Mixed mesodermal tumor of the ovary: analysis of prognostic factors in 31 cases.

OBJECTIVE: To determine significant prognostic factors in patients with mixed mesodermal tumors of the ovary. METHODS: Thirty-one cases of mixed mesodermal tumor of the ovary treated at Memorial Sloan-Kettering Cancer Center between 1977-1990 were reviewed retrospectively. The mean patient age was 61 years. Distribution by stage was as follows: I, seven (23%); II, one (3%); III, 15 (48%); and IV, eight (26%). The median follow-up for survivors was 62 months. Following primary surgery, chemotherapy included cisplatin (four), doxorubicin (seven), or both (ten); six patients received various other treatments. RESULTS: The median survival for the entire group was 10.6 months. In 19 cases (61%), heterologous sarcomatous elements were present in the primary tumor, whereas 12 (39%) contained homologous elements only. There was a trend toward improved survival in patients whose primary tumors had only homologous stromal elements (P = .06). The overall survival was significantly better for the eight patients with early-stage (I, II) disease than for the 23 patients with advanced-stage (III, IV) disease (P = .01). The size of residual disease after cytoreductive surgery was not a significant prognostic factor. There was no difference in survival between the ten women whose metastatic disease contained only epithelial elements and the 16 whose metastases contained mesenchymal elements as well (P = .23). CONCLUSIONS: This study confirms previous observations that mixed mesodermal tumors of the ovary are a highly malignant group of tumors that respond poorly to chemotherapy. In addition, we demonstrated that prognosis is independent of the presence or absence of sarcomatous elements in the metastases.     

Reproductive function after conservative surgery and chemotherapy for malignant germ cell tumors of the ovary

OBJECTIVE: To analyze the long-term effects on reproductive function of fertility-preserving treatment for malignant germ cell tumors of the ovary. METHODS: A case series analysis was performed on patients with malignant germ cell tumors of the ovary seen or consulted on at our institution between 1975 and 1995. Follow-up information regarding reproductive function was obtained by a mailed or telephone questionnaire. RESULTS: A total of 106 patients with malignant germ cell tumors of the ovary were included in the study. Twenty patients were excluded because of loss of follow-up or death. For the remaining 86 patients, the median follow-up was 122 months (24-384 months). Fertility-preserving surgery was performed in 64 patients. Thirty-eight have attempted conception and 29 have achieved at least one pregnancy (76%). Among the patients who conceived, 20 were International Federation of Gynecology and Obstetrics (FIGO) stage I, one was stage II, and eight were stage III. Sixteen received vincristine, actinomycin D, and cyclophosphamide; three received cisplatin, vinblastine, and bleomycin; three received bleomycin, etoposide, and cisplatin; one received etoposide and cisplatin; four did not receive any chemotherapy; and two were treated with other combinations. Among the nine patients who could not conceive, seven were FIGO stage I and two were stage III. Four of these patients received vincristine, actinomycin D, and cyclophosphamide; three received etoposide and cisplatin; one received cisplatin, vinblastine, and bleomycin; and one patient received no chemotherapy. A total of 38 children were born to these women. Follow-up was available for 16 of these children, who have no evidence of congenital anomalies. CONCLUSION: Fertility-preserving surgery followed by chemotherapy, even in advanced-stage malignant germ cell tumors of the ovary, is effective in conserving the reproductive function of women with malignant germ cell tumors of the ovary.     

Clinical features of advanced ovarian mixed mesodermal tumors and treatment with doxorubicin- and cis-platinum-based chemotherapy

Records of 15 patients with stage III and IV malignant mixed mesodermal tumors of the ovary treated between 1977 and 1988 were reviewed. All patients had primary surgery; 13 were given postoperative chemotherapy including doxorubicin and cis-platinum. Median survival for patients receiving chemotherapy is 16 months; 62% were alive at 12 months and 31% at 24 months. Progression-free responses were seen in 85% of treated patients and 55% of these recurred. All recurrences involved the pelvis and were predominantly mesenchymal. Serum CA-125 values accurately reflected tumor presence in 82% of tested patients. Cytoreductive surgery followed by treatment including doxorubicin- and cis-platinum-based chemotherapy is effective in treatment of disseminated ovarian mixed mesodermal tumors, but additional components must be added to achieve durable responses and consistently prolonged survivals.     

