Extragenital malignant mixed müllerian tumor

A case of extragenital malignant mixed müllerian tumor that appeared to represent a second primary tumor in a patient previously treated for disseminated ovarian serous papillary carcinoma is reported and compared with other reported cases of extragenital malignant mixed müllerian tumor.     

Taxol given weekly in advanced previously treated ovarian carcinomas – a pilot study

In this pilot study, 42 patients with advanced ovarian carcinoma were treated with Taxol weekly instead of every 3 weeks. The initial dose was 67 mg m⁻². The clinical response rate was 29%. The response rate was 22% for patients with platinum-resistant tumors compared to 42% for patients with platinum-sensitive tumors. This difference was not statistically significant. The median progression free survival was 110 days and the median overall survival 341 days. The toxicity was acceptable and probably less pronounced than with the standard tri-weekly schedule. A randomized multicenter study comparing these two treatment schedules is presently under way in Scandinavia.     

Extragenital malignant mixed müllerian tumor: review of the literature.

A case of extragenital malignant mixed mullerian tumor (MMMT) of the heterologous type 11 years after treatment of a Stage I ovarian neoplasm in a 65-year-old patient is described. There was no evidence of a histologic relationship between these two neoplasms. This case represents the 10th report of an extragenital MMMT. The medical literature and histogenesis of this rare tumor are discussed.     

Extragenital primary mixed malignant mesodermal tumor.

Malignant mixed mesodermal tumors (MMMT) are infrequent neoplasms characteristically arising in the endometrium. Extragenital MMMTs are extremely rare, with but 11 cases reported in the literature. Previous extragenital MMMTs have been associated with endometriosis, Wolfian duct remnants, and ovarian cyst adenocarcinoma and have been presumed to arise from coelomic and subcoelomic structures. We report a case of a MMMT arising extragenitally in the cul-de-sac in a 54-year-old White female patient in whom disseminated intraperitoneal serosal papillary serous adenocarcinoma of the peritoneum was present. The histogenesis of this rare neoplasm is discussed along with a brief review of previously reported cases.     

Prognostic factors in malignant mixed Müllerian tumor of the fallopian tube: the Radiumhemmet series 1923–1994

Malignant mixed Müllerian tumors (MMMT) of the fallopian tube are extremely rare and account for less than 4% of all MMMT. The most common site of origin for MMMT is the endometrium followed, in decreasing order, by the vagina, cervix and ovary. To date less than 60 cases of MMMT in the fallopian tube have been reported. The prognosis is poor. We report nine cases of fallopian tube MMMT treated during the period 1923–1994. Analysis of ploidy, p53, tumor angiogenes and cell proliferation did not help to discriminate long and short time survivors. Three cases receiving complete therapy including surgery, chemotherapy and external radiation belong to the long-survival group. Our conclusion is that MMMT represent clinically and biologically highly malignant tumors. Thus aggressive treatment comprising complete surgery, chemotherapy and external radiation seems to be necessary in order to improve survival both in early and advanced stage patients.     

Heterologous malignant mixed Müllerian tumor of the cervical stump

A 76-year-old woman presented with vaginal bleeding 37 years following supracervical hysterectomy for a benign disease. Extensive malignant mixed Müllerian tumor was found in the remaining uterine cervix. This appears to be the first documented case of the malignant mixed Müllerian tumor arising in a cervical stump. Possibility of derivation of this tumor from endometriosis notwithstanding, the cervical Müllerian neoplastic potential parallels that of the endometrium because of the developmental kinship of the two. This potential has been fully retained and expressed as a tumor in the cervical stump in this case.     

A phase II study of fixed dose rate gemcitabine in patients with relapsed müllerian tumors

Gemcitabine (2',2'-difluorodeoxycytidine) is a novel purine analog with clinical activity against ovarian cancer. Accumulation of gemcitabine triphosphate (dFdCTP) increases in a linear fashion with prolonged infusions of gemcitabine, and there is a strong relationship between intracellular accumulation of dFdCTP and DNA damage. Women with ovarian, fallopian tube, or primary peritoneal carcinoma and documented recurrent disease were eligible for the study. Patients could not have received more than four prior lines of chemotherapy and had to have measurable or evaluable disease. Gemcitabine 800 mg/m2 administered by intravenous infusion at 10 mg/m2/min (fixed dose rate [FDR]) on days 1 and 8 of a 21-day schedule. Twenty-eight patients with a median age 60 (range, 40-77) years were treated. Although 43% were Eastern Cooperative Oncology Group 0, 50% had liver metastases. Eighty-eight cycles of therapy were delivered (median 2 [range, 1-6]). Five of the first ten patients treated at 800 mg/m2 could not receive day 8 FDR-gemcitabine because of neutropenia, and the starting dose was reduced to 700 mg/m2. Even at this dose there was cumulative hematologic toxicity resulting in dose reductions. Vomiting, mucositis, diarrhea, allergy, rash, fever, and alopecia were mild. In 28 patients, there was only one partial response (4%, 95% CI 0-18%) and median time to progression was 1.7 (interquartile range, 1.2-3.9) months. FDR-gemcitabine 700 mg/m2 administered by intravenous infusion at an FDR of 10 mg/m2/min had minimal activity against heavily pretreated recurrent tumors of müllerian origin. The optimal dose and schedule of gemcitabine is yet to be defined in this population.     

