Page mackup-Final.qxd Case Report Isolated Nerve Sheath Tumor Of Jejunum Presenting As Acute Abdomen Mohanty J1, Prasad S2 Abstract Nerve sheath tumors of gastrointestinal tract are rare and occurs more frequently as systemic manifes- tations of generalized neurofibromatosis type-1(NF-1).In neurofibromatosis-1 lesions may be scattered or solitary tumor. Occasionally ,such tumors may be the initial presenting feature of NF-1.Rarely ,cases of iso- lated nerve sheath tumor of small intestine presenting with bleeding and obstruction have been document- ed. We report a 19years male presenting with nerve sheath tumor of jejunum in acute abdomen. His inves- tigations show no features of NF-1. Keywords: nerve sheath tumor, neurofibromatosis, isolated, intestinal. Introduction Nerve sheath tumors of small intestine may manifest in various forms and accordingly given different descriptive terms including intestinal neurofibro- matosis, ganglioneuromatosis, neuronal intestinal dysplasia and diffuse plexiform neurofibromatosis. These lesions may be seen in 25% cases of neurofi- bromatosis-1 and multiple endocrine neoplasia type 2b.There occurrence without NF-1 and MEN-2b are very rare1. No consensus has been made yet whether these isolated lesions represent different phenotypic manifestations of the neurocutaneous or multiple endocrine neoplasia syndromes or whether they rep- resent separate and distinct entities. Case Report A 19 years old male presented to us with acute abdomen of 1 day history with symptoms of pain around umbilicus and 2 episodes of vom- iting. On examination he had tachycardia and tenderness over umbilical and hypogastrium regions with bp-90/60mm of Hg. He had rigidity and rebound tenderness more marked in lower abdomen. USG shows a oval well defined hypo echoic solid lesion seen in lower abdomen infraumblical region to the left of midline. CECT shows a isodense inhomogeneously enhancing soft tissue exophytic tumor of size 5.8*4.2*4.3 arising from small bowel likely to represent gas- trointestinal stromal tumor. There is no lym- phadenopathy with moderate amount of free fluid in the pelvis. Initially we proceed with laparo- scopic exploration .But due to dense adhesions we converted it into formal laparotomy with lower midline incisions. After entering into abdominal cavity a tumor arising from jejunum approximately 7 feet distal to D-J junction with dense adhesions with surrounding bowels was seen .[Figure-1] After clearing all adhesions carefully segmental resection of bowel having tumor carried out with 1. Dr. Jignyasu Mohanty, Associate Professor, Room No- 307, Mgm Medical Staff Quarters, Mgm Medical College, Sector 18 Kamothe, Navi Mumbai, Raigad District, Maharashtra, India, E-mail: Drjignyasu@rediffmail.com 2. Dr. Sujit Prasad, Consultant General Surgeon, Mgm Hospital, Sector 7, Vashi, Navi Mumbai, Maharashtra, India, E-mail:prasadsujitin@yahoo.com Corressponds to: Dr. Jignyasu Mohanty, Associate Professor, Room No- 307, Mgm Medical Staff Quarters, Mgm Medical College, Sector 18 Kamothe, Navi Mumbai, Raigad District, Maharashtra, India, E-mail: Drjignyasu@rediffmail.com Bangladesh Journal of Medical Science Vol. 12 No. 01 January’13 91 Figure I Intra operative photograph showing tumor arising from jejunum end to end anastomosis. [Figure-II] The postoper- ative orally allowded on 4th postoperative day and recovery was uneventful .Biopsy sent to 3 dif- ferent labs. Histopathology from all the 3 labs reported uniform epithelioid morphology with spindle component . These cells are very strong- ly positive for S100[Figure-3] and negative for C-kit, CD34, SMA, Calponin, HMB45, Melan A. There is no mitoses or necrosis. The patient is followed for 8months without any complication. Discussion Isolated nerve sheath tumors are benign neural lesions of intestinal tract which may be initial pre- senting feature of NF-1 and MEN tepe2b. Affected patient may present with altered bowel habit2, abdominal pain3, intestinal obstruction4 or with pal- pable abdominal mass. In setting of NF-1 associated clinical findings including dermal neurofibromas, café-au-lait spots and lisch nodules may be seen. In MEN type 2b thickened lips, marfanoid habitus and medullary carcinoma of thyroid may be seen. Isolated nerve sheath tumors commonly involve colon , terminal ileum , and appendix5. Surgical excision is the treatment of choice for these lesions These cases need to be followed up closely to exclude NF-1 and MEN type2b in future. References 1. Hochberg FH, Dasilva AB, Galdabini J, Richardson EP Jr. Gastrointestinal involvement in von Recklinghausen's neurofibromatosis. Neurology. 1974;24:1144-1151 http://dx.doi. org/10.1212/WNL.24.12.1144 PMid:4374669 2. Kim HR, Kim YJ. Neurofibromatosis of the colon and rectum combined with other manifes- tations of von Recklinghausen's disease: report of a case. Dis Colon Rectum. 3. Bononi M, De Cesare A, Stella MC, Fiori E, Galati G, Atella F, Angelini M, Cimitan A, Lemos A, Cangemi V. Isolated intestinal neu- rofibromatosis of colon. Single case report and review of the literature. Dig Liver Dis. 2000;32:737-742. http://dx. doi.org/10.1016/S1590-8658(00)80340-0 4. Urschel JD, Berendt RC, Anselmo JE. Surgical treatment of colonic ganglioneuromatosis in neurofibromatosis. Can J Surg. 1991;34:271- 276 PMid:1905194 5. Shekitka KM, Sobin LH. Ganglioneuromas of the gastrointestinal tract. Relation to Von Recklinghausen disease and other multiple tumor syndromes. Am J Surg Pathol.1994;18:250-257 http://dx.doi. org/10.1097/00000478-199403000-00004 PMid:7906923 Isolated Nerve Sheath Tumor Of Jejunum Presenting As Acute Abdomen 92 Figure II Immunohistochemistry showing strong s- 100 reaction