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Open Access Highly Accessed Case report

Concomitant composite adrenal pheochromocytoma, multiple gastric stromal tumours and pseudohermaphrodism in a patient with von Recklinghausen's disease

Dean Lisewski1, Simon Ryan1*, Ee Mun Lim2, Felicity Frost2 and Hieu Nguyen1

Author Affiliations

1 Department of Surgery, Sir Charles Gairdner Hospital, Nedlands, Western Australia

2 The Western Australian Centre for Pathology & Medical Research (PathCentre), Nedlands, Western Australia

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International Seminars in Surgical Oncology 2006, 3:11  doi:10.1186/1477-7800-3-11

Published: 26 April 2006

Abstract

Although pheochromocytoma occurs in 1% of patients with von Recklinghausen's disease, composite tumors in this syndrome are much rarer, with isolated case reports in the literature. Most gastrointestinal stromal tumors (GISTs) are solitary and sporadic. Multiple GISTs however, are associated with clinical syndromes particularly von Recklinghausen's disease. We believe this is the first report of composite adrenal pheochromocytoma and multiple GISTs occurring in an 82 year old woman with neurofibromatosis type 1 (NF1), manifested by clitoral and subcutaneous neurofibromas, epilepsy and Lisch nodules. The extreme clitoromegaly raised concerns of pseudohermaphrodism at presentation.