title: A rare combination of pheochromocytoma and gastrointestinal stromal tumour in a patient with neurofibromatosis 1 syndrome-a case report.
abstract: Neurofibromatosis 1 is a rare inherited autosomal dominant syndrome. It comprises 90 % of neurofibromatosis cases. These patients may develop various types of tumours in early age, especially multiple neurofibromas with a high risk of developing malignant peripheral nerve sheath tumours. Other tumours can also develop like pheochromocytoma, optic nerve and brain stem gliomas, carcinoids and rarely gastrointestinal stromal tumours. A combination of pheochromocytoma and gastrointestinal stromal tumour is very rare. Only a few cases have been reported. Here, we are reporting a case of NF1 syndrome with a combination of pheochromocytoma and gastrointestinal stromal tumour with additional findings of multiple clear cell nodules and brown fat in the periadrenal connective tissue.
authors.affiliation: Government Medical College, Thrissur, Kerala, India. psjayalakshmy@gmail.com.|, Parijatham, Royal Avenue, Near Attore Road Bus Stop, Kuttur. P.O., Thrissur, PIN-680013, Kerala, India. psjayalakshmy@gmail.com.
authors.forename: P S
authors.initials: PS
authors.lastname: Jayalakshmy
authors.affiliation: Government Medical College, Thrissur, Kerala, India. anishmohan44@gmail.com.
authors.forename: A Anish
authors.initials: AA
authors.lastname: Mohan
authors.affiliation: Government Medical College, Thrissur, Kerala, India. drrajeshkkumar@gmail.com.
authors.forename: Rajesk K
authors.initials: RK
authors.lastname: Kumar
authors.affiliation: Government Medical College, Thrissur, Kerala, India. junainabeevip@gmail.com.
authors.forename: P Junaina
authors.initials: PJ
authors.lastname: Beevi
keywords: Brown fat; Clear cell nodules; Gastrointestinal stromal tumour (GIST); Inherited syndromes; Neurofibromatosis 1 (NF1); Pheochromocytoma