Primary ovarian malignant mixed mesodermal tumor: report of four cases.

Malignant mixed mesodermal tumors (MMMTs) are highly aggressive and usually diagnosed at advanced stages. MMMT originates from either the ovary or the uterus. Because this disease is relatively rare, an optimal treatment modality has not yet been established. The authors report four cases of ovarian MMMT (one heterologous MMMT and three homologous MMMTs) during 1990-2011. The patients underwent operation immediately after histopathologically confirmation and were treated with platinum-based combination chemotherapy. The extent of operation, the outcomes of radiation therapy, and the proper chemotherapeutic regimen are still controversial. The authors report herein four cases of ovarian MMMTs alone with a brief literature review.

Ovarian malignant mixed mesodermal tumor with neuroectodermal differentiation: a multifaceted evaluation.

Malignant mixed mesodermal tumors (MMMTs) of the ovary are rare, highly aggressive neoplasms that arise most commonly in postmenopausal women. Histologically, they consist of a mixed population of malignant epithelial and mesenchymal elements. Neuroectodermal differentiation in ovarian MMMTs is exceedingly uncommon, with only a few case reports in the literature. We present a case of an ovarian MMMT with neuroectodermal differentiation in a 78-year-old female patient. Histologically, the tumor was composed of epithelial, mesenchymal, and neuroectodermal elements. The neuroectodermal component was predominantly that of a medulloepithelioma, with scattered areas displaying features of an anaplastic astrocytoma, including rare ganglion cell differentiation. The neuroectodermal component showed immunoreactivity for glial fibrillary acidic protein, synaptophysin, and S100 protein. Ultrastructurally, the neuroectodermal component was populated by cells with irregular nuclei, finely dispersed chromatin, rudimentary cell junctions, and a delicate basement membrane, all of which have been described in medulloepitheliomas. DNA ploidy analysis was also performed on the various components of the tumor and compared with 3 additional cases of MMMT without neuroectodermal differentiation and 2 ovarian immature teratomas. Our findings suggest that the neuroectodermal component may arise from a separate clone or at least evolves at an earlier stage of tumor development.

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 mesodermal tumor of the uterus in 32 year old woman]. / Mieszany guz mezodermalny trzonu macicy u 32-letniej kobiety.

A rare case of uterine's mixed mesodermal malignant tumor in young woman was described. Clinical symptoms, risk groups, treatment, and prognosis were presented. It was noticed that every year younger women are attacked by this kind of tumor.

Cytoreductive surgery plus intraperitoneal hyperthermic perfusion is an effective treatment for metastasized malignant mixed mesodermal tumours (MMMT)--report of six cases.

BACKGROUND: Malignant mixed mesodermal tumours (MMMT) of the female genital tract are rare and heterogeneous malignancies that impart grim prognosis. These tumours are characterized by an admixture of malignant epithelial and stromal elements comprising carcinomatous and sarcomatous neoplastic cells. Thus far, almost 350 cases of MMMT have been recorded in the international medical literature. Due to its rarity, there is no agreement on the best treatment strategy in women with metastasized MMMT. METHODS: Six women (mean age 59 years) with metastasized MMMT defined to the peritoneal cavity have been treated by cytoreductive surgery plus hyperthermic peritoneal perfusion plus postoperative adjuvant chemotherapy. All patients have been pre-treated by surgery for primary tumour and one by systemic chemotherapy. As cytostatics for hyperthermic peritoneal perfusion, we have used Mitomycin in a dosage of 18 mg/m2 plus Melphalan in a dosage of 25 mg/m2. As adjuvant treatment CDDP 40 mg/m2/dl, Mitomycin 7 mg/md2/dl and Ifosfamid 100 mg/kg 24 h/dl was applicated via intraaortic catheter three times with a treatment free interval of 3 weeks. RESULTS: A complete cytoreduction without remnant tumour formations in the peritoneal cavity could be carried out in all six patients. The postoperative course was uneventful in all cases except for one where a spontaneous small bowel perforation and prolonged gall secretion had to be treated by re-operation. One patient died 4 months later by pneumonia without evidence of disease. Four patients are without evidence of disease after 2, 4, 14 and 19 months, whereas one patient developed liver metastases after 9 months still treated by systemic chemotherapy. CONCLUSION: Complete cytoreduction plus hyperthermic peritoneal perfusion plus adjuvant chemotherapy seems to be an effective treatment for recurrent or metastasized MMMT. Further studies have to define the value of this new treatment strategy for this rare tumour entity.

