Benign retroperitoneal schwannoma presenting as colitis:A case report  

作　　者：Gary Fass Didier Hossey Michel Nyst Dirk Smets Esmail Najar Saligheh Ruth Duttmann Kathleen Claes Pierre Mendes da Costa 

机构地区：[1]Department of Digestive,Laparoscopic and Thoracic Surgery,Brugmann University Hospital,Brussels,Belgium [2]Department of Radiology,Brugmann University Hospital,Brussels,Belgium [3]Department of Pathology,Brugmann University Hospital,Brussels,Belgium [4]Laboratory of Molecular Genetics,Centre for Medical Genetics,Ghent University Hospital,Ghent,Belgium

出　　处：《World Journal of Gastroenterology》2007年第41期5521-5524,共4页世界胃肠病学杂志（英文版）

摘　　要：We report a case of a patient presenting with clinical,radiological and endoscopic features of colitis due to a compressive left para-aortic mass. Total open surgical excision was performed,which resulted in complete resolution of colitis. Histopathology and immunohistochemistry revealed benign retroperitoneal schwannoma. These neural sheath tumors rarely occur in the retroperitoneum. They are usually asymptomatic but as they enlarge they may compress adjacent structures,which leads to a wide spectrum of non-specific symptoms,including lumbar pain,headache,secondary hypertension,abdominal pain and renal colicky pain. CT and MR findings show characteristic features,but none are specific. Schwannoma can be isolated sporadic lesions,or associated with schwannomatosis or neurofibromatosis type Ⅱ (NF2). Although they vary in biological and clinical behavior,their presence is,in nearly every case,due to alterations or absence of the NF2 gene,which is involved in the growth regulation of Schwann cells. Both conditions were excluded by thorough mutation analysis. Diagnosis is based on histopathological examination and immunohistochemistry. Total excision is therapeutic and has a good prognosis. Schwannomatosis and NF2 should be excluded through clinical diagnostic criteria. Genetic testing of NF2 is probably not justified in the presence of a solitary retroperitoneal schwannoma.We report a case of a patient presenting with clinical, radiological and endoscopic features of colitis due to a compressive left para-aortic mass. Total open surgical excision was performed, which resulted in complete resolution of colitis. Histopathology and immunohistochemistry revealed benign retroperitoneal schwannoma. These neural sheath tumors rarely occur in the retroperitoneum. They are usually asymptomatic but as they enlarge they may compress adjacent structures, which leads to a wide spectrum of non-specific symptoms, including lumbar pain, headache, secondary hypertension, abdominal pain and renal colicky pain. CT and MR findings show characteristic features, but none are specific. Schwannoma can be isolated sporadic lesions, or associated with schwannomatosis or neurofibromatosis type Ⅱ （NF2）. Although they vary in biological and clinical behavior, their presence is, in nearly every case, due to alterations or absence of the NF2 gene, which is involved in the growth regulation of Schwann cells. Both conditions were excluded by thorough mutation analysis. Diagnosis is based on histopathological examination and immunohistochemistry. Total excision is therapeutic and has a good prognosis. Schwannomatosis and NF2 should be excluded through clinical diagnostic criteria. Genetic testing of NF2 is probably not justified in the presence of a solitary retroperitoneal schwannoma.

关 键 词：COLITIS NEUROFIBROMATOSIS RETROPERITONEUM SCHWANNOMA 

分 类 号：R574.6[医药卫生—消化系统]