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CASE REPORT
Year : 2011  |  Volume : 15  |  Issue : 8  |  Page : 402-405

Role of cortical sparing adrenalectomy and novel variant of mutation in patient with von Hippel-Lindau disease


1 Department of General Surgery, Christian Medical College, Vellore, Tamil Nadu, India
2 Department of Endocrine Surgery, Christian Medical College, Vellore, Tamil Nadu, India
3 Department of Pathology, Christian Medical College, Vellore, Tamil Nadu, India
4 Department of Endocrinology, Christian Medical College, Vellore, Tamil Nadu, India

Correspondence Address:
M J Paul
Department of Endocrine Surgery, Christian Medical College, Vellore - 632 004, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2230-8210.86987

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Neurofibromatosis type 1 is the most common phakomatoses and is inherited in autosomal dominant fashion with complete penetrance. Secondary hypertension is common in these patients due to various causes including adrenal tumors. Pheochromocytoma is a rare catecholamine producing tumor seen in 0.5% to 5% of patients with neurofibromatosis. The combination of pheochromocytoma with neurofibromatosis is rarely reported in the literature. We recently encountered an elderly lady with this combination who successfully underwent adrenalectomy. We report the case for the uncommon occurrence and to highlight the relevant literature review about pheochromocytoma in neurofibromatosis.


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