RESUMEN
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.
Asunto(s)
Neoplasias Abdominales/diagnóstico , Sarcoma de Células Claras/secundario , Neoplasias de los Tejidos Blandos/diagnóstico , Neoplasias Abdominales/genética , Neoplasias Abdominales/metabolismo , Adulto , Anciano , Biomarcadores de Tumor/metabolismo , ADN de Neoplasias/análisis , Femenino , Fusión Génica , Reordenamiento Génico , Humanos , Hibridación Fluorescente in Situ , Intestino Delgado/patología , Ganglios Linfáticos/patología , Metástasis Linfática , Masculino , Persona de Mediana Edad , Mucina-1/metabolismo , Patología Molecular/métodos , Peritoneo/patología , Sarcoma de Células Claras/genética , Sarcoma de Células Claras/metabolismo , Neoplasias de los Tejidos Blandos/genética , Neoplasias de los Tejidos Blandos/metabolismo , Translocación GenéticaRESUMEN
Malignancy in struma ovarii is a rare form of ovarian germ cell tumour. Because of its rarity, the diagnosis and management of the tumour have not been clearly defined. We present a case of 67- year-old female with papillary carcinoma arising in struma ovarii and review the literature on malignancy in struma ovarii cases, focusing on management of these cases.