Hoang Mai P, Amirkhan Robin H
Department of Pathology, Unviersity of Texas Southwestern Medical Center and Veterans Affairs north Texas health Care System, Dallas, TX 75390-9073, USA.
Am J Surg Pathol. 2003 Aug;27(8):1152-6. doi: 10.1097/00000478-200308000-00014.
Inhibin alpha subunit (inhibin A) expression in hemangioblastomas has not been previously reported in the literature. We analyzed the expression of inhibin A in 25 hemangioblastomas from 22 patients. Eleven cases were from 8 patients with von Hippel-Lindau disease, and these tumors were multicentric and/or recurrent. The remaining 14 cases from 14 patients were sporadic. The male-to-female ratio was 8:3, and the age at presentation ranged from 19 to 78 years (mean 35 years; median 45 years). Eighteen tumors were located in the cerebellum/posterior fossa, 1 in the medulla, 1 in the occipital lobe, and 5 in the spinal cord. Four metastatic renal cell carcinomas in brain, 10 renal cell carcinomas from 8 patients with von Hippel-Lindau disease, and 5 sporadic clear cell renal cell carcinomas were also included. Two patients with von Hippel-Lindau disease had both renal cell carcinoma and hemangioblastoma. The stromal cells of all 25 cases of hemangioblastoma expressed inhibin A. Strong, moderate, and weak cytoplasmic immunoreactivity was noted in 17, 5, and 3 cases, respectively. In contrast, none of the 19 renal cell carcinomas, primary as well as metastatic, expressed inhibin A. There was no difference in the inhibin A staining pattern between the sporadic hemangioblastoma and those associated with VHL. These findings demonstrate inhibin A to be a useful marker in distinguishing hemangioblastoma from metastatic clear cell renal cell carcinoma. While the diagnostic importance is evident, the pathophysiology of inhibin A expression by the stromal cells of hemangioblastoma remains unknown and further investigation is required.
文献中此前尚未报道过成血管细胞瘤中抑制素α亚基（抑制素A）的表达情况。我们分析了来自22例患者的25个成血管细胞瘤中抑制素A的表达。11例来自8例von Hippel-Lindau病患者，这些肿瘤为多中心性和/或复发性。其余14例来自14例患者，为散发性。男女比例为8:3，就诊时年龄范围为19至78岁（平均35岁；中位数45岁）。18个肿瘤位于小脑/后颅窝，1个位于延髓，1个位于枕叶，5个位于脊髓。还纳入了4例脑转移肾细胞癌、8例von Hippel-Lindau病患者的10例肾细胞癌以及5例散发性透明细胞肾细胞癌。2例von Hippel-Lindau病患者同时患有肾细胞癌和成血管细胞瘤。所有25例成血管细胞瘤的基质细胞均表达抑制素A。分别在17例、5例和3例中观察到强、中、弱细胞质免疫反应性。相比之下，19例原发性和转移性肾细胞癌均未表达抑制素A。散发性成血管细胞瘤与VHL相关的成血管细胞瘤在抑制素A染色模式上没有差异。这些发现表明抑制素A是区分成血管细胞瘤与转移性透明细胞肾细胞癌的有用标志物。虽然其诊断重要性显而易见，但成血管细胞瘤基质细胞表达抑制素A的病理生理学仍不清楚，需要进一步研究。
