Alsabeh R, Mazoujian G, Goates J, Medeiros L J, Weiss L M
Department of Pathology, City of Hope National Medical Center, Duarte, California 91010, USA.
Am J Clin Pathol. 1995 Oct;104(4):382-90. doi: 10.1093/ajcp/104.4.382.
The authors report five patients with adrenal cortical tumors in whom the preoperative diagnosis of pheochromocytoma was made. All patients had biochemical evidence of elevated catecholamine secretion in serum or urine. Clinically, two patients presented with symptoms suggestive of pheochromocytoma, and one patient had systemic hypertension that resolved following surgical excision of the tumor. Histologically, the findings were typical of adrenal cortical tumors: two adrenal cortical carcinomas and three adrenal cortical adenomas. Nevertheless, the immunohistochemical studies showed evidence of neuroendocrine differentiation in four tumors. Three tumors were positive for neuron-specific enolase and synaptophysin and a fourth tumor was positive for synaptophysin only. All neoplasms were negative for chromogranin. Electron microscopic studies in three tumors showed abundant endoplasmic reticulum and tubulovesicular cristae consistent with adrenal cortical cell origin. Neurosecretory granules, 150-300 mu in diameter, were found in one tumor. This current series of patients provides evidence that adrenal cortical neoplasms may be associated with clinical findings that simulate pheochromocytoma (so-called pseudo-pheochromocytoma). These clinical findings may be mediated by the presence of neuroendocrine features in these tumors.
作者报告了5例肾上腺皮质肿瘤患者，术前诊断为嗜铬细胞瘤。所有患者均有血清或尿液中儿茶酚胺分泌升高的生化证据。临床上，2例患者表现出提示嗜铬细胞瘤的症状，1例患者患有全身性高血压，肿瘤手术切除后血压恢复正常。组织学上，这些发现是肾上腺皮质肿瘤的典型表现：2例肾上腺皮质癌和3例肾上腺皮质腺瘤。然而，免疫组织化学研究显示4例肿瘤有神经内分泌分化的证据。3例肿瘤神经元特异性烯醇化酶和突触素呈阳性，第4例肿瘤仅突触素呈阳性。所有肿瘤嗜铬粒蛋白均为阴性。对3例肿瘤进行的电子显微镜研究显示，内质网丰富，有管状泡状嵴，符合肾上腺皮质细胞起源。在1例肿瘤中发现了直径为150 - 300微米的神经分泌颗粒。这组患者提供了证据，表明肾上腺皮质肿瘤可能与模拟嗜铬细胞瘤的临床表现（所谓的假性嗜铬细胞瘤）有关。这些临床表现可能由这些肿瘤中神经内分泌特征的存在介导。
