Ileoileal intussusception induced by a gastrointestinal stromal tumor

Abstract

Background: Gastrointestinal stromal tumors are mesenchymal tumors of the gastrointestinal tract of varying malignant potential that are believed to originate from neoplastic transformation of the interstitial cells of Cajal. They may occur anywhere along the gastrointestinal tract, but most commonly arise in the stomach or small intestine. They usually grow exophytically invading adjacent organs or perforating into the peritoneal cavity. They may also cause bleeding or obstructive symptoms. Intussusception and obstruction is a very uncommon presentation of these lesions because of their tendency to grow in an exraluminal fashion.

Small Bowel Obstruction in a Neurofibromatosis Patient—A Rare Presentation of Gastro-Intestinal Stromal Tumors (GISTs): Case Report and Literature Review

“…It is important to distinguish this category of NF1 patients from familial GIST syndrome (numerous at times diffuse GISTs with undefined skin hyper pigmentation, urticaria pigmentosa, nevi including melanoma or achalasia mostly associated with germline KIT mutation) or sporadic GIST patients with multiple metastatic nodules carrying high mitotic index [1,8]. These tumours are usually asymptomatic (95 %) and incidentally found (33 %) at early stages [2][3][4]7] and later may present with abdominal mass, pain, nausea, weight loss, GI haemorrhage and anaemia [1,2,[4][5][6][7]9]. Differential diagnosis of NF1 patients with acute abdomen may range from non-obstructive presentations (massive intra-luminal or intra-peritoneal haemorrhage, intra-peritoneal or intra-hepatic abscess, tumour rupture and perforation) [9,10] to very uncommon obstructive presentation (6 %) creating surgical emergency [1,2,4,5,7,9].…”

“…These tumours are usually asymptomatic (95 %) and incidentally found (33 %) at early stages [2][3][4]7] and later may present with abdominal mass, pain, nausea, weight loss, GI haemorrhage and anaemia [1,2,[4][5][6][7]9]. Differential diagnosis of NF1 patients with acute abdomen may range from non-obstructive presentations (massive intra-luminal or intra-peritoneal haemorrhage, intra-peritoneal or intra-hepatic abscess, tumour rupture and perforation) [9,10] to very uncommon obstructive presentation (6 %) creating surgical emergency [1,2,4,5,7,9]. Obstruction of small bowel by GISTs may occur due to intussusception, volvulus, luminal obstruction or rarely by torsion and adhesion [5,7].…”

“…Differential diagnosis of NF1 patients with acute abdomen may range from non-obstructive presentations (massive intra-luminal or intra-peritoneal haemorrhage, intra-peritoneal or intra-hepatic abscess, tumour rupture and perforation) [9,10] to very uncommon obstructive presentation (6 %) creating surgical emergency [1,2,4,5,7,9]. Obstruction of small bowel by GISTs may occur due to intussusception, volvulus, luminal obstruction or rarely by torsion and adhesion [5,7]. Larger tumours may cause luminal obstruction, while smaller tumours have tendency to twist, adhere and kink the affected bowel [7].…”

“…GI symptoms account for less than 5 % of patients [3,5,6]. GISTs may cause chronic or intermittent bleeding, perforation and very uncommonly small bowel obstruction [1,5,7]. We report an unusual case of acute small bowel obstruction in an elderly woman with NF1 caused by numerous jejunal GISTs.…”

“…As these tumours are mostly smaller in size, they grow mainly in an extraluminal fashion. GI symptoms account for less than 5 % of patients [3,5,6]. GISTs may cause chronic or intermittent bleeding, perforation and very uncommonly small bowel obstruction [1,5,7].…”