Multiple gastrointestinal stromal tumors and pheochromocytoma in a patient with von Recklinghausen's disease

详细信息    查看全文

• 作者：Beyza Ozcinar^a ; ^{drbeyza@hotmail.com} ; Nihat Aksakal^a ; Orhan Agcaoglu^a ; Mustafa Tukenmez^a ; Ibrahim A. Ozemir^a ; Umut Barbaros^a ; Nese Colak^b ; Yesim Erbil^a
• 关键词：Neurofibromatozis type 1 ; Von Recklinghausen's disease ; Gastrointestinal stromal tumor ; Pheochromocytoma
• 刊名：International Journal of Surgery Case Reports
• 出版年：2013
• 出版时间：2013
• 年：2013
• 卷：4
• 期：2
• 页码：216-218
• 全文大小：565 K

文摘

INTRODUCTION

Neurofibromatosis type 1 is a genetic disease characterized by neoplastic and non neoplastic disorders involving tissues of neuroectodermal and mesenchymal origin. Herein, we present a case with von Recklinghausen's disease, right adrenal heochromocytoma and multiple gastrointestinal stromal tumors.

PRESENTATION OF CASE

A forty-eight year old male patient was admitted to our Emergency Department with melena. His physical examination revealed multiple neurofibromas all over the skin, kyphosis, multiple cafe au lait spots and Lisch nodules on the eye and, melena on digital rectal examination. Abdominal computerized tomography scan showed a mass on right adrenal gland and multiple soft tissue mass lesions between distal part of pancreas and small bowel. Adrenal mass was determined as a pheochromocytoma and small bowel lesions were verified as stromal tumors.

DISCUSSION

In patients with NF1, pheochromocytomas and GISTs are well known neoplasms seen with increased incidence than the general population.

CONCLUSION

In patients with NF1, any symptoms with other systems should be managed carefully for underlying malignity.