Immature teratoma of the ovary

Forty-one patients with pure immature teratoma of the ovary treated at The University of Texas M. D. Anderson Hospital and Tumor Institute at Houston from 1944 to 1985 were reviewed. The median age of these patients was 17 years. The most common symptom was abdominal pain, which occurred in 39 (95%) patients. FIGO stages included 24 patients with stage I, four with stage II, 12 with stage III, and one stage IV. The tumors ranged in size from 6 to 31 cm in greatest diameter, with a median of 17 cm. Of 39 cases reexamined microscopically, four were grade 1 tumors, 22 were grade 2 tumors, and 13 were grade 3 tumors. Initial surgery included ovarian cystectomy in one patient, unilateral salpingo-oophorectomy in 27 patients, and bilateral salpingo-oophorectomy in 13 patients. Fifteen of 16 patients treated with surgery alone developed recurrent disease; 11 are surviving after further therapy. Two patients died after treatment with radiotherapy plus chemotherapy. Twenty-one patients received a combination of vincristine, actinomycin-D, and cyclophosphamide postoperatively, and 18 are alive and well. One of two patients who received other combination regimens is alive and well. Therefore, 29 of the 41 patients (71%) are alive and well. Optimal management of patients with pure immature teratoma of the ovary consists of initial surgery (with preservation of a normal contralateral ovary in most patients) followed by combination chemotherapy in all patients except those with stage I, grade 1 disease; the latter may be treated safely with surgery alone.     

Treatment of ovarian stromal tumors.

Fifty-one patients with granulosa-cell tumors and nine patients with Sertoli-Leydig-cell tumors have been treated at the M. D. Anderson Hospital. The most important prognostic finding was the stage of the tumor when first seen. Conservative surgery was utilized in young patients with lesions confined to one ovary. More advanced tumors were treated with maximal tumor resection and postoperative treatment with either irradiation or chemotherapy. Postoperative radiation was given when the tumor capsule had ruptured or residual tumor less than 2 cm. in diameter was present after surgery. Single-agent chemotherapy was ineffective in ovarian stromal tumors, but combination chemotherapy was found to be effective in advanced or recurrent stromal tumors. Actinomycin-D, 5-fluorouracil, and cyclophosphamide were effective in granulosa-cell tumors. Vincristine, actinomycin-D, and cyclophosphamide were effective in Sertoli-Leydig--cell tumors.     

Advanced malignant rhabdoid tumor of the ovary effectively responding to chemotherapy: a case report and review of the literature

BACKGROUND: Malignant rhabdoid tumors (MRTs) are highly malignant neoplasms that consist of both renal and extrarenal subtypes. Primary ovarian cases are extremely rare. We herein describe the third known case of ovarian origin, which effectively responded to combination chemotherapy with ifosfamide, epirubicin, and cisplatin (IEP chemotherapy). CASE: A 19-year-old woman was diagnosed to have stage IIIc primary MRT of the ovary following the resection of tumors. Two months after surgery, an 8 cm-sized pelvic mass and enlarged retroperitoneal lymphnodes were detected. The patient received intravenous tri-weekly IEP chemotherapy. After the second course of chemotherapy, she demonstrated a complete clinical response. CONCLUSION: Although this type of tumor is quite aggressive and chemotherapy is generally not considered to be effective, IEP chemotherapy may be useful in the treatment of MRT of the ovary.     