Malignant mixed mesodermal tumours: biology and clinical aspects.

Mixed mesodermal tumours (MMTs) are relatively rare gynaecological tumours that have been poorly studied in clinical and molecular terms. They are chemosensitive (at least initially), although ultimately they have a poor prognosis. The biology of the tumour is fascinating in view of its composition of both epithelial and mesenchymal entities. We review herein the literature on the clinical and biological aspects of this malignancy.

Coexistent choriocarcinoma and malignant mixed mesodermal tumor of the uterus.

OBJECTIVE: The purpose of this article is to report a case of coexisting uterine choriocarcinoma and uterine malignant mixed mesodermal tumor (MMMT). The relevant literature is reviewed and possible pathogenesis discussed. METHODS: The clinical course and histopathology of the case were reviewed and a Medline literature search for other cases was performed. RESULTS: The patient's uterine tumor contained syncytiotrophoblastic and cytotrophoblastic cells that stained positively for the beta subunit of human chorionic gonadotrophin consistent with uterine choriocarcinoma. Pathology also revealed a second distinct neoplasm composed of adenocarcinoma admixed with sarcoma, compatible with a uterine MMMT. The patient experienced metastatic choriocarcinoma to her lungs, lymph nodes, and brain. She suffered a complicated clinical course and died 7 months after her initial diagnosis. The literature search revealed that various gynecologic and nongynecologic carcinomas with trophoblastic differentiation have been described, but an association with uterine MMMT has not been previously reported. CONCLUSIONS: Trophoblastic differentiation and choriocarcinoma associated with gynecologic and nongynecologic tumors is rare. We document the presence of uterine MMMT coexisting with uterine choriocarcinoma that followed an aggressive clinical course and review the possible pathogenesis of this lesion.

Malignant mixed mesodermal tumor arising in a benign cystic teratoma.

The occurrence of sarcoma in a benign cystic teratoma is very rare. We report the first poorly differentiated, malignant mixed mesodermal tumor with a component of rhabdomyosarcoma to arise in a benign cystic teratoma of the ovary. The tumor was staged as FIGO IC due to capsule invasion. Although combination chemotherapy of cisplatin, ifosfamide and mesna, was instituted, the disease took a rapidly progressive course. After an unusual metastasis to the scapula was detected, the patient deteriorated and died in the forth postoperative month.

Pelvic malignant mixed mesodermal tumor of uncertain origin: a case report.

Extra-uterine, and especially extragenital, malignant mixed mesodermal tumors (MMMT) are very rare. A large intrapelvic tumor resected from a 56-year-old woman was investigated with morphological and immunohistochemical methods. A large, soft and fragile tumor was located in the pelvic space. The tumor showed high cellularity and was biphasic; it consisted of an admixture of adenocarcinoma and various kinds of sarcomas. The latter were comprised of high-grade endometrial stromal sarcoma, pleomorphic sarcoma, and chondrosarcoma. The pleomorphic sarcoma showed a storiform pattern. The periodic acid-Schiff-positive eosinophilic hyaline droplets and globules in multinucleated giant cells revealed a typical ring-like or peripheral staining for alpha-1-antitrypsin and alpha-1 antichymotrypsin. We considered this case to be pelvic MMMT of uncertain origin, heterologous type.

[Malignant mixed mesodermal tumors of the ovary: a clinical analysis of 12 cases].

