Vankadari Kousik, Boddula Raman, Hegde Aditya Gajanan, Chinte Chimutai
Department of Nuclear Medicine, Yashoda Hospital, Secunderabad, Telangana, India.
Department of Endocrinology, Yashoda Hospital, Secunderabad, Telangana, India.
World J Nucl Med. 2022 Apr 30;21(1):73-75. doi: 10.1055/s-0042-1746177. eCollection 2022 Mar.
Pheochromocytoma is a rare catecholamine secreting neuroendocrine tumor arising from chromaffin cells of adrenal medulla with approximate prevalence of 0.1 to 0.6% in patients suffering from hypertension. Hypertensive control followed by surgical resection remains the primary treatment of choice. Although it is considered a slow growing benign tumor, it rarely leads to recurrence of tumor in the lymph nodes, liver, and lungs. Association of benign pheochromocytoma with familial or de novo Von Hippel-Lindau (VHL) mutations is well reported in literature. Here, we report a case of metastatic pheochromocytoma arising from commonly seen benign VHL mutation.
嗜铬细胞瘤是一种罕见的分泌儿茶酚胺的神经内分泌肿瘤，起源于肾上腺髓质的嗜铬细胞，在高血压患者中的患病率约为0.1%至0.6%。控制高血压后进行手术切除仍然是主要的治疗选择。虽然它被认为是一种生长缓慢的良性肿瘤，但很少导致肿瘤在淋巴结、肝脏和肺部复发。文献中已充分报道了良性嗜铬细胞瘤与家族性或新发的冯·希佩尔-林道（VHL）突变的关联。在此，我们报告一例由常见的良性VHL突变引起的转移性嗜铬细胞瘤病例。
