RESUMO
OBJECTIVE: To report the immunohistochemical and molecular evaluation of a patient with ectopic ACTH syndrome (EAS) from a MCAT which has single cells with features of both 96 medullary and cortical differentiation. Case Description and Methods. A 16-year-old woman presented with severe EAS and a large right MCAT composed of ACTH-secreting cells resembling pheochromocytoma and another lineage similar to adrenal carcinoma. Immunohistochemistry (IHC) showed positivity for medullary (ACTH, chromogranin A, synaptophysin, and PS-100) and epithelial components (inhibin, melan-A, and calretinin). Embryonic stem cell markers were evaluated using RT/PCR and immunofluorescence. After initial surgery, the tumor recurred shifting to rapidly progressive ACTH-independent liver metastasis. RESULTS: Histopathology and IHC revealed two distinct and intermingled cellular patterns, while some cells immunostained for both medullary and cortical markers. Demonstration of all stem cell biomarkers by RT/PCR and immunofluorescence was predominantly localized to the nucleus, whereas SOX2 immunoreactivity was evident in the cytoplasm as well. CONCLUSION: The expression of cancer stem cell biomarkers points towards the involvement of primitive embryonic cells as the origin of this neoplasm and maybe to the clinically aggressive and biochemically changing behavior.
RESUMO
Primary liver sarcomas make up 2% of all malignant neoplasms of the liver; of these, angiosarcoma is the most common type. Primary liver tumours rarely cause fulminant hepatic failure (FHF), which is most frequently caused by non-neoplasmic pathologies. In the case of neoplasms, the most frequent are lymphoma and metastatic carcinomas. We describe the case of a 76-year-old man who suffered from FHF as a result of a liver angiosarcoma and we present a review of the medical literature in which we found only two cases of liver angiosarcomas linked to FHF.