Koo Joo-Yeon, Lee Kyung-Hwa, Choi Joon Hyuk, Chung Ho Seok, Choi Chan
Department of Pathology, Chonnam National University Research Institute of Medical Science, Bio-Medical Sciences Graduate Program (BMSGP), Chonnam National University Hwasun Hospital and Medical School, Hwasun, Korea.
Department of Pathology, Yeungnam University Hospital, Daegu, Korea.
J Pathol Transl Med. 2022 May;56(3):161-166. doi: 10.4132/jptm.2021.12.28. Epub 2022 Feb 28.
Hemangioblastoma (HB) is a rare benign tumor that most commonly occurs in the cerebellum. HB is composed of neoplastic stromal cells and abundant small vessels. However, the exact origin of stromal cells is controversial. Extraneural HBs have been reported in a small series, and peripheral HBs arising in the adrenal gland are extremely rare. Herein, we report a case of sporadic adrenal HB in a 54-year-old woman. The tumor was a well-circumscribed, yellow mass measuring 4.2 cm in diameter. Histologically, the tumor was composed of small blood vessels and vacuolated stromal cells with clear cytoplasm. On immunohistochemical stain, the stromal cells were positive for S-100 protein, neuron-specific enolase, and synaptophysin. The tumor did not reveal mutation of VHL alleles. We herein present a case of HB of the adrenal gland and review of the literature.
血管母细胞瘤(HB)是一种罕见的良性肿瘤,最常发生于小脑。HB由肿瘤性基质细胞和丰富的小血管组成。然而,基质细胞的确切起源存在争议。小系列报道中曾提及神经外HB,而起源于肾上腺的外周HB极为罕见。在此,我们报告一例54岁女性散发性肾上腺HB病例。肿瘤为边界清晰的黄色肿块,直径4.2厘米。组织学上,肿瘤由小血管和具有透明细胞质的空泡状基质细胞组成。免疫组化染色显示,基质细胞S-100蛋白、神经元特异性烯醇化酶和突触素呈阳性。肿瘤未显示VHL等位基因突变。我们在此呈现一例肾上腺HB病例并复习相关文献。