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1/158. dna mismatch repair deficiency in curatively resected sextuple primary cancers in different organs: a molecular case report.

    A male patient synchronously or metachronously underwent six curative resections after diagnoses of cancers in the rectum, urinary bladder, stomach, colon, liver and lung. Five cancers, excluding early colon cancer, were analyzed for instability in seven microsatellite markers and in transforming growth factor beta type II receptor, insulin-like growth factor ii receptor and BAX. All analyzed cancers had replication errors and instability in at least one target gene. These results suggest that abnormal dna mismatch repair system plays a major role in the occurrence of multiple primary cancers in this case.
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ranking = 1
keywords = sex
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2/158. Villoglandular papillary adenocarcinoma of the uterine cervix.

    Villoglandular papillary adenocarcinoma of the uterine cervix was recently (1989) described by three main histological features: exophytic proliferation, papillary architecture and mild to moderate cellular atypicality. The authors report a case of villoglandular papillary adenocarcinoma, clinical stage IB, which was peculiar because of its association with a co-existing and simultaneously discovered invasive squamous cell carcinoma. These two patterns were juxtaposed and not intermingled. The patient was treated with radical hysterectomy followed by vaginal radiation therapy. She remains without evidence of recurrence after 12 months of follow-up. Five main clinicopathological features of the villoglandular papillary adenocarcinoma could be stressed: rare histological variant (72 described cases), young age of patients (25-45 years old), superficial stromal invasion, usual association with other tumoral patterns (in situ or invasive adenocarcinoma as well as in situ or invasive squamous cell carcinoma) and excellent prognosis. For selected cases, a conservative surgical approach (cervical conization) was possible.
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ranking = 21.17198177994
keywords = stromal
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3/158. 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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ranking = 21.17198177994
keywords = stromal
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4/158. Cytokeratin immunoreactivity in Ewing's sarcoma: prevalence in 50 cases confirmed by molecular diagnostic studies.

    Ewing's sarcoma (ES) and primitive neuroectodermal tumor (PNET) are characterized by the presence of the specific t(11;22)(q24;q12) or variants thereof, producing diagnostic EWS fusion transcripts. Cytokeratin has been reported sporadically to be expressed in some cases of ES/PNET. However, its prevalence has not been assessed systematically in a series of cases with confirmatory molecular or cytogenetic evidence of a diagnostic translocation. We present in detail three index patients in whom strong cytokeratin immunoreactivity was a confounding factor in the diagnosis. To establish further the prevalence of cytokeratin immunoreactivity in a series of well-characterized ES/PNET, we then performed immunohistochemical studies with antibodies CAM5.2 and AE1/AE3 on 50 cases of ES/PNET diagnosed at Memorial Sloan-Kettering Cancer Center in which molecular evidence of a specific ES/PNET-associated translocation were available. Immunoreactivity to cytokeratin was present in 10 cases (20%), in five diffusely and five focally. There was no significant association between cytokeratin expression and the following parameters: patient age, sex, skeletal and extraskeletal primary site, and the type of EWS fusion transcript. Cytokeratin expression, a manifestation of epithelial differentiation, is present in as many as 20% of ES/PNET in either a diffuse or focal pattern.
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ranking = 0.25
keywords = sex
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5/158. Pleomorphic adenoma (benign "mixed" tumor) of the human female breast. Case report.

    A case of solitary pleomorphic adenoma, ("mixed" tumor of salivary gland type) of the left breast associated with the right breast fibroadenoma in 43-year-old woman is reported. The paper describes clinical, cytological, immunohistological and pathological findings in this case and indicates the importance of separating this benign entity from malignances with stromal metaplasia.
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ranking = 21.17198177994
keywords = stromal
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6/158. Synchronous occurrence of epithelial and stromal tumors in the stomach: a report of 6 cases.

    OBJECTIVE: The synchronous development of epithelial and stromal tumors in the stomach has been reported rarely in the literature. A series of 6 such cases is described in this article. methods: Clinical and pathologic data were recorded and the literature was reviewed. RESULTS: Five cases featured the simultaneous occurrence of stromal tumors (1 benign, 3 borderline, 1 malignant) and adenocarcinomas, whereas the stromal tumor in the sixth case was found in association with a carcinoid. No collision tumors were observed. In 2 cases, tumors arose from the same site and were closely juxtaposed, but in 4 patients they developed from different areas of the stomach. A preoperative histologic diagnosis of both tumors was not achieved in any case. Two patients harbored occult infiltrative epithelial lesions (1 diffuse-type adenocarcinoma, 1 carcinoid), which were detected only at pathologic examination of the gastric mucosa adjacent to the stromal tumor. CONCLUSIONS: The simultaneous occurrence of epithelial and stromal tumors in the stomach can be less rare than usually expected. Coincidence alone could account for such an association, particularly in areas with high incidence rates of gastric cancer. The hypothesis that a single carcinogenic agent might interact with two neighboring tissues in the stomach inducing the development of tumors of different histotype cannot be theoretically discarded.
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ranking = 190.54783601946
keywords = stromal
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7/158. Sinonasal teratocarcinosarcoma: ultrastructural and immunohistochemical evidence of neuroectodermal origin.

