Cases reported "Mesothelioma"

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1/22. Mesotheliomas with deciduoid morphology: a morphologic spectrum and a variant not confined to young females.

    Deciduoid mesotheliomas are rare with only four previously reported cases, all affecting the peritoneum of young females. We describe another six cases (three men and three women; age range 52-65 yrs, median 55 yrs; five peritoneal and one pleural). Three patients had an occupational history of asbestos exposure. The deciduoid appearance predominated in four cases, whereas in two it represented a minor component within conventional tubulopapillary epithelioid mesothelioma. All tumors were strongly cytokeratin-positive (including CK5/6) and all showed at least focal staining for thrombomodulin, HBME-1, and calretinin. All were negative for epithelial mucin (D/PAS), CEA, BerEP4, LeuM1 (CD15), CD21, CD35, and S100 protein. Five of six cases (83%) were vimentin-positive and two (33%) were focally positive for alpha-smooth muscle actin. A differential diagnosis of gastrointestinal autonomic nerve tumor (GANT) had been initially considered from the morphology of one case, and we found positivity for some of the "neuronal" markers described in GANTs. This prompted us to apply such a panel to the other five tumors, accepting that the cytokeratin positivity encountered in all of our cases would exclude GANT. All cases of deciduoid mesothelioma (100%) were positive for PGP 9.5 and NSE and four of six (66%) were positive for NKI/C3. Weak focal staining (<5% cells) for synaptophysin was seen in two of six tumors. All cases were chromogranin-negative. All cases examined by electron microscopy showed desmosomes and smooth microvilli without rootlets but no neuroendocrine granules. In conclusion, a deciduoid morphology appears to be part of the histopathologic spectrum encountered in epithelioid mesothelioma. This variant is not confined to female patients and occurs over a wider age range than previously recognized. The overlapping immunophenotype with GANTs illustrates that caution should be exercised when interpreting positivity for "neuronal" markers in this context. An immunohistochemical panel that includes cytokeratins should always be used.
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2/22. '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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3/22. Deciduoid mesothelioma: a rare, distinct entity with unusual features.

    An unusual case of deciduoid mesothelioma occurring in the anterior abdominal wall of a 30-year-old woman is reported. The patient had a palpable mass that was resected. The mass appeared largely cystic with solid areas. Histologically, the tumor cells appeared epitheloid with eosinophilic cytoplasm and prominent nucleoli. The tumor was positive for keratins and vimentin and negative for CEA and Ber-EP4. Electron microscopy showed features of mesothelial cells characterized by well-formed desmosomes and long, slender microvilli. In contrast to previously reported cases of deciduoid mesothelioma, this tumor developed in the abdominal wall and appears to have a benign course.
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4/22. An intriguing case: malignant mesothelioma presenting as inguinal lymph node metastases.

    A 56-year-old woman presented with a right inguinal mass. biopsy revealed multiple lymph nodes involved with papillary and gland-like structures extending into the surrounding fibroadipose tissue. There was no history of carcinoma or other malignancy. Ultrastructural findings included long microvilli, desmosomes, and tonofilament bundles, indicating metastatic malignant mesothelioma. Malignant mesothelioma only rarely presents as a lymph node metastasis, and electron microscopy is very useful in establishing this diagnosis.
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5/22. Malignant peritoneal mesothelioma in childhood with long-term survival.

    A diffuse, well-differentiated, malignant peritoneal mesothelioma (MPM) developed in a nine-year-old girl. She received limited chemotherapy and radiation therapy and is alive and well without clinical evidence of disease 109 months after diagnosis. The neoplastic cells stained immunohistochemically for cytokeratin and epithelial membrane antigen but were unreactive with B72.3, anti-carcinoembryonic antigen, and anti-Leu-M1. Ultrastructurally, the tumor cells had abundant desmosomes, numerous tonofilament bundles, and variable-length microvilli. These findings confirm the mesothelial nature of the cells. Features consistent with malignancy included dna aneuploidy by flow cytometric analysis and diffuse peritoneal involvement. The three previously described survivors with MPM were also premenarchal girls. Some MPMs in premenarchal girls have an indolent biologic behavior similar to that of low-grade peritoneal serous neoplasia or well-differentiated papillary mesothelioma in adult women.
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6/22. Diffuse malignant peritoneal mesothelioma in a young woman with a high serum level of CA125.

