A rare case of Von Hippel Lindau disease

Abdul Ghani Rohana, M. K. Norazmi, Norlaila Mustafa

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

Pheochromocytoma is a rare catecholamine-secreting tumour typically arising within the adrenal medulla. It may occur sporadically or be associated as part of a tumour syndrome including Von Hippel Lindau (VHL), Multiple Endocrine Neoplasia (MEN) 2 and Neurofibromatosis Type 1. VHL is associated with multi-organ involvement of benign and malignant tumours characterized by the presence of retinal angiomas, hemangioblastomas of the cerebellum and spinal cord, renal cell carcinomas, pheochromocytomas and other cystic lesions in the kidneys, pancreas, and epididymis. It is a rare disorder with prevalence estimated at 2-3 per 100,000. This case report describes a 37 years old Chinese gentleman who presented to our institution for further management of bilateral pheochromocytoma and retinal angioblastoma with problems of duodenal ulcer and anaemia. There was no family history of similar problems. With these features the criteria for the diagnosis of von Hippel Lindau disease was established.

Original languageEnglish
Pages (from-to)254-257
Number of pages4
JournalMedical Journal of Malaysia
Volume61
Issue number2
Publication statusPublished - Jun 2006

Fingerprint

von Hippel-Lindau Disease
Pheochromocytoma
Hemangioblastoma
Multiple Endocrine Neoplasia Type 1
Neoplasms
Neurofibromatosis 1
Adrenal Medulla
Epididymis
Hemangioma
Duodenal Ulcer
Renal Cell Carcinoma
Cerebellum
Catecholamines
Anemia
Pancreas
Spinal Cord
Kidney

Keywords

  • Adrenalectomy
  • Cerebellar hemangioblastoma
  • Optic disc angioma
  • Pheochromocytoma
  • Renal cell carcinoma
  • Von Hippel Lindau Disease

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Rohana, A. G., Norazmi, M. K., & Mustafa, N. (2006). A rare case of Von Hippel Lindau disease. Medical Journal of Malaysia, 61(2), 254-257.

A rare case of Von Hippel Lindau disease. / Rohana, Abdul Ghani; Norazmi, M. K.; Mustafa, Norlaila.

In: Medical Journal of Malaysia, Vol. 61, No. 2, 06.2006, p. 254-257.

Research output: Contribution to journalArticle

Rohana, AG, Norazmi, MK & Mustafa, N 2006, 'A rare case of Von Hippel Lindau disease', Medical Journal of Malaysia, vol. 61, no. 2, pp. 254-257.
Rohana, Abdul Ghani ; Norazmi, M. K. ; Mustafa, Norlaila. / A rare case of Von Hippel Lindau disease. In: Medical Journal of Malaysia. 2006 ; Vol. 61, No. 2. pp. 254-257.
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