Clinical Medicine: Endocrinology and Diabetes
Synopsis: An open access, peer reviewed electronic journal that covers diabetes and endocrine disorders.
Indexing: 6 major databases. Pubmed indexing for NIH-funded research.
Processing time: Decision in 2 weeks for 90% of papers.
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Intestinal Obstruction and Pheochromocytoma in a Patient Suffering from von Recklinghausen’s Disease
B. Bouhanick1, M. Berry1, S. Hascouet1, J. Selves2, J.M. Coindre3, J. Fauvel4, J. Amar1 and B. Chamontin1
1Department of Internal Medicine and Hypertension, CHU Rangueil, Toulouse, France. 2Department of Histopathology, CHU Purpan, Toulouse, France. 3Department of pathology and INSERM U 916, Institut Bergonié, Bordeaux, France. 4Department of Biochemistry, CHU Rangueil, Toulouse, France.
Abstract
GISTs are rare neoplasms, which were recently identified to be a distinct pathologic entity. They can develop in patients with neurofibromatosis type 1 (NF1) or may be sporadic. NF1 is one of the most common inherited diseases and is a complex disease, with patients having an increased prevalence of benign and malignant tumors, including pheochromocytomas. The association of pheochromocytoma(s) and GISTs in NF1 is very rare. We report an additional case of this triple association in a normotensive 60-year- old female with NF1 admitted for intestinal obstruction: a pheochromocytoma of the left adrenal gland was discovered and surgical resection is performed. We provide an overview of the literature. The coexistence of NF1-related pheochromocytoma and GISTs is uncommon, but perhaps not fortuitous, and endocrinologists should be aware of this.
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