Cases reported "Peritoneal Neoplasms"

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1/71. Pseudomesotheliomatous carcinoma involving pleura and peritoneum: A clinicopathologic and immunohistochemical study of three cases.

    Pseudomesotheliomatous carcinoma is a rare variant of peripheral adenocarcinoma of the lung that can manifest clinical, radiologic, and pathologic features similar to malignant mesothelioma. We present three patients with pseudomesotheliomatous carcinoma of the lung. In one patient the carcinoma extended beyond the thorax and extensively involved the peritoneum, mesentery, omentum, and intestines. All patients experienced weight loss and chest pain. All were white men aged 63, 65, and 67 years. Two were smokers and had shortness of breath, cough, and pleural effusion. One had a history of asbestos exposure. No patient developed dyspnea or hemoptysis. One was successfully treated for prostatic carcinoma 18 months earlier. Radiographically, all tumors were pleura-based. Grossly, the tumors spread extensively over pleural (and in one case peritoneal) surfaces and mimicked malignant mesothelioma. Histologically, all tumors were poorly differentiated and necrotic; two tumors exhibited spindle-cell components and desmoplasia. Mucin production was detectable in none, 10%, and 50% of tumor cells. The percentages of tumor cells immunoreactive for Ber-EP4 were 70%, 100%, and 80%; for Leu MI 0%, 90%, and 50%; for epithelial membrane antigen 80%, 80%, and 100%; for B 72.3%, 0%, 90%, and 20%; for polyclonal carcinoembryonic antigen 0%, 10%, and 10%; and for monoclonal 5%, 0%, and 0%. Of these, Ber-EP4 and B 72.3 rendered the most reliable diagnostic results. The clinical, radiologic, and gross and routine histologic findings were similar to those of a malignant mesothelioma; the final diagnosis could be made based mainly on immunocytochemical results. We have reviewed the English and German literature regarding 65 such tumors and present our experience with three additional cases. We emphasize the application of immunocytochemical studies on pleura-based poorly or undifferentiated malignant tumors of unknown origin.
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2/71. pseudomyxoma peritonei with high serum CA19-9: report of three cases.

    pseudomyxoma peritonei (PMP) is an unusual form of intraabdominal neoplasm that produces a large amount of extracellular mucin. It is often associated with mucinous tumors of gastrointestinal tract or ovary. Herein, we report 3 patients with pseudomyxoma peritonei with high serum carbohydrate antigen 19-9 (CA19-9) levels. The first patient, who had a CA19-9 level of 1132 U/ml, had well-differentiated rectal cancer and died of chemotherapy complications, pneumonia and septic shock; one month after admission. The other 2 cases with CA19-9 levels of 2520 U/ml and 679 U/ml had tumors of unknown origins and had survived more than 1 year and 3 months after treatment, respectively. Usually, elevated serum CA19-9 levels are found in patients with pancreatic, biliary, colorectal, gastric or liver cancers. However, many studies have shown high serum CA19-9 levels are associated with mucinous carcinoma. Immunochemical studies also showed positive staining of CA19-9 in mucinous tumors. PMP is composed of large amounts of mucin, therefore, we suggest that serum and ascites CA19-9 levels should be routinely checked in patients with PMP.
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3/71. Retroperitoneal peripheral hemangioblastoma: a case report and review of the literature.

    central nervous system hemangioblastomas are uncommon tumors of controversial etiology that are usually found in the posterior fossa of the cranial cavity, retina, and spinal cord. Peripheral involvement is rare; only isolated case reports have been identified. We report an unusual case of hemangioblastoma involving the retroperitoneum. A 47-year-old African-American man presented with polycythemia on routine laboratory testing. Computed tomography revealed a large retroperitoneal mass near the pancreas, in a left suprarenal location, without adrenal involvement and without attachment to a nerve. Although hemangioblastoma may be associated with the von Hippel-Lindau syndrome, this patient did not have any of the stigmata of this disease. The histologic features included a highly vascular tumor with cellular areas composed of plump, pleomorphic spindled and epithelioid (stromal) cells with variable cytoplasmic lipid vacuoles and hypocellular areas with inflammatory cells and collagenous fibrils. Immunohistochemical staining showed that the tumor (stromal) cells were positive for vimentin, calponin, S-100 protein, neuron-specific enolase, and CD57 and negative for glial fibrillary acidic protein, cytokeratins, epithelial membrane antigen, CD34, HMB-45, desmin, and the actins. These morphologic and immunohistochemical findings are consistent with hemangioblastoma. To our knowledge this is the first reported case of a hemangioblastoma in this location. Based on this case we conclude that hemangioblastoma may occur in the retroperitoneum and outside of the central nervous system in a patient without von Hippel-Lindau syndrome. The immunoprofile of this case suggests that hemangioblastomas are mesenchymal neoplasms exhibiting both neural and myofibroblastic differentiation.
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4/71. Biphasic intra-abdominal desmoplastic small cell tumor in a patient with proximal spinal muscular atrophy.

