Cases reported "Mixed Tumor, Mullerian"

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1/36. Malignant mixed Mullerian tumor with rhabdoid features: a report of two cases and a review of the literature.

    Rhabdoid tumors were originally described as a type of pediatric renal neoplasm that contains cells resembling rhabdomyoblasts but lacking muscle differentiation. Extrarenal rhabdoid tumors have since been reported in multiple anatomic sites in the pediatric and adult population. These tumors are characterized by an aggressive clinical course, resistance to treatment, and a rapidly fatal outcome. Eight cases of uterine neoplasms with rhabdoid differentiation have been previously reported. In the three cases where clinical follow-up was available, the patients died of disease within 3 to 17 months after the diagnosis was established. We report two cases of uterine malignant mixed Mullerian tumor (carcinosarcoma) with rhabdoid differentiation. The findings and clinical outcome confirm the aggressive nature of uterine tumors with rhabdoid differentiation. One of the patients died of disease 3 months after initial operative treatment while the other patient's tumor recurred in 1 month and she died within 10 weeks. The poor prognosis of these neoplasms makes their histopathologic recognition important.
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2/36. Collision of uterine rhabdoid tumor and endometrioid adenocarcinoma: a case report and review of the literature.

    Extrarenal malignant rhabdoid tumors have been reported in a variety of anatomic sites but infrequently in the female genital tract. In the uterus, they have been described as a pure tumor, in association with endometrial stromal sarcomas, and as a component of a malignant mullerian mixed tumor. This study reports an unusual uterine neoplasm in a 49-year-old woman, in which a malignant rhabdoid tumor occurred as a collision tumor with a well-differentiated endometrioid adenocarcinoma. The tumor was a 14-cm polypoid mass that filled the endometrial cavity. The two neoplastic components were distinct on microscopic and immunohistochemical examination. Ultrastructural examination confirmed the rhabdoid phenotype of the sarcomatous component. The patient died of disease 4 months after diagnosis with progression of the malignant rhabdoid tumor. The highly aggressive behavior of the rhabdoid (i.e., nonepithelial) component in this collision tumor lends support for a distinction of this neoplasm from a malignant mullerian mixed tumor, with which it may be confused.
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3/36. Good response of malignant mixed mullerian tumor of the ovary to paclitaxel and cisplatin chemotherapy.

    Survival of patients with advanced stage malignant mixed mullerian tumor of the ovary is poor and the best treatment remains unknown. A 62-year-old woman diagnosed with stage IIIC heterologous malignant mixed mullerian tumor of the ovary underwent optimal cytoreductive surgery followed by paclitaxel (135 mg/m2 over 24 hours) and cisplatin (75 mg/m2) every 4 weeks for 6 courses. The patient tolerated chemotherapy well, had complete clinical response and remained without disease for 21 months following diagnosis. paclitaxel and cisplatin may be effective in malignant mixed mullerian tumors of the ovary.
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4/36. Malignant mixed mullerian tumors of the ovary.

    Malignant mixed Mullerian tumor (MMMT) of the ovary is very rare, and to the best of our knowledge, only a few cases have been reported in the literature from taiwan. We report two recent cases of ovarian MMMT at our hospital. Case 1 was a 59-year-old female with stage IIIC MMMT of the ovary, with a tumor having carcinomatous and sarcomatous elements. The carcinomatous component was composed of a high-grade epithelial malignancy including serous, endometrioid, clear cell and undifferentiated carcinoma elements. The sarcomatous component was composed of a homologous malignant mesenchymal element with conspicuous hyaline globules. The patient died of the disease six months after debulking surgery. Case 2 was a 42-year-old female with ovarian stage IIC MMMT. The carcinomatous component was composed of grade II-III clear cell carcinoma and the sarcomatous component was composed of high-grade non-specific spindle cell sarcoma, which was positive for vimentin, but negative for cytokeratin, desmin and S-100 protein on immunostaining. The patient died of the disease four months after debulking surgery. These two patients both underwent hysterectomy, bilateral salpingo-oophorectomy and omentectomy and both received platinum-based chemotherapy after debulking surgery.
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5/36. Management of uterine Mullerian adenosarcoma with extrauterine metastatic deposits.

    OBJECTIVE: The aim of this study was to provide the management and outcome of three patients who presented with uterine Mullerian adenosarcoma associated with extrauterine metastases. methods: A retrospective study of three patients who were referred to our hospital was performed. One patient was referred because of vaginal metastatic deposits that were noted during investigations for primary infertility. The other two were referred because of abnormal vaginal bleeding; one of these had a large polyp protruding through her cervix into the vagina. RESULTS: In two patients the preoperative diagnosis and extent of their disease were known while in the third patient the diagnosis was only made postoperatively. All patients had a type II radical abdominal hysterectomy, bilateral salpingo-oophorectomy, and omentectomy. Two patients were given three cycles of neoadjuvant chemotherapy and pelvic irradiation over 12 weeks. Both of these patients had their diagnosis made preoperatively and the chemotherapy consisted of 240 mg/m(2) carboplatin and 80 mg/m(2) farmorubicin per cycle. The pelvic irradiation consisted of daily fractions of 1.8-Gy irradiation to a total of 45 Gy over the first 6 weeks. The other patient was given the same regime postoperatively. All patients are still alive and free of disease between 34 and 56 months. CONCLUSION: Radical surgery, chemotherapy, and irradiation provide a management option with seemingly favorable outcome for patients with uterine Mullerian adenosarcoma associated with extrauterine metastases.
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6/36. Heterologous mullerian mixed tumor after whole body irradiation because of Hodgkin's disease in stage IV.

