Concomitant compostite adrenal phoechromocytoma, multipte gastric stromal tumours and pseudohermaphrodism in a patient with von Recklinghausen's disease.

International Seminars in Surgical Oncology : ISSO
Dean LisewskiHieu Nguyen

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.

References

Apr 1, 1981·Ophthalmology·R A Lewis, V M Riccardi
Dec 31, 1981·The New England Journal of Medicine·V M Riccardi
May 1, 1980·Intensive Care Medicine·D PerrotinF Lamisse
Apr 1, 1995·Southern Medical Journal·P J Moore, P J Biggs
Jan 30, 1995·American Journal of Medical Genetics·R SutphenB G Kousseff
Jan 1, 1994·Journal of Pediatric Surgery·D A RogersA S Pappo
Aug 1, 1993·The American Journal of Surgical Pathology·R Chetty, J D Duhig
Jan 15, 1999·The American Journal of Surgical Pathology·T S EmoryT J O'Leary
May 8, 1999·Scandinavian Journal of Gastroenterology·T ArtazaJ J Sánchez

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Citations

Feb 17, 2009·World Journal of Surgical Oncology·Edward AlabrabaPhilippe Taniere
Jan 16, 2009·Orvosi hetilap·Judit Bajor
Feb 19, 2009·The American Journal of Gastroenterology·Josué Barahona-GarridoJesús K Yamamoto-Furusho
Jun 18, 2010·Journal of Huazhong University of Science and Technology. Medical Sciences = Hua Zhong Ke Ji Da Xue Xue Bao. Yi Xue Ying De Wen Ban = Huazhong Keji Daxue Xuebao. Yixue Yingdewen Ban·Yunan ZhouBo Hu
Oct 17, 2009·Endocrine Practice : Official Journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists·Amna N KhanRichard Dale Childress
Mar 20, 2014·Endocrine Practice : Official Journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists·Hassan ShawaMouhammed Amir Habra
Jul 3, 2020·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Pia BernardoClaudia Santoro
Mar 3, 2021·Langenbeck's Archives of Surgery·K DhanasekarS P Balasubramanian

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Methods Mentioned

BETA
biopsy
biopsies

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