    A case is reported of intra-abdominal desmoplastic small cell tumor (IDSCT) with biphasic histologic features in a patient with proximal spinal muscular atrophy. The tumor was composed of small epithelial cell nests with spindle cell sarcomatous areas. Both areas were surrounded by a desmoplastic stroma. Immunohistochemical studies revealed reactivity for low molecular weight cytokeratin, epithelial membrane antigen, vimentin, desmin and Leu-7 in both areas. Electron microscopic examination demonstrated paranuclear aggregates of intermediate filaments, zonula adherens and basement membrane-like material in the epithelial cells, while spindle cells in the tumor had fewer intracytoplasmic organelles. However, intermediate or transitional forms of both types of tumor cells were frequently observed. Although IDSCT are known to express multi-phenotypes immunohistochemically, attention should be paid to the broad spectrum of cell morphology in these tumors.
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5/71. Recurrent prostate carcinoma presenting as omental large cell carcinoma with neuroendocrine differentiation and resulting in bowel obstruction.

    Neuroendocrine differentiation in the neoplastic prostate varies from foci of adenocarcinoma showing immunoreactivity to the pure small cell carcinoma, which correlates with poor prognosis. Widely metastatic disease in unusual sites is reported for small cell carcinoma, and rarely is the serum prostate-specific antigen level elevated. We report a case of recurrent prostate adenocarcinoma presenting as bowel obstruction due to widespread metastatic disease in the omentum and peritoneum. The histopathology of the omental metastasis was that of a large cell neuroendocrine carcinoma, without evidence of an adenocarcinoma. The absence of a clinically evident second primary tumor, the concomitant elevated serum prostate-specific antigen level, and the positive tissue immunoreactivities to prostatic markers all supported the prostatic origin of the omental tumor. review of the importance of prostatic neuroendocrine differentiation and its unusual metastatic patterns is presented.
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6/71. 'Mucin-positive' epithelial mesothelioma of the peritoneum: an unusual diagnostic pitfall.

    AIMS: The histopathological, immunohistochemical and ultrastructural features of a primary 'mucin-positive' epithelial mesothelioma of the peritoneum are reported to draw attention to a potential cause of diagnostic error. methods AND RESULTS: light microscopy showed an infiltrative neoplasm within the gastric wall and omentum which was composed of sheets of 'signet- ring' tumour cells and contained abundant diastase-resistant periodic acid-Schiff-positive material. immunohistochemistry supported a mesothelial phenotype (cytokeratin AE1/3, thick membranous HBME-1, focal thrombomodulin and calretinin expression and no reactivity for carcinoembryonic antigen, Leu-M1 and Ber-EP4). Ultrastructural features showed large cells with prominent intercellular desmosomes and numerous delicate, elongated microvilli. Within intracytoplasmic neolumina, crystalloidal 'fern-like' bodies were identified with features similar to the ultrastructural appearances of hyaluronic acid crystals. Repeat histochemical analysis following hyaluronidase pretreatment revealed a significantly diminished diastase-resistant periodic acid-schiff reaction. CONCLUSIONS: This case of primary peritoneal 'mucin-positive' epithelial mesothelioma demonstrates morphological and histochemical mimicry with diffuse gastric adenocarcinoma. A similar case has not been previously reported in the peritoneum and an awareness of the tumour (with application of suitable ancillary studies) prevents misdiagnosis and assists in potentially difficult medicolegal cases.
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7/71. Morphologic-cytogenetic analysis of dedifferentiated liposarcomas with an extensive misleading leiomyosarcomatous component.

