RESUMO
Perivascular epithelioid cell tumour (PEComa) of the liver is very uncommon and may be overlooked in the clinical and histological differential diagnosis of a liver tumour. We report the case of an incidentally discovered liver mass suspicious for hepatocellular carcinoma, which on biopsy was suggestive of a pseudocyst but after resection was found to be hepatic PEComa with some of the usual characteristics of this neoplasm as well as several less familiar features. We have also reviewed cases of hepatic PEComa from the literature in order to provide insight into recognising possible PEComa preoperatively and assessing its risk of malignancy after diagnosis.
Assuntos
Carcinoma Hepatocelular/diagnóstico , Neoplasias Hepáticas/diagnóstico , Fígado/patologia , Neoplasias de Células Epitelioides Perivasculares/diagnóstico , Biópsia com Agulha de Grande Calibre , Diagnóstico Diferencial , Humanos , Achados Incidentais , Fígado/diagnóstico por imagem , Neoplasias Hepáticas/patologia , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Neoplasias de Células Epitelioides Perivasculares/patologia , Tomografia Computadorizada por Raios X , UltrassonografiaRESUMO
We discovered two well-demarcated nodules incidentally in a thyroid removed because of a nodular goiter. Histologically, the nodules showed a pattern of paraganglioma or so-called paraganglioma-like adenoma of the thyroid (PLAT), with lobules of polygonal and oval cells in a vascular stroma, but the immunohistochemical markers typical of paraganglioma, including chromogranin, synaptophysin, Leu 7 and 5-100, and thyroglobulin, characteristic of PLAT, were negative in the tumor cells. C-cell markers calcitonin and somatostatin were also negative. Stain for neuron-specific enolase (NSE), however, showed a distinctive pattern of reactivity within cells at the periphery of the lobules, whereas all tumor cells stained positively for keratins. Stain for carcinoembryonic antigen showed a focal interstitial pattern that corresponded to small intercellular spaces filled by microvilli identified ultrastructurally. This pattern of immunohistochemical staining has not been previously described in paraganglioma or in PLAT, and may have implications about the origin and nature of these controversial entities.