Cases reported "Mixed Tumor, Mullerian"

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1/28. 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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2/28. Synchronous genital tract neoplasms.

    Synchronous genital tract neoplasms constitute a more common clinical problem than would be generally expected. This case focuses on mixed mullerian tumours and postulates a mechanism for an increased incidence found associated with synchronous genital tract neoplasms.
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3/28. 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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4/28. Cytological diagnosis of malignant mixed mullerian tumor of the uterus in ascitic fluid.

    The malignant mixed Mullerian tumour (MMMT) is a rare and aggressive neoplasm of the uterus, seen in postmenopausal women. In this case, an uncommon neoplasm was diagnosed cytologically in the ascitic fluid of a woman 58 years old and was confirmed histologically after hysterectomy and bilateral adnexectomy.
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5/28. 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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6/28. Primary mesenteric malignant mixed mesodermal (mullerian) tumor with neuroendocrine differentiation.

    Extragenital malignant mixed mesodermal (mullerian) tumors (MMMT) are rare neoplasms, with but 24 well documented cases in the literature. Neuroendocrine differentiation in mixed mullerian 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.
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7/28. Villoglandular adenocarcinoma of the vulva.

    BACKGROUND: Only two previous cases of villoglandular adenocarcinoma of the vulva, an entity morphologically similar to tumors found in the uterine cervix and colorectum, have been reported. This paper communicates the first complete immunohistochemical study in villoglandular adenocarcinoma in order to determine its phenotype and histogenesis. CASE: A 69-year-old woman had a 1.5-cm nodule in the right labium majus. Histologically, it corresponded to a minimally atypical, villoglandular adenocarcinoma with a small microinvasion. Immunohistochemically, it was positive to OC125, CEA, and OC19.9 and coexpressed cytokeratins 7 and 20. Chromogranin, nuclear estrogen, and progesterone receptors were negative. CONCLUSION: Phenotypic expression was more consistent with a papillary mucinous ovarian or cervical neoplasm than of a colonic one. Its behavior was similar to that of its morphologic counterpart in the cervix, since the patient had no recurrence 3 years after a wide local excision.
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8/28. Mullerian adenosarcoma of the cervix with heterologous elements: report of a case and review of the literature.

    BACKGROUND: Mullerian adenosarcoma (MA) is a rare neoplasm composed of benign epithelial and malignant stromal components. Its location in the cervix and the presence of heterologous elements are extremely infrequent (to our knowledge, only 14 more cases have been reported in the English literature). We describe another case of MA of the uterine cervix with heterologous elements and review the clinical and pathological features of these tumors. CASE REPORT: A nulliparous 25-year-old woman was admitted to the hospital because of metrorrhagia and recurrent masses, dependent on the cervix, initially considered endocervical polyps. Microscopically, the last curettage performed revealed a tumor composed of two elements, epithelial and mesenchymal. The epithelial elements were benign endocervical type glands, and the mesenchymal were sarcomatous, containing minor foci of cartilage. A diagnosis of endocervical heterologous adenosarcoma was reached, and a total hysterectomy and bilateral salpingo-oophorectomy were performed. The patient is alive 2 years after the surgical procedure. Neither chemotherapy nor other adjuvant therapies have been administered, and she is clinically free of disease at the moment. CONCLUSION: Because MA with heterologous elements seems to appear at the earliest stages of the reproductive lifespan in women, commonly with a history of recurrent polyps, and because its malignant potential is uncertain, gynecologists and pathologists should be aware and think about the possibility of this tumor. Long-term follow-up is essential and so is the accumulation of individual cases to provide further experience with these unusual neoplasms.
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9/28. Malignant mixed Mullerian tumor of the uterine cervix with neuroendocrine differentiation.

    Malignant mixed Mullerian tumors (MMMTs) are rare neoplasms. We report the clinical, pathologic, and immunohistochemical features of an MMMT primary of uterine cervix. This lesion was composed of a poorly differentiated epithelial component (cytokeratin positive) and a spindle cell component (vimentin positive) with heterologous (myoblastic) differentiation (focal 1A4 positive). There were also cells with neuroendocrine features that expressed S-100 and chromogranin a. Along with a brief review of this amazing neoplasm, some histogenetic concepts relevant to this case are discussed. To our knowledge this is the first report of a malignant mixed Mullerian tumor of the uterine cervix with neuroendocrine differentiation.
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10/28. Malignant mixed mullerian tumor of the ovary and false negative punctures.

    Malignant mixed mullerian tumour (MMMT) of the ovary is a rare and aggressive tumour with a poor prognosis. We present a case of a 57-year-old woman with a large pelvic mass, omental cake, ascites and pleural effusions, clinically highly suspect of an ovarian neoplasm. paracentesis and ultrasound-guided biopsy of the ovary were negative for malignant disease. Therefore a CT-guided true cut biopsy was performed. The latter gave a histopathologic diagnosis of an endometrioid adenocarcinoma of the ovary. However after cytoreductive surgery anatomopathologic examination revealed a malignant mixed mullerian tumour of the ovary with heterologous differentiation. Apparently only one of the two components was found in the puncture. Adjuvant chemotherapy, active against the sarcomatous and the carcinomatous component, was given. At present the patient is well and disease free 35 months after the initial diagnosis. Cytological examination of ascites may be negative in the presence of malignant disease. If a tumour consists of two components, puncture can miss one, which may lead to undertreatment. punctures should be discouraged as a diagnostic tool in patients in whom an ovarian malignancy is suspected.
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