    An autopsy case of diffuse malignant peritoneal mesothelioma in a young woman who showed a high serum level of CA125 is reported. autopsy revealed extensive tumor involvement of the visceral and parietal peritoneum. The liver, spleen and other abdominal viscera were encased by tumor nodules. Histologically, the polygonal tumor cells were arranged mostly in a sheet-like fashion with a few tubular or papillary forms. No PAS reaction-positive mucin was recognized, but there was a strongly positive colloidal iron reaction. The colloidal iron positivity was effaced after combined treatment with hyaluronidase and sialidase. Immunohistochemically the tumor cells showed strongly positive reactions for CA125, epithelial membrane antigen (EMA) and cytokeratin, weak positivity for carcinoembryonic antigen (CEA) and focal positivity for vimentin. Ultrastructurally, the most characteristic feature was the expression of numerous long microvilli projecting from the tumor cell surfaces and abundant long desmosomes between the tumor cells. We consider that pretreatment using a combination of hyaluronidase and sialidase might be useful for the diagnosis of malignant mesothelioma. CA125 staining should be performed routinely in cases where this tumor is suspected.
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7/22. The fine structure of so-called minute pulmonary chemodectomas.

    Electron microscopy was performed on several minute tumors of the type called chemodectomas, all from the lung of a single patient. The cells had a whorling pattern with extensive interdigitating cytoplasmic processes joined by desmosomes. Except for tangles of cytoplasmic fibrils, the tumor cells had few distinctive organelles. They had no endocrine-like granules and were not associated with nerves or basement membranes. The tumors had little resemblance to paragangliomas, but displayed a puzzling similarity to meningiomas. Our observations permit no definite conclusions as to the histogenesis of these lung tumors. Viewed in the light of recent physiologic studies, they cast doubt on the presence of special chemoreceptive paraganglia in the lung.
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8/22. Malignant mesothelioma of peritoneum.

    A 57-year-old man with malaise, ascites, and abdominal pain was found to have a peritoneum studied with numerous, small nodular tumor masses. light microscopy revealed an anaplastic malignant tumor of uncertain differentiation. Mucin stains were negative. Electron microscopy revealed pleomorphic tumor cells with diffusely distributed cytoplasmic tonofilaments and well-developed true desmosomes. No long, thin, branching microvilli were present, yet tumor cells were strongly positive for both callus keratin (polyclonal) and monoclonal cytokeratin (AE1/3) in a diffuse cytoplasmic distribution (a pattern corresponding to the diffuse cytoplasmic tonofilaments). Tumor cells were negative for Leu-M1 and carcinoembryonic antigen. The findings were most consistent with malignant mesothelioma, and additional questioning, after tissue diagnosis, revealed a work history of asbestos exposure.
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9/22. Cystic adenomatoid tumor of the uterus.

    We describe a case of cystic adenomatoid tumor of the uterus in a 38-year-old woman. The tumor was a subserosal cystic mass with a maximum diameter of 8 cm and located at the posterior wall of the right cornual region. Histologically, the tumor was composed of multiple cavities lined by flattened cells, lying among thin septa of connective tissue. The neoplasm showed small solid areas with a more typical histologic pattern of adenomatoid tumor. Immunohistochemical techniques showed the cells to be positive for keratins. They showed desmosomes, abundant intracytoplasmic filaments, and microvilli on ultrastructural study. The histologic, immunohistochemical, and ultrastructural characteristics of the present case closely resemble those of benign cystic mesothelioma and strongly support the mesothelial origin of the adenomatoid tumor of the uterus.
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10/22. An autopsy case of peritoneal malignant mesothelioma in a radiation technologist.

    A case of peritoneal malignant mesothelioma in a radiation technologist, who had worked in this field for 34 years, is reported. Histopathologically, a biopsy specimen from the retroperitoneal tumor revealed a biphasic type of malignant mesothelioma. Electron microscopy disclosed that the tumor cells contained prominent microvilli, basal laminae adjacent to the stroma, junctional complexes, desmosomes, tonofilaments, clusters of glycogen granules, well developed rough endoplasmic reticulum (RER), confronting cisternae showing direct continuity with the RER and membrane-bound granules suggestive of secretory activity. No increased amount of asbestos was detected in autopsied lung material or the peritoneal mesothelioma. The estimated cumulative dose of occupational irradiation was calculated to be about 40 to 50 rad at most. Irradiation was discussed in relation to the etiology of the peritoneal mesothelioma.
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