Response of recurrent uterine high-grade malignant mixed müllerian tumor to letrozole

Uterine malignant mixed müllerian tumor (MMMT) is a rare malignancy occurring most often in postmenopausal women. Despite the use of multimodality treatments including surgery, chemotherapy, and radiotherapy, prognosis is still poor in most cases. We report the case of a 69-year-old woman with recurrent metastatic high-grade MMMT that responded to letrozole, an aromatase inhibitor. At the initial diagnosis of high-grade uterine MMMT in February 2001, the patient underwent total abdominal hysterectomy, bilateral salpingo-oophorectomy, and postoperative pelvic radiotherapy. Two years later, an asymptomatic retroperitoneal mass was discovered on surveillance abdominal computed tomography scanning. The 3.5- x 3.0-cm mass was considered inoperable owing to its location near the aorta at the level of the renal vessels. The patient declined radiation or chemotherapy. Treatment with letrozole was begun at 2.5 mg daily. Serial computed tomography scans demonstrated marked tumor shrinkage; after 11 months of letrozole therapy, the tumor had shrunk to less than 25% of its original volume. Further study of letrozole for high-grade uterine MMMT is warranted.     

Hysteroscopic diagnosis of malignant mixed müllerian tumor of the corpus uteri

Hysteroscopic findings of two cases with homologous type of malignant mixed müllerian tumor of the corpus uteri are described. On hysteroscopic examination, not only were nodular or polypoid lesions with fairly smooth surfaces found but also lesions with rough uneven surfaces and dilated vessels resembling the figures of endometrial carcinoma were observed. The blood vessels of lesions with fairly smooth surfaces were not dilated. Under hysteroscopy, each lesion with a smooth surface seemed to be more closely related to a benign lesion, such as an endometrial polyp or submucous myoma. The lesions with smooth surfaces corresponded to the histologically sarcoma-dominant areas and the lesions with uneven surfaces and dilated vessels were equivalent to the sites where adenocarcinoma was noted. Thus, hysteroscopic findings of this tumor well reflected the histology near the surface of the endometrium.     

Is there an association between long-term tamoxifen treatment and the development of carcinosarcoma (malignant mixed Müllerian tumor) of the uterus?

A cluster of five patients with endometrial carcinosarcoma (malignant mixed Müllerian tumor) occurred at our center during 1993; four of these had been prescribed long-term tamoxifen for breast carcinoma. Searching the archives for the 9-year period 1983–1992 revealed two further cases of uterine carcinosarcoma occurring in patients prescribed tamoxifen, from a total of 16 cases of this tumor. Five of the six patients diagnosed with carcinosarcoma who had been taking tamoxifen had been maintained on the drug for at least 6 years, while the other had a 3-year history of tamoxifen therapy. From these data we raise the question of an association between long-term tamoxifen treatment and development of endometrial carcinosarcoma, following the dramatic increase in the prescribing of this drug after 1984.     

三种化疗方案治疗晚期上皮性卵巢癌的疗效比较

目的 :确定治疗晚期上皮性卵巢癌的最佳一线化疗方案。方法 :回顾性分析 1992年 1月至 1999年 1月收治的晚期上皮性卵巢癌患者的治疗结果。对采用 PAM加 HMM(米法兰加六甲咪胺 )、PAC方案或 PC方案及 TP方案治疗的 92例患者的化疗疗效进行了比较。结果 :1TP方案的总有效率显著高于 PAM加 HMM(P<0 .0 5 ) ;2 TP方案的完全缓解率显著高于 PAM加 HMM和 PAC或 PC(P<0 .0 5 ) ;3TP方案的 2年无瘤存活率显著高于 PAM加 HMM和 PAC或 PC(P<0 .0 5 )。结论 :对晚期上皮性卵巢癌患者 ,TP方案效果优于 PAM加 HMM方案及 PAC和 PC方案 ,目前应作为首选一线方案     

Malignant mixed müllerian tumors of the ovary: experience with cytoreductive surgery and platinum-based combination chemotherapy

This study reviews the clinical outcome and prognosis of patients with malignant mixed müllerian tumors (MMMTs) of the ovary treated with optimal cytoreductive surgery, leaving no residual disease, and platinum-based chemotherapy. Ten patients diagnosed with MMMT of the ovary after complete surgical staging from February 1993 to February 2004 at Asan Medical Center in Korea were studied retrospectively. All ten patients were treated with optimal cytoreductive surgery, leaving no gross residual disease. Seven patients received ifosfamide/cisplatin chemotherapy, and the remaining three patients received other platinum-based combination chemotherapy. Demographic data, pathologic findings, treatments, and survival time were reviewed. Of the ten patients, two were scored at FIGO stage IIC, seven were at stage IIIC, and one was at stage IV. The median survival time of all ten patients was 46 months. The overall survival rate was 60.0% at 1 year, 40.0% at 2 years, and 20.0% at 5 years. Platinum-based combination chemotherapy after optimal cytoreductive surgery may be effective in the treatment of ovarian MMMT.     

Clinicopathologic analysis of uterine malignant mixed müllerian tumors

A clinicopathologic evaluation of 60 patients (median age, 66 years) presenting for primary treatment of uterine malignant mixed müllerian tumors between 1959 and 1982 was conducted. Surgical stage was utilized for assessment of survival by stage. With a minimum follow-up of 5 years, overall 2- and 5-year Kaplan-Meier survival estimates were 53 and 39%, respectively; they were 75 and 58% when disease was confined to the uterus and 27 and 15% when disease extended beyond the uterus. By log-rank and logit-rank analysis, surgical stage and depth of invasion stratified by stage were significant prognostic determinants; no significant association was found with carcinoma grade, sarcoma mitotic figure count, sarcoma histologic subtype, sarcoma necrosis, or capillary-like space involvement. No significant survival advantage was found for surgery plus irradiation or surgery plus chemotherapy compared with surgery alone after stratification according to stage. Progression-free survival after complete extirpation of macroscopic disease was not significantly different, stage for stage, between surgery alone and surgery plus radiotherapy.