原发性卵巢恶性苗勒管混合瘤10例临床和病理分析

目的:分析原发性卵巢恶性苗勒管混合瘤(OMMMT)的临床和病理特点,以期提高诊治水平。方法:回顾性分析收集的10例OMMMT患者临床和病理资料,对其年龄、临床症状、血清CA125水平、手术分期、残留病灶、病理结果、化疗方案、化疗反应率、药物不良反应和生存情况进行分析。结果:OMMMT患者平均年龄60.1岁,主要临床表现是腹胀、腹痛和腹部包块。血清CA125均升高。Ⅱ期1例,Ⅲ期8例,Ⅳ期1例。所有患者首先接受肿瘤细胞减灭术,5例无肉眼残留,3例残余病灶<2cm,2例残留病灶>2cm。病理检查上皮癌成分有子宫内膜样腺癌、浆液性腺癌和透明细胞癌;肉瘤成分同源性7例,异源性2例,同源性和异源性同时存在1例。所有患者术后均接受了以铂类为基础的联合化疗。7例采用紫杉醇+卡铂/顺铂方案化疗,完全缓解率是71.4%(5/7),总反应率是85.7%(6/7),仅2例出现Ⅲ度以上骨髓抑制;1例采用异环磷酰胺+多柔比星+顺铂方案化疗,获得完全缓解,但出现Ⅳ度骨髓抑制;2例经济条件差的患者采用环磷酰胺+多柔比星+顺铂方案化疗,获得完全缓解,药物不良反应轻微。随访复发3例,8例患者存活(3例带瘤生存,5例无瘤生存),1例死亡,失访1例。结论:OMMMT患者年龄大、分期晚,确诊依靠病理检查。治疗原则参照卵巢上皮性癌的治疗方案,首选肿瘤细胞减灭术,紫杉醇+铂类为基础的联合化疗作为一线化疗方案,异环磷酰胺+顺铂作为二线化疗方案。     

Endodermal sinus tumor of the ovary: the M. D. Anderson experience

Forty-one patients with pure endodermal sinus tumor of the ovary who were treated at the M. D. Anderson Hospital from 1944 to 1981 are retrospectively reviewed. The median age of these patients was 19 years. The most common presenting symptom was abdominal pain, occurring in 33 patients (80%). No bilateral ovarian involvement was noted at initial surgery. With the exception of 1 patient treated by surgery alone, only patients treated with postoperative combination chemotherapy survived. Twenty-one of the 41 patients are alive and well. Sixteen of 22 patients treated with vincristine, actinomycin-D, and cyclophosphamide have survived. The roles of second-look laparotomy and alpha-fetoprotein monitoring are discussed. Optimal treatment seems to consist of surgery followed by aggressive combination chemotherapy.     

卵巢卵黄囊瘤手术和化疗后自然周期体外受精-胚胎移植成功分娩一例并文献复习

卵巢卵黄囊瘤属卵巢恶性生殖细胞肿瘤,占卵巢恶性肿瘤的1%。由于卵巢恶性生殖细胞肿瘤多见于儿童及年轻女性,故保存生育能力是关键。卵巢卵黄囊瘤对化疗药物敏感,手术联合术后辅助化疗是标准治疗方案。对于对侧卵巢和子宫未受肿瘤累及,并且有生育需求的患者均应行保留生育功能的手术。但肿瘤本身、手术和放化疗均可能造成患者不孕,术后可能需要借助辅助生殖技术助孕,本文通过1例卵巢卵黄囊瘤患者手术和术后化疗后行自然周期-体外受精-胚胎移植(NC-IVF-ET)的个案报道和文献复习,探讨卵巢卵黄囊瘤患者的生育助孕策略。     

Diagnosis and clinical management in malignant Müllerian tumors of the fallopian tube

Four out of 42 cases of primary tubal malignancy diagnosed in our histopathological laboratory were malignant mixed Müllerian tumors (MMMT). All four patients were postmenopausal with a mean age of 66.5 years at diagnosis. A correct preoperative diagnosis was made only in one case. Tumor staging (FIGO) revealed stage Ila, IIIc and IV. One patient died of postoperative pulmonary embolism, a second patient of an unknown cause five month after surgery and a third patient died of disease after I I months with secondary deposits in pelvic peritoneum, omentum and paraaortic lymph nodes. The fourth patient is still alive. One patient received chemotherapy alone, one by radiation and chemotherapy and two patients by radiation alone. Tumor spread at the time of diagnosis and the residual tumor volume were the most important prognostic factors. All tumors were histologically the homologous type of MMMT (carcinosarcomas). No heterologous elements were found. Metastatic tumors showed only sarcomatous elements.     