OBJECTIVE: To explore the clinical characteristics, treatment and prognostic factors of patients with malignant mixed mesodermal tumors of the ovary (MMMTO). METHODS: Clinical data of 12 patients with MMMTO between 1983-1997 were reviewed retrospectively. The median age of the patients was 50.7 years. According to FIGO staging system (1985), there were 4 cases in stage II, 6 in stage III and 1 in stage IV. Staging was unclear in the remaining case. Heterologous and homologous elements were present in the primary ovarian tumors in 4 and 8 of the 12 patients, respectively. All patients received cytoreductive surgery. Eleven of them received postoperative chemotherapy and 3 of these eleven patients received additional radiotherapy of pelvic field. RESULTS: The median survival of this group of 12 patients was 24 months. The 2- and 5-year survival rate was 33.3% (4/12) and 8.33% (1/12), respectively. The 2-year survival rate of stage II and III was 50% and 33.3% respectively. One patent in stage IV died within 1 year. Four of the 8 patients with homologous histology survived over 2 years while all the 4 cases with heterologous histology died within 2 years. Five of the 11 patients (45.5%) received combination therapy died within 1 year while 1 patient treated with surgery alone died within 6 months. CONCLUSIONS: The prognosis of MMMTO is rather poor. Surgery combined with chemotherapy and/or radiotherapy seems to be a better choice of approach. The histological tpyes of tumor and stage of the disease are related to prognosis.

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.

Uterine sarcoma: a clinicopathological study of 93 cases.

OBJECTIVE: To evaluate the clinical outcome of patients suffering from primary uterine sarcoma diagnosed and treated in our Hospital. SETTING: Department of Gynecologic Surgery/Gynecologic Oncology, Hospital Universitario Materno-Infantil Vall d'Hebron de Barcelona, Barcelona, Spain. SUBJECTS AND METHODS: A retrospective review from 1967 to 1995 of clinical and pathological characteristics of 93 patients with primary uterine sarcoma was done. Patients were staged using the 1988 FIGO histological classification for uterine cancer. Clinical features, type of surgery, adjuvant therapy, recurrences, distant metastasis, and survival were recorded. RESULTS: Our study included three main histologic types: 44 patients with leiomyosarcoma, 26 patients with endometrial stromal sarcoma, and 18 patients with mixed Müllerian sarcomas. The mean age for all patients was 54.8 years, and the most common symptom was vaginal bleeding. Other clinicopathological features were examined. Although surgery was the most frequent treatment, adjuvant therapies have been analyzed and discussed. The overall three-year survival rate was 67.9% and the overall five-year survival rate was 64.5%. We found statistical differences (p < 0.001) between the stage I survival rate and other stage survival rates. CONCLUSIONS: Uterine sarcoma is an uncommon neoplasia diagnosed in the 6th decade of life. Leiomyosarcoma is the most frequent histologic type (47.3%). Stage I uterine sarcoma has a better prognosis than other stages.

[Therapeutic approach in sarcoma of corpus uteri]. / Lechebnaia taktika pri sarkomakh tela matki.

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.

Mixed mesodermal sarcoma of the ovary in a young patient.

Mixed mesodermal tumors (MMT) of the ovary are rare and have a poor prognosis. This ovarian malignancy usually occurs in postmenopausal women. We report an unusual ovarian MMT in a young woman given treatment similar to one used for ovarian germ cell malignancies. We believe this is the youngest patient reported with an homologous MMT of the ovary.

Malignant mixed mesodermal tumors of the ovary.

OBJECTIVE: To review the experience at Women & Infants Hospital and Hartford Hospital of patients with malignant mixed mesodermal tumors of the ovary, and to review the pertinent literature. METHODS: Fourteen cases of malignant mixed mesodermal tumors of the ovary at the two hospitals over a 5-year period were identified through their tumor registries. Demographic data, pathology, treatment, and survival rates were reviewed. RESULTS: The median survival of the patients in our series was 7 months, with 64% dead of disease in 1 year. A review of the pertinent literature indicated median survivals of 6-12 months, with more than 70% of the patients dead of disease at 1 year, despite treatment. CONCLUSION: Further investigation is needed to determine the proper management for malignant mixed mesodermal tumors of the ovary. Meanwhile, current treatment strategies should recognize the present therapeutic limitations, so as not to diminish any further the quality of life for women with this malignancy.