    The authors report a case of sinonasal teratocarcinosarcoma in a 37-year-old man, which was located in the anterior skull base and extended to the right nasal cavity and paranasal sinuses. The tumor was surgically resected twice, but it could not be removed completely. Microscopically, it was mainly composed of primitive cell nests within a moderately cellular stroma. The components of squamous cell epithelia with focal teratoid appearance and adenocarcinomatous differentiation were observed. There were many rhabdomyoblasts scattered in the nests and stroma. Ultrastructurally, the primitive cells had many neural processes with parallel microtubules, resembling olfactory neuroblastoma. Rhabdomyoblasts showed various degrees of skeletal muscle differentiation. Some of the stromal spindle cells had actin filaments with dense patches and dense core granules. Immunohistochemically, the primitive cells were positive for epithelial markers, neuron-specific enolase, synaptophysin, and myogenic regulatory proteins. The rhabdomyoblasts showed immunoreactivity for myoid markers, cytokeratin, epithelial membrane antigen, and synaptophysin. Most of the stromal spindle cells were positive for smooth muscle actin, neuron-specific enolase and synaptophysin. The immunohistochemical and ultrastructural findings suggest that primitive cells had the most primitive phenotype of placodes, and support the possibility that sinonasal teratocarcinosarcoma is essentially a neuroectodermal tumor with divergent differentiation.
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ranking = 42.343963559879
keywords = stromal
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8/158. Effects of leuprolide acetate on low-grade endometrial stromal sarcoma.

    We describe a low-grade endometrial stromal sarcoma coexistent with leiomyoma and adenomyosis treated with leuprolide acetate. We describe its histologic characteristics and clinical significance.
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ranking = 105.8599088997
keywords = stromal
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9/158. endometriosis-associated intestinal tumors: a clinical and pathological study of 6 cases with a review of the literature.

    This clinicopathologic study of primary Mullerian tumors of the bowel arising in foci of endometriosis is based on six new cases and an analysis of 17 previously reported cases. Varieties of Mullerian tumors occur in the bowel; the most common types are endometrioid carcinoma, followed by various mixed Mullerian tumors and stromal sarcomas. Seventy-eight percent develop in the rectosigmoid colon, the remaining in the cecum or ileum. Those in the latter area tend to be sarcomas or mixed Mullerian tumors. Certain architectural growth characteristics, derived from precursor endometriosis, are common to most endometriosis-associated intestinal tumors (EAITs). Seventy percent of EAITs occur in the outer bowel wall. Transmural tumors tend to form luminal polyps and assume an hourglass shape. Metachronous or synchronous Mullerian tumors occur in 39% of cases. Seventy percent of women with EAITs are in their mid 30s to early 50s. Common presenting symptoms are abdominal or pelvic pain, melena, and an abdominal or pelvic mass. Documented in 26% of patients is a history of prolonged unopposed estrogen therapy. Only 28.5% of cases die of their tumors, but follow-up is less than 5 years in all but 2 patients.
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ranking = 21.17198177994
keywords = stromal
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10/158. Blue rubber bleb nevus syndrome.

    The blue rubber nevus syndrome consists of multiple venous malformations in the skin and gastrointestinal tract associated with intestinal hemorrhage and iron deficiency anemia. Other organs may be involved. The causes of this syndrome are unknown. Its most common presentation is in the form of sporadic cases, but dominant autosomal inheritance has been described. It is a condition that affects both sexes equally, and its occurrence is rare in the black race. We present a case of this syndrome diagnosed in a 11-year-old patient. He had severe anemia and a venous swelling on the trunk. Similar lesions were found in the stomach, bowel, and on his foot. We emphasize the main clinical aspects: intestine, eyes, nasopharynx, parotids, lungs, liver, spleen, heart, brain, pleura, peritoneum, pericardium, skeletal muscles, bladder, and penis lesions, systemic complications that may occur to these patients which are thrombosis and calcification, as well as consumptive coagulopathy and thrombocytopenia that may occur within the nevi.
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ranking = 0.25
keywords = sex
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