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.     

Management of malignant ovarian germ cell tumors

Malignant ovarian germ cell tumors are rare, highly curable cancers of young women. The majority of patients can be cured with either fertility-preserving surgery alone or a combination of surgery and chemotherapy. Relapses occur in 10% to 20% of patients, and the significant proportion of them can be salvaged with chemotherapy. There is no evidence that treatment for malignant ovarian germ cell tumors will adversely affect menstrual or reproductive functions, increase future pregnancy loss, or increase the risk of congenital malformations of the fetus. Late effects, such as secondary leukemia, from chemotherapy are reported but rare. TARGET AUDIENCE: Obstetricians & Gynecologists and Family Physicians. LEARNING OBJECTIVE: After completing this CME activity, physicians should be better able to diagnose ovarian germ cell tumors, outline management of malignant ovarian germ cell tumors, and understand the impact of treatment on fertility and late effects.     

Stage III ovarian tumors of low malignant potential treated with cisplatin combination therapy (A Gynecologic Oncology Group Study)

By serendipity we have had the opportunity to evaluate cis-platin-based chemotherapy in ovarian tumors of low malignant potential (LMP). Optimal (less than 1 cm residual disease) FIGO stage III ovarian carcinomas were randomly assigned to treatment with cisplatin plus cyclophosphamide with or without doxorubicin on a prospective Gynecologic Oncology Group Study. On review by the Gynecologic Oncology Group Pathology Committee, 32 of these cases were determined to represent low malignant potential tumors. Mean age of patients with these lesions was 48 years (range, 25-75 years). After initial cytoreduction, 19 patients had residual disease less than 1 cm and 13 had no residual. Twenty (62.5%) received cisplatin plus cyclophosphamide and 12 cisplatin, cyclophosphamide, and doxorubicin chemotherapy; 75% of patients received six or more courses. Second-look surgery was done in 15 cases; only six were negative. However, with a median follow-up of 31.7 months (range, 1-75), only 1 patient has died; no cancer was found at autopsy. The remaining patients are alive without clinical evidence of disease at a median of 30 months. The need for adjunctive therapy in patients with advanced LMP tumors remains speculative.     

Primary malignant mesodermal ovarian sarcomas

Primary malignant mesodermal ovarian sarcomas are rare tumors and have a poor prognosis. The disease is usually diagnosed at a late stage and 5-year survivals are uncommon. Most patients are treated with debulking surgery followed by adjuvant chemotherapy. We report ten patients treated at a single institution. All patients underwent surgery and 90% received adjuvant chemotherapy. The median survival was 20 months, and only one patient survived beyond 5 years. Newer treatment strategies are urgently needed in the management of this disease.     

Malignant mixed müllerian tumor of the ovary and false negative punctures

Malignant mixed müllerian tumour (MMMT) of the ovary is a rare and aggressive tumour with a poor prognosis. We present a case of a 57-year-old woman with a large pelvic mass, omental cake, ascites and pleural effusions, clinically highly suspect of an ovarian neoplasm. Paracentesis and ultrasound-guided biopsy of the ovary were negative for malignant disease. Therefore a CT-guided true cut biopsy was performed. The latter gave a histopathologic diagnosis of an endometrioid adenocarcinoma of the ovary. However after cytoreductive surgery anatomopathologic examination revealed a malignant mixed müllerian tumour of the ovary with heterologous differentiation. Apparently only one of the two components was found in the puncture. Adjuvant chemotherapy, active against the sarcomatous and the carcinomatous component, was given. At present the patient is well and disease free 35 months after the initial diagnosis. Cytological examination of ascites may be negative in the presence of malignant disease. If a tumour consists of two components, puncture can miss one, which may lead to undertreatment. Punctures should be discouraged as a diagnostic tool in patients in whom an ovarian malignancy is suspected.     

