy. Grade 3 or 4 toxicity was predominantly nausea and vomiting (32%) and hematologic toxicity (23%). CONCLUSIONS: The combination of cisplatin and pentoxifylline at the dose and schedule tested has limited activity in previously treated advanced or recurrent cervical cancer.

Hypogastric artery aneurysm masquerading as an ovarian neoplasm.

Hypogastric artery aneurysm in women is rare. The case presented demonstrates how this lesion can easily mimic an ovarian neoplasm. The missed diagnosis can be catastrophic if the surgeon is unfamiliar with the retroperitoneal anatomy and is confronted with arterial hemorrhage. Hypogastric artery aneurysm should be included in the differential diagnosis of a pelvic mass in elderly women with atherosclerotic disease. The report reviews the literature on the presentation, diagnosis, and recommended treatments.

Hepatic venoocclusive disease as a complication of whole abdominopelvic irradiation and treatment with the transjuglar intrahepatic portosystemic shunt: case report and literature review.

We report the novel use of the transjugular intrahepatic portosystemic shunt (TIPS) procedure for the treatment of intractable ascites due to hepatic venooclusive disease as a result of whole abdominopelvic radiotherapy. A patient with Stage III endometrioid carcinoma of the endometrium treated with postoperative whole abdominopelvic irradiation developed intractable ascites. Multiple paracenteses and computerized tomography were negative for recurrent carcinoma. Liver biopsy demonstrated hepatic venoocclusive disease, a rare complication of therapeutic radiation involving the liver. Successful relief of ascites and its adverse symptomology were achieved with the transjugular intrahepatic portosystemic shunt. Relevant literature regarding the pathogenesis, prognosis, and treatment of radiotherapy-related hepatic venoocclusive disease are reviewed.

Expression of EGFR, HER-2/neu, P53, and PCNA in endometrioid, serous papillary, and clear cell endometrial adenocarcinomas.

Expression of four biologic markers was studied in 69 cases of endometrial cancer to identify their association with cell type, decreased survival, and increased tumor metastasis. Cell types included endometrioid (n = 45), serous papillary (n = 16), and clear cell (n = 8). Immunohistochemical stains were employed to detect the presence of epidermal growth factor receptor (EGFR), HER-2/neu, p53, and proliferating cell nuclear antigen (PCNA). Analysis revealed that EGFR was expressed in 49%, HER-2/neu in 59%, p53 in 9%, and PCNA in 16% of tumor specimens. HER-2/neu overexpression was significantly associated with depth of myometrial invasion. p53 and PCNA immunoreactivity significantly correlated with nonendometrioid histology, although PCNA was less specific in labeling these less favorable cell types. EGFR immunoreactivity also significantly correlated with nonendometrioid cell types and tumor metastases at time of diagnosis. Seventy-seven percent of patients with metastatic disease were EGFR-positive versus 36% positivity in patients with no evidence of metastases (P < 0.002). For patients with endometrioid adenocarcinoma, evidence of EGFR overexpression decreased survival from 89 to 69% (P < 0.04). In the serous papillary and clear cell category, EGFR positivity decreased survival from 86 to 27% (P < 0.03). EGFR strongly correlates with tumor metastasis and patient survival in endometrial cancer. Altered expression of this oncoprotein may serve as a guide to prognosis and treatment in these patients.

Primary endodermal sinus tumor of the vulva: a case report and review of the literature.

BACKGROUND: Extragonadal endodermal sinus tumors arising in the external genitalia represent an exceedingly rare malignancy in women. Six cases of endodermal sinus tumors of the vulva have been reported to date, with three cases failing to respond to conservative surgery and vincristine-based chemotherapy. We report a seventh case of vulvar endodermal sinus tumor that was treated with radical surgery and platinum-based chemotherapy. CASE: RT is an 18-year-old female who presented with a vulvar mass that was diagnosed as endodermal sinus tumor at the time of biopsy. She was subsequently treated with modified radical vulvectomy and ipsilateral groin lymphadenectomy, followed by bleomycin, etoposide, and cisplatin chemotherapeutic regimen. She has since remained free of disease for 18 months as evidenced by serum alpha-fetoprotein and physical exam at 18 months. CONCLUSIONS: Vulvar endodermal sinus tumors represent a very small number of germ cell tumors in women. Based on the previous accounts, this disease appears to be more fatal than endodermal sinus tumor arising at other sites. These tumors also have a predilection for local metastasis. Due to the previous accounts, we chose to treat this patient with radical surgery and platinum-based chemotherapy. This treatment regimen has resulted in a disease-free state for 18 months.