    PURPOSE: With an incidence of 1.5% of all malignant diseases of the uterus as specified in the literature, the mullerian mixed tumor is a rarity amongst the malignancies of the female genital tract. methods: A retrospective analysis of an individual case report with the occurrence of heterologous mullerian mixed tumor years after irradiation because of Hodgkin's disease. RESULTS: This case reports describes the occurrence of a mullerian mixed tumor 12 years after the treatment of Hodgkin's disease by whole body irradiation. To our knowledge, the incidence of a mullerian mixed tumor after the treatment of Hodgkin's disease has rarely been described up to now in the literature. CONCLUSION: This case report appears to indicate the possible carcinogenic potency of radiotherapy when administered many years before. A causal connection between the administration of whole body irradiation and the development of a mullerian mixed tumor cannot be established.
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7/36. Long-term tamoxifen treatment: a possible aetiological factor in the development of uterine carcinosarcoma: two case-reports and review of the literature.

    Two cases of uterine carcinosarcoma developing after long-term tamoxifen (TAM) treatment are presented. The patients, 67 and 72 years old, were treated with TAM for 6 and 7 years, continuously. They both developed an heterologous malignant mixed Mullerian tumor (mmMt). At laparotomy, an advanced stage of disease was found with peritoneal spread. In spite of the surgical and the postoperative treatment, they both died of disease, 3 and 10 months later. There are only 10, well documented, similar cases reported. Another 7 were identified in series of uterine malignancies developing after TAM treatment. Considerable evidence suggests that mmMt represents an epithelial cancer with sarcomatous dedifferentiation. Prolonged (> 5 years) TAM treatment may represent a causative factor in the development of this highly lethal disease (80% of the reported patients had a dismal prognosis). Large uterine polyps with special histological features, may represent an intermediate step in the tumor formation. Close follow-up of the patients is warranted.
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8/36. Mullerian adenosarcoma of the uterus: case report and review of literature.

    Mullerian adenosarcoma--a variant of mullerian mixed mesodermal tumor of the uterus--is typically composed of benign but sometimes mildly atypical glandular epithelial elements admixed with malignant sarcomatous stroma. This rare tumor, which accounts for only about 8% of all uterine sarcomas, usually originates in the endometrium and grows as a polypoid mass within the endometrial cavity. The most prevailing presenting symptom is abnormal vaginal bleeding and the most common finding is a polypoid mass protruding through a dilated cervical canal. The case of a woman, who at age 62 presented with symptoms and signs of acute pelvic inflammatory disease and on vaginal examination an infected mullerian adenosarcoma protruding through a dilated cervical canal was discovered, is reported. Treatment consisted of extensive antibiotic treatment and surgery comprised of total abdominal hysterectomy and bilateral salpingo-oophorectomy followed by postoperative adjuvant pelvic radiotherapy. One year later, the patient is alive with no evidence of disease.
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9/36. Primary peritoneal malignant mixed Mullerian tumors. A clinicopathologic, immunohistochemical, and genetic study.

    BACKGROUND: Primary peritoneal malignant mixed Mullerian tumors (MMMTs) are rarely reported in the literature. methods: The clinical, pathologic, and immunohistochemical features of five cases of MMMT of female peritoneum were analyzed. The tumors were also investigated for expression of hormone receptors, specific BRCA-1 mutations, and clonality. RESULTS: The patients' ages ranged from 33 to 67 years. They presented with abdominal pain or mass. One case of peritoneal MMMT was associated with a synchronous endometrial carcinoma whereas another case was detected 2 years after the diagnosis of a primary adenocarcinoma of the fallopian tube. One patient died 1 month after diagnosis whereas 2 patients died with disease within 1 year. Both carcinomatous and sarcomatous elements are present in all the tumors. Squamous differentiation was noted in two cases. Heterologous elements, including chondroid, rhabodomyoblastic, and osteoid differentiation were detected in all tumors. Immunohistochemical studies confirm the biphasic differentiation with variable demonstration of neural and smooth muscle differentiation. All five MMMTs were negative for estrogen and progestogen receptors although the related endometrial and tubal carcinomas were positive. heteroduplex analysis used to screen for specific BRCA-1 mutations were negative in all five MMMTs. Clonality study of the two MMMTs found in association with endometrial carcinoma and tubal carcinoma was inconclusive. CONCLUSIONS: Our study confirmed that primary peritoneal MMMTs were aggressive tumors with poor prognosis. The presence of synchronous or metachronous genital carcinomas suggests multifocal tumorigenesis from tissue of same embryologic origin. The lack of hormone receptor in these tumors indicates deviation from hormonal control. Specific BRCA-1 mutations found in ovarian carcinoma in Chinese patients could not be detected in our series.
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keywords = fallopian tube, tube, disease
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10/36. Malignant mixed mullerian tumor of the ovary: report of four cases.

    INTRODUCTION: Malignant mixed mullerian tumor (MMMT) of the ovary is an extremely rare gynaecologic neoplasm that represents 1% of the malignances of this organ. Stage I disease is rare because it is asymptomatic in early stage. We describe four cases. case reports: In the Department of obstetrics and gynecology of the University of Bari four cases of MMMT of the ovary were diagnosed. Three patients were in stage IIIC and one of them was a homologous MMMT; the fourth patient was affected by a heterologous stage IV MMMT. All women were treated with surgery and chemotherapy. Two patients are alive 14 and 12 months after diagnosis. The other two died after 37 months and one month, respectively. CONCLUSIONS: The malignant mixed mullerian tumor (MMMT) of the ovary is a particularly aggressive tumor, especially in advanced stages. The survival rate is very low in spite of surgery, chemotherapy and radiotherapy. The optimal treatment for this neoplasm is unknown because of its rarity. Our experience, when considering survival, seems to confirm the use of cisplatin and ifosfamide and to give new horizons to taxol.
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