Evidence for the efficacy of adjuvant therapy in epithelial ovarian tumors of low malignant potential

During the 10-year period from January 1975 through December 1985, 48 patients with low malignant potential epithelial ovarian tumors were treated on the Gynecology Service at Memorial Sloan-Kettering Cancer Center. No patients were lost to follow-up and the mean duration of follow-up was 42 months. Twenty-nine patients were Stage I, 5 patients were Stage II, 11 patients were Stage III, 1 patient was Stage IV, and 2 patients were unstaged. Patients were divided into those with no residual disease after initial surgical treatment (29), microscopic residual disease (7), and gross residual disease (12). No patient without residual disease died from cancer. Of the 19 patients with residual disease, all received adjunctive chemotherapy alone, radiation therapy alone, or a combination of both. Three of 19 patients died from cancer. Twelve patients with residual disease were found to be free of disease at second-look surgical reassessment following adjunctive therapy. This review indicates that adjunctive therapy can eradicate residual disease in patients with epithelial ovarian tumors of low malignant potential.     

Cisplatin, adriamycin and ifosfamide-based combination chemotherapy for gynecological cancers derived from the extended müllerian system.

Their histogenetic similarity suggests that a group of malignant tumors may have a common sensitivity to a cytotoxic chemotherapy. Seventy patients with a variety of gynecological cancers arising from the uterine cervix, endometrium, ovary and pelvic peritoneum were treated with a combination of cisplatin, adriamycin and ifosfamide (PAI). As schedule modifications, PAI plus bleomycin for cancers containing squamous components and PAI plus etoposide for nonepithelial malignancies were recommended. In twenty-five evaluable cases, including 12 recurrent tumors after previous radiation therapy or PAC (cisplatin, adriamycin and cyclophosphamide) chemotherapy, the total response rate was 95% for epithelial cancers (vaginal cancer: 1/1, cervical: 9/10, endometrial: 2/2, ovarian: 6/6 and peritoneal: 2/2), and 100% for nonepithelial malignancies including one uterine leiomyosarcoma, one uterine mixed Müllerian tumor and one extragonadal mixed Müllerian tumor. The survival rates of patients with non measurable lesion were 100% for cervical cancer (the observation period: 65-879 days), 92.9% for endometrial cancer (96-975 days) and 88.9% for ovarian cancer (148-976 days). The hematological toxicity of this treatment was severe but acceptable. The results obtained indicate that a wide range of gynecological cancers originating in the primary and secondary Müllerian tissues (extended Müllerian system) must have a similar sensitivity to cytotoxic treatment with a PAI-based combination chemotherapy.     

Surgery as sole treatment for serous borderline tumors of the ovary with noninvasive implants

OBJECTIVES: The objectives were to describe the clinical characteristics and prognosis of surgically treated patients with stage II and III serous borderline tumors of the ovary with noninvasive implants. MATERIALS AND METHODS: From 1990 to 2000, 16 patients with stage II and III ovarian serous borderline tumors and noninvasive implants were diagnosed and prospectively followed at our center. All patients underwent surgical treatment including staging and their pathology was reviewed. Fifteen patients had thorough surgical staging by laparotomy, while one patient was staged laparoscopically. No patient was treated with adjuvant therapy (radiation or chemotherapy) after surgical treatment and none were lost to follow-up. RESULTS: The mean age at diagnosis was 42 years (range 26-59). Fourteen patients were treated by abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy, and multiple peritoneal biopsies, while 2 patients were treated conservatively for fertility preservation. Two patients underwent pelvic and para-aortic lymph node dissection. Fifteen of 16 patients had ovarian surface involvement with tumor. All patients but 2 had clinical evidence of extraovarian disease at the time of surgery. The mean duration of follow-up was 60.7 months (range 2-134 months). Thirteen patients (81%) are alive without evidence of disease. Four patients (25%) required subsequent surgery for recurrent disease and all are still alive. Two patients have been treated with chemotherapy (paclitaxel/carboplatin) for progressive borderline disease, while an additional patient was treated after first relapse with chemotherapy for an invasive recurrence. CONCLUSIONS: Carefully staged patients with advanced serous borderline tumors of the ovary and noninvasive implants have a good prognosis without adjuvant therapy.     

