Adrenal Haemangioblastoma Presenting as Phaeochromocytoma: A Rare Manifestation of Extraneural Hemangioblastoma

作者：Deb Prabal^*; Pal Seerat; Dutta Vibha; Srivastava Anand; Bhargava Akshay; Yadav Krishan Kumar

来源：Endocrine Pathology, 2012, 23(3): 187-190.

DOI：10.1007/s12022-012-9207-x

• Adrenal
• Aquaporin-1
• Extraneural hemangioblastoma
• Phaeochromocytoma

Adrenal haemangioblastoma presenting clinically as pheochromocytoma is a rare manifestation of extraneural haemangioblastoma. We present an unusual case of von Hippel-Lindau (VHL) disease that had adrenal and cerebellar haemangioblastoma with multiple renal cysts, and a review of the literature.
Unlike the usual manifestations of secondary polycythemia or increased intracranial pressure and hydrocephalus due to cerebellar lesion, this 36-year-old male presented with hypertension. Investigations revealed right suprarenal mass with raised urinary catecholamines and serum vanillylmandelic acid (VMA) levels, apparently confirming the clinical diagnosis of phaeochromocytoma.
Histopathology of the biopsy specimen showed features of haemangioblastoma, which was confirmed by immunohistochemistry using antibodies to neuron specific enolase and aquaporin-1. Based on this, the patient was screened for possible features of VHL, which revealed cerebellar haemangioblastoma and multiple renal cysts with angiomatous lesion. Postoperative follow-up showed normal levels of catecholamines without any symptoms of phaeochromocytoma.
Adrenal haemangioblastoma is a rare entity with only four cases reported in the literature. Surgical removal is the treatment of choice. However, screening for other possible features of VHL, even in the absence of clinical features, is essential to exclude other potential lesions.

• 出版日期2012-9
• 单位中国人民武装警察部队后勤学院