A Rare Association of Pheochromocytoma and Gastrointestinal Stromal Tumor in a Patient with Recklinghausen’s Disease: A Case Report

Neurofibromatosis type 1 (von Recklinghausen's disease-NF1) is a genetic disease characterized by neoplastic and non neoplastic disorders involving tissues of neuroectodermal and mesenchymal origin. It comprises 90% of neurofibromatosis cases. It is characterized by abnormal skin pigmentation (cafe au lait spots and axillary freckling), skeletal dysplasias, cutaneous and plexiform neurofibromas, and Lisch nodules. 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 about 16 cases documented till 2015 in the English literature. Here, we are reporting a rare combination of pheochromocytoma and gastrointestinal stromal tumour in a patient with neurofibromatosis type 1.