Outcome of reproductive age women with stage IA or IC invasive epithelial ovarian cancer treated with fertility-sparing therapy

OBJECTIVES: The purpose of this study was to determine the recurrence rate, survival, and pregnancy outcome in patients with Stage IA and Stage IC invasive epithelial ovarian cancer treated with unilateral adnexectomy. METHODS: A multi-institutional retrospective investigation was undertaken to identify patients with Stage IA and IC epithelial ovarian cancer who were treated with fertility-sparing surgery. All patients with ovarian tumors of borderline malignancy were excluded. Long-term follow-up was obtained through tumor registries and telephone interviews. The time and sites of tumor recurrence, patient survival, and pregnancy outcomes were recorded for every patient. RESULTS: Fifty two patients with Stage I epithelial ovarian cancer treated from 1965 to 2000 at 8 participating institutions were identified. Forty-two patients had Stage IA disease, and 10 had Stage IC cancers. Cell type was distributed as follows: mucinous, 25; serous, 10; endometrioid, 10; clear cell, 5; and mixed, 2. Histologic differentiation was as follows: grade 1, 38; grade 2, 9; and grade 3, 5. Twenty patients received adjuvant chemotherapy (mean 6 courses, range 3-12 courses). Patients received the following chemotherapeutic agents: cisplatin/taxol or carboplatin/taxol, 11; melphalan, 5; cisplatin and cyclophosphamide, 3; and single-agent cisplatin, 1. Eight patients had second-look laparotomies and all were negative. Duration of follow-up ranged from 6 to 426 months (median 68 months). Five patients developed tumor recurrence 8-78 months after initial surgery. Sites of recurrence were as follows: contralateral ovary, 3; peritoneum, 1; and lung, 1. Nine patients underwent subsequent hysterectomy and contralateral oophorectomy for benign disease. At present, 50 patients are alive without evidence of disease and 2 have died of disease 13 and 97 months after initial treatment. The estimated survival was 98% at 5 years and 93% at 10 years.Twenty-four patients attempted pregnancy and 17 (71%) conceived. These 17 patients had 26 term deliveries (no congenital anomalies noted) and 5 spontaneous abortions. CONCLUSION: The long-term survival of patients with Stage IA and IC epithelial ovarian cancer treated with unilateral adnexectomy is excellent. Fertility-sparing surgery should be considered as a treatment option in women with Stage I epithelial ovarian cancer who desire further childbearing.     

卵巢非特异性类固醇细胞瘤2例分析及文献复习

目的：探讨卵巢非特异性类固醇细胞瘤的临床、病理学特点,以及诊断和治疗方法。方法：回顾性分析复旦大学附属妇产科医院2000—2010年间收治的2例卵巢非特异性类固醇细胞瘤患者的临床资料,并进行文献复习。结果：良、恶性卵巢非特异性类固醇细胞瘤各1例。2例患者均表现为闭经、男性化等特征,术中及病理所见均与已报道特点一致。良性患者经保守治疗后各项生化指标恢复正常,随访至今状况良好;而恶性患者虽经手术和化疗,10个月后仍死于肿瘤复发。结论：卵巢非特异性类固醇细胞瘤是罕见的卵巢肿瘤,治疗以手术为主,部分患者为恶性,可行化疗,但预后较差。