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Malignant mixed mesodermal ovarian tumor treatment and prognosis: a 20-year experience.

Mixed mesodermal sarcoma of the ovary is a rare clinical entity. To review the epidemiology, prognostic factors, and treatment results related to primary ovarian sarcoma at our center, a retrospective chart review of all patients referred for ovarian cancer was carried out from 1974 to 1994. Cases with confirmed pathologic diagnosis of primary mixed mesodermal ovarian sarcomas were selected, forming the present study group. Thirty-six charts were identified. The median age at presentation was 67.5 years. Findings at laparotomy demonstrated extraovarian metastasis in 33/35 patients. Total abdominal hysterectomy and bilateral salpingo-oophorectomy +/- omentectomy were performed in 34 patients, with 22 patients left with macroscopic residual disease after surgery. Follow-up adjuvant chemotherapy consisting of cisplatin and doxorubicin was administered to 29/36 patients. Follow-ups ranged from 1 to 11 years with a median of 2 years. As with epithelial ovarian cancer, residual disease after initial surgery is an important prognostic factor. Thirteen patients had a second-look laparotomy. Five patients were positive for disease. Eight patients, one of whom recurred, were histologically negative. The patients with positive second-look findings, as well as all those who recurred clinically, subsequently died within 12 months despite trials with different second-line chemotherapeutic agents. Survival analysis showed a median survival of 3 years among patients treated with combination cytotoxic chemotherapy. Primary ovarian sarcomas make up about 2-3% of all ovarian cancer cases seen in our center. These are often very aggressive tumors with widespread metastasis at the time of presentation, making optimal tumor debulking difficult. The combination of cisplatin and doxorubicin appears to have activity resulting in a survival of 35% at 5 years. Second-look surgery offers little helpful information on the management of these tumors.

Adult↗

Systemic therapy for advanced uterine sarcoma: a systematic review of the literature.

OBJECTIVE: To conduct a systematic review of the literature regarding the systemic treatment of advanced uterine sarcoma and provide an evidence-based summary of the available literature. METHODS: MEDLINE, EMBASE, and the Cochrane Library databases were searched. "Uterine sarcoma," "leiomyosarcoma," "mixed mesodermal tumor," "chemotherapy," and "systemic therapy" were combined with the search terms for study designs. RESULTS: Three randomized controlled trials and 24 prospective phase II trials were included in the systematic review. In a randomized trial of doxorubicin versus doxorubicin plus cyclophosphamide for advanced or recurrent uterine sarcoma, doxorubicin produced an overall response rate (RR) of 19% and median survival of 11.6 months, which was similar to the response with combination chemotherapy (RR 19%, median survival 10.9 months). A randomized trial comparing ifosfamide plus cisplatin versus ifosfamide alone in mixed mesodermal tumors showed a significant improvement in RR and progression-free survival with the combination compared with ifosfamide alone, however, the combination was associated with increased toxicity including death. A randomized trial comparing doxorubicin to doxorubicin with dacarbazine in women with advanced or recurrent uterine sarcoma demonstrated a significantly higher RR with the combination (P < 0.05), but no significant difference in survival. CONCLUSIONS: Offering palliative chemotherapy to patients with advanced, unresectable uterine sarcoma who are symptomatic from this disease is a reasonable decision. Doxorubicin is an option for women with advanced uterine sarcoma. The combination of cisplatinum and ifosfamide is also an option for women with metastatic mixed mesodermal tumors; however, this combination is associated with significant toxicity when compared to ifosfamide alone.

Clinical Trials, Phase II as Topic↗

Primary mesenteric malignant mixed mesodermal (müllerian) tumor with neuroendocrine differentiation.

Extragenital malignant mixed mesodermal (müllerian) tumors (MMMT) are rare neoplasms, with but 24 well documented cases in the literature. Neuroendocrine differentiation in mixed müllerian neoplasms has been mentioned only anecdotally. We report on the clinical, pathological, and immunohistochemical features of a hitherto-undescribed extragenital MMMT with prominent neuroendocrine differentiation arising from the jejunal mesentery. This lesion was composed of a poorly differentiated epithelial component and a spindle cell component with heterologous (rhabdomyoblastic) differentiation. The bulk of the tumor consisted of small cell neuroendocrine carcinoma, which exhibited strong immunoreactivity for NSE, LEU-7, chromogranin A and synaptophysin. Electronmicroscopy confirmed the presence of neurosecretory dense-core granules. The primary mesenteric origin of the tumor was established at autopsy. Along with a brief review of previously reported extragenital MMMT some histogenetic concepts relevant to this case are discussed.

Aged↗

[Therapeutic approach in sarcoma of corpus uteri].

The results of treatment of 419 patients with endometrial sarcoma are presented. Five-year survival was 42.7% and it appeared to depend on histological pattern substantially: leimyosarcoma--49.5; endometrial stromal sarcoma--43.5; mixed mesodermal tumors--40.2% (carcinosarcoma included--26.4%). With localized tumors (stage I) of all histological patterns, survival was 3 times (58.9%) that in cases of cervix uteri involvement (19.4%). The recommendations for treatment of endometrial sarcoma are given: uterine extirpation with adnexa in patients with leimyosarcoma and a modified extended extirpation of the uterus for mixed mesodermal tumors. Radiotherapy is recommended for all patterns of tumor, except for leimyosarcoma. Adjuvant chemotherapy will increase the chances of better prognosis.

Chemotherapy, Adjuvant↗

[Uterine mixed malignant mesodermal tumors--a case with an unusual distribution of components].

In a 67-year old woman diagnosis of uterine carcinosarcoma was settled according to curettage biopsy but papillary endometrioid adenocarcinoma pattern prevailed in the resected large tumour. An extensive search was needed to find a focal heterologous sarcomatous component in the tumour center. A more detailed microscopical investigation of some presumed uterine carcinomas may succeed in the same way.

Aged↗

[Mesodermal (Muller) mixed ovarian tumor].

A case of mesodermal (Müller) mixed tumor of the ovary is described. The tumor consisted of the endometrial component with benign epithelial structures, stromal sarcoma, rhabdomyosarcoma, and elements of undifferentiated sarcoma. By its histological structure the tumor may be classified as one of the variants of mesodermal (Müller) mixed tumor of the heterologous adenosarcoma type.

Adult↗