Desmoplastic malignant mesothelioma: Two cases and a literature review

Second Department of Pathology, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo 663-8501, Japan.
Medical Electron Microscopy 10/2003; 36(3):173-8. DOI: 10.1007/s00795-003-0217-0
Source: PubMed


We present two cases of desmoplastic malignant mesothelioma (DMM) with pathological, immunohistochemical, and ultrastructural features. Each patient showed rapid progress and died within 1 year from appearance of the initial symptoms. Macroscopically, both showed a thickened pleura replaced by a tumor that encased the lung. Microscopic results of each showed that the tumors consisted of a dense fibrous area, with mild nuclear irregularities and hyperchromatism. In case 1, the tumor had invaded the diaphragm, chest wall, and cardiac sac; the mass in case 2 invaded the lung and diaphragm, and distal metastases were seen in the thoracic vertebrae, meninges, and liver. Ultrastructural findings in case 1 showed a few short blunt microvilli on the cell surfaces. DMM is occasionally difficult to distinguish from fibrous pleurisy and solitary fibrous tumor. Immunohistochemical examinations of the present cases showed the expression of cytokeratin and vimentin, and focal positive stainings of antihuman mesothelial cell antibody (HBME-1) in both, whereas CD34 and bcl2 were negative. Solitary fibrous tumor was excluded. Therefore, pathological, ultrastructural, and immunohistochemical findings led us a diagnosis of DMM in each case. The Ki-67 labeling index (Ki-67 LI) of cases 1 and 2 was 25.5 and 15.5, respectively, both high, which suggested malignancy. Widespread immunohistological panels of malignant mesothelioma were not evaluated; Immunohistological markers commonly used for the diagnosis of malignant mesothelioma were not evaluated; however, the high ki-67 LI results and positive HBME-1 staining were helpful factors for the diagnoses of DMM.

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    • "Desmoplastic mesothelioma (DMM) is a rare and highly lethal variant of malignant pleural mesothelioma: This subtype, which accounts for 5–10% of malignant mesotheliomas, most commonly affects the pleura[1] and less commonly the peritoneum and the pericardium.[2–4] DMM was first described by Kannerstein and Churg in 1980[5] and since then the number of reports, although sporadic, has been constantly increasing.[2] Findings of the disease include a male-to-female ratio 2:1, a wide range of ages (12–77 years), and a documented asbestos exposure in 14% of cases.[3] "
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    ABSTRACT: Malignant mesothelioma is an uncommon malignant epithelial neoplasm originating from the serosal surface of body cavities. Because serosal surfaces are a common site of metastatic spread for a variety of malignant neoplasms originating from internal organs, separating malignant mesothelioma from metastatic tumors is of clinical importance. The diagnosis of malignant mesothelioma is complex and usually requires a multimodal approach that includes careful clinical history and physical examination, imaging studies, and tissue sampling for multimodal evaluation including routine histology, histochemistry, electron microscopy, and immunohistochemical tests. Of these, immunohistochemistry has emerged as the most valuable and readily available modality for the routine evaluation of these tumors. Unfortunately, no specific antibodies have yet been developed that can be accepted as exclusive for these tumors. The immunohistochemical diagnosis of malignant mesothelioma therefore depends on the use of a panel of stains that includes markers that are commonly expected to react with these tumors ("positive" markers) and markers that are not commonly expected to react with these tumors ("negative" markers). Additionally, the selection and utility of these various markers can vary considerably based on a constellation of circumstances, including patient sex, histologic appearance of the tumor (ie, epithelioid vs. sarcomatoid, etc), and various other clinical circumstances. Herein, we will review the currently available immunohistochemical markers used for the diagnosis of malignant mesothelioma and offer suggestions for the use of appropriate panels of stains based on specific morphologic types and clinical circumstances.
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