[Embryonal adenorhabdomyosarcoma of the uterus. Malignant mesodermal mixed tumor of the uterus].
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A case of mixed sarcoma of the uterine cervix in a 17-year-old girl is reported. The tumor showed polypoid features resembling sarcoma botryoides, and was histologically composed of chondrosarcoma as a heterologous element and stromal sarcoma as a homologous one. Leiomyosarcoma was also found in the tumor, but striated rhabdomyoblasts were not present. No epithelial component was detected. After a total hysterectomy, the patient was given chemotherapy and is in good condition.
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The authors report a case of carcinosarcoma (malignant mesodermal mixed tumor of the urinary bladder. We discuss the histological criteria of positive and differential diagnosis, the histogenesis, as well as their biological behavior.
A 59-year-old male took total cystourethrectomy on July, 1991, since the bladder tumor recurred 2 years and 4 months after transurethral resection. Six months after total cystourethrectomy, an abnormal mass shadow appeared on the right lower lung field. Metastatic lung tumor was strongly suspected from CT scan. Despite chemotherapy, the pulmonary lesion grew rapidly and the patient died. From the autopsy, metastatic lesions were found in the bilateral lung fields, skin (face, head and abdominal wall), pleura, bilateral kidneys, small intestine and lymph nodes (para-aortic and mesenteric). The primary bladder tumor contained histologically transitional cell carcinoma as the epithelial element and sarcomatous changes with osteoid formation as the non-epithelial elements. Thus, the primary lesion was diagnosed as a malignant mesodermal mixed tumor. However, all of the metastatic lesions showed only sarcomatous changes. Only 10 cases of malignant mesodermal mixed tumor of the bladder have been reported in Japan since Fujita's report. In general, total cystectomy is necessary for the treatment of this disease. It has a poor prognosis; 5 of the 10 patients died within one year after operation.
37 patients with malignant mesodermal mixed tumor of the uterus treated from 1958 to 1987 were reviewed. It accounted for 38.5% of all uterine sarcomas during the same period. Six were homologous and 31 were heterologous in origin. The mean age was 56.8 years. 75.5% of the patients were postmenopausal. Nine (24.3%) patients had previous pelvic irradiation. There were 13 Stage I, 4 Stage II, 5 Stage III, 2 Stage IV and 13 unstaged (including 11 misdiagnosed and 2 operated in other hospital) lesions as classified according to the FIGO Staging System for malignant tumors of the Endometrium and Cervix. Twenty-six patients were treated by combined surgery and radiotherapy or chemotherapy and radiotherapy, 5 by radiotherapy alone and 6 by surgery alone. The overall 2-year survival rate was 35.1%. Twenty-two (59.5%) patients developed recurrence and/or metastasis and 86.4% (19/22) did so within 2 years after treatment. The most significant prognostic factors were: the depth of myometrial invasion, extent of lesion and method of treatment. The best 2-year survival rate (83.3%) was seen in patients treated by the combined use of surgery, radiotherapy and chemotherapy which is considered the treatment of choice for this malignancy.
A 58-year-old patient presented with poorly differentiated adenocarcinoma, probably primary in the ovary and, later in the course of her illness, with pure pleomorphic rhabdomyosarcoma. There was no evidence by light or electron microscopy of a mixture of these two tumor types. Further analysis by immunoperoxidase demonstrated scattered desmin-positive (muscle) cells in the adenocarcinoma portion of the tumor, establishing the diagnosis of malignant mesodermal mixed tumor.