    This report describes two cases of recurrent retroperitoneal dedifferentiated liposarcoma characterized by an extensive leiomyomatous component that prevented the correct diagnosis before the last recurrence. Strong immunoreactivity with smooth muscle and desmin antibodies and ultrastructural features consistent with leiomyosarcoma were observed in the spindle-cell and/or myxoid-like components in all four recurrences in case 1, and in the spindle-cell component of the primary tumor and the first recurrence in case 2. In case 1, the correct diagnosis was suggested by the cytogenetic evidence of ring markers, a hallmark of well-differentiated/dedifferentiated liposarcoma. In case 2, tumor type was yielded mainly by the morphology of the second recurrence, which consisted entirely of a well-differentiated liposarcoma, a sclerosing inflammatory variant, as confirmed by the karyotype. Reevaluation of the first two surgical specimens of each case revealed small areas consistent with well-differentiated liposarcoma that had been previously overlooked. Despite the smooth-muscle antigen profile, both cases retained an mdm2 /p53 /cdk4 immunophenotype consistent with the genotype.
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8/71. Early localized peritoneal mesothelioma as an incidental finding at laparoscopy. Report of a case and implications regarding natural history of the disease.

    BACKGROUND: Peritoneal mesothelioma is regarded as a fatal disease that presents with progressive ascites in a relatively late stage of its natural history. To the authors' knowledge, prior published articles have not described the early manifestations of this cancer. methods: A 30-year-old asymptomatic woman underwent laparoscopy for an infertility workup. Nodules noted in the pelvis were biopsied and determined to be mesothelioma. Standard immunohistochemical studies were performed. Cytoreductive surgery and heated intraoperative intraperitoneal chemotherapy were used for treatment. RESULTS: Multiple (approximately 30) tumor nodules up to 2 mm in dimension and limited to the pelvis were observed and resected. No primary tumor focus was evident. These tumor nodules stained positive for Calretinin and negative for carcinoembryonic antigen immunohistochemically. CONCLUSIONS: In this patient, no incidence for transcoelomic dissemination of mesothelioma from a single primary site was observed. Rather, this patient's clinical presentation suggested that mesothelioma may be multifocal in origin within a limited region of the peritoneal cavity. This hypothesis may support a rationale for aggressive local-regional management of selected patients in whom peritoneal mesothelioma is of limited distribution and mass.
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9/71. CD138-positive and Kaposi's sarcoma-associated herpesvirus (KSHV)-negative B-cell lymphoma with serosal spreading of the body cavity and lymphadenopathy: an autopsy case.

    CD138-positive and Kaposi's sarcoma-associated herpes virus (KSHV)-negative B cell lymphoma with serosal spreading of the body cavity and lymphadenopathy is presented. Our lymphoma cells showed pleomorphic morphology and a clonal immunoglobulin gene rearrangement. Immunophenotypically, they lacked B- and T-cell-associated antigens but expressed strong membranous CD138 antigen along the serosa. Although our case was not conventional primary effusion lymphoma (PEL) because of the absence of KSHV and the presence of lymphadenopathy, its unique phenotype and serosal spreading were consistent with those of PEL. Our case suggests that, irrespective of KSHV infection, some pleomorphic B cell lymphomas with membranous CD138 expression show a peculiar serosal spreading.
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10/71. Preperitoneal lipoleiomyoma of the abdominal wall in a postmenopausal woman.

    OBJECTIVE: Extrauterine leiomyomas are rare events. These tumors may be easily misdiagnosed as ovarian tumors at the clinical investigation. We present the first case of an otherwise healthy postmenopausal woman, hysterectomized 20 years ago, who developed a preperitoneal lipoleimoyoma in the 30-year-old scar of a Pfannenstiel incision. The patient received continuous hormone replacement therapy (HRT) for 5 years with 1.25 mg conjugated estrogen and 5 mg medrogeston per day. methods: In sections of the tumor, immunohistochemical reactions with antibodies against actin, desmin, vimentin, estrogen and progesterone receptors and factor viii related antigen was performed. RESULTS: Histologic findings revealed cellular fascicles of spindle-shaped smooth muscle cells in a whorled arrangement. Mitotic figures were absent. Central degenerative changes and focal edema were observed. Between muscle fascicles, a significant amount of fat cells (20% of tumor volume) was visible. Leiomyocytes showed immunohistochemicaly positive reactions with actin, desmin, vimentin, and steroid hormone receptors. Based on these findings, the tumor was diagnosed as lipoleiomyoma. CONCLUSIONS: Origin of the tumor of smooth muscle cells of vessels located in the abdominal wall and development under influence of oral steroids seems most probable. HRT appears to promote the development of extrauterine leiomyomas in postmenopausal women.
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