RESUMO
Clear-cell sarcoma (CCS) is a soft-tissue neoplasm that morphologically resembles cutaneous malignant melanoma but has a distinct molecular profile. Gastrointestinal and intra-abdominal CCSs are very rare. Here, the authors present 3 cases of intra-abdominal CCS and review the literature. Of these cases, 2 involved the small bowel, and 1 involved the peritoneum. Cases 1 and 3 had the characteristic CCS morphology, but case 2 was morphologically unusual and therefore difficult to diagnose. It had relatively small cells with less prominence of clear cells; many pseudoglandular structures were also present. It also showed aberrant expression of epithelial membrane antigen (EMA). The other 2 cases also involved some diagnostic uncertainty and were therefore referred to specialized centers. The authors wish to emphasize the importance of molecular studies in making a conclusive diagnosis of intra-abdominal CCS.
Assuntos
Neoplasias Abdominais/diagnóstico , Sarcoma de Células Claras/secundário , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias Abdominais/genética , Neoplasias Abdominais/metabolismo , Adulto , Idoso , Biomarcadores Tumorais/metabolismo , DNA de Neoplasias/análise , Feminino , Fusão Gênica , Rearranjo Gênico , Humanos , Hibridização in Situ Fluorescente , Intestino Delgado/patologia , Linfonodos/patologia , Metástase Linfática , Masculino , Pessoa de Meia-Idade , Mucina-1/metabolismo , Patologia Molecular/métodos , Peritônio/patologia , Sarcoma de Células Claras/genética , Sarcoma de Células Claras/metabolismo , Neoplasias de Tecidos Moles/genética , Neoplasias de Tecidos Moles/metabolismo , Translocação GenéticaRESUMO
A case of multiple myeloma is reported in which the bone marrow contained sheets of histiocytes with light microscopic features mimicking Gaucher cells. The patient had no clinical evidence of inherited Gaucher's disease. These pseudo-Gaucher cells obscured neoplastic plasma cells causing diagnostic difficulty.
