Ramsay J A, Asa S L, van Nostrand A W, Hassaram S T, de Harven E P
Am J Surg Pathol. 1987 Jun;11(6):480-6. doi: 10.1097/00000478-198706000-00009.
A case of bilateral adrenal pheochromocytomas with unusual morphologic features is reported in a 27-year-old man with a family history of von Hippel-Lindau disease. In both glands, the medulla was replaced by neoplasms with two distinct gross and microscopic appearances. There was typical pheochromocytoma in areas of dusky red tissue. The yellow nodules noted on gross examination were microscopically composed of large cells with vacuolated cytoplasm. Fat stains confirmed the presence of lipid in these areas. Immunohistochemistry revealed positivity for neuron-specific enolase and chromogranin in the cytoplasm of pheochromocytes, as well as in the perinuclear cytoplasm and processes of cells scattered in the yellow nodules. Ultrastructural examination of the yellow lesions showed characteristics of pheochromocytoma and an extensive accumulation of lipid. Although gross and light-microscopic examination of the yellow tissue suggested adrenal cortical nodules, immunohistochemistry and electon microscopy elucidated lipid degeneration within pheochromocytoma, a finding not previously described.
本文报道了一例27岁男性双侧肾上腺嗜铬细胞瘤，具有不寻常的形态学特征，该患者有冯·希佩尔-林道病家族史。双侧肾上腺髓质均被肿瘤取代，肿瘤具有两种不同的大体和显微镜下表现。暗红色组织区域可见典型的嗜铬细胞瘤。大体检查发现的黄色结节，显微镜下由胞质呈空泡状的大细胞组成。脂肪染色证实这些区域存在脂质。免疫组化显示嗜铬细胞的胞质、以及散在于黄色结节中的细胞的核周胞质和突起中，神经元特异性烯醇化酶和嗜铬粒蛋白呈阳性。黄色病变的超微结构检查显示出嗜铬细胞瘤的特征以及脂质的广泛积聚。尽管黄色组织的大体和光学显微镜检查提示为肾上腺皮质结节，但免疫组化和电子显微镜检查阐明了嗜铬细胞瘤内的脂质变性，这一发现此前未见报道。
