title: Adrenal haemangioblastoma presenting as phaeochromocytoma: a rare manifestation of extraneural hemangioblastoma.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Pathology, Armed Forces Medical College, Pune, India. debprabal@indiatimes.com
authors.forename: Prabal
authors.initials: P
authors.lastname: Deb
authors.forename: Seerat
authors.initials: S
authors.lastname: Pal
authors.forename: Vibha
authors.initials: V
authors.lastname: Dutta
authors.forename: Anand
authors.initials: A
authors.lastname: Srivastava
authors.forename: Akshay
authors.initials: A
authors.lastname: Bhargava
authors.forename: Krishan Kumar
authors.initials: KK
authors.lastname: Yadav
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D000315:Adrenalectomy; D000328:Adult; D002395:Catecholamines / Q000652:urine; D002528:Cerebellar Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000601:surgery; D003560:Cysts / Q000175:diagnosis; D003937:Diagnosis, Differential; D018325:Hemangioblastoma / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D006801:Humans; D006973:Hypertension; D007150:Immunohistochemistry; D007674:Kidney Diseases / Q000175:diagnosis; D008297:Male; D010673:Pheochromocytoma; D014642:Vanilmandelic Acid / Q000097:blood; D006623:von Hippel-Lindau Disease / Q000175:diagnosis*
chemical_list: D002395:Catecholamines; D014642:Vanilmandelic Acid