Peritonitis Secondary to Spontaneous Perforation of a Primary Gastrointestinal Stromal Tumor of The Small Intestine: A Case Report and Review of The Literature

Abstract: Gastro-intestinal Stromal tumours (GIST) account for less than 1% of Gastro-intestinal tract (GIT) neoplasms. GISTs are the most common mesenchymal tumours of the Gastro-intestinal tract and are associated with overall high rates of malignant transformation. GISTs are commonly found on abdominal Computed tomography (CT) scans, by chance during endoscopy of GIT, or during surgical manoeuvres initially carried out addressing different pathologies. Between 60 and 80% of patients are Symptomatic. However, these patients most commonly present with predominantly non-specific signs and symptoms which are not pathognomonic for GISTs. Such signs and symptoms include, but are not limited to: nausea, vomiting, distension of the abdomen, abdominal pain, early satiety, or possibly bleeding in GIT or a palpable intra-abdominal mass. Acute presentations of GISTs are few and far between, including larger tumours that may cause intestinal obstruction, constipation or compression of the GIT from exophytic growth leading to Dysphagia, obstructive jaundice depending on the location of the mass. In addition, GISTS can present as medical and surgical emergencies, however this group represents the least common presentation of this tumour. Such emergency presentations include acute obstruction, perforation, rupture, peritonitis and indolent or profuse intra-abdominal bleeding. Diagnosis is established via histo-pathological and immuno-chemistry studies, most GISTs stain positive for CD117 (C-Kit), CD34, and/or DOG-1. Molecularly targeted therapy, such as imatinib mesylate therapy, has revolutionized the treatment of advanced GIST and facilitates scientific research on this tumor entity. Nevertheless, surgery remains a mainstay of treatment to obtain a permanent cure for GIST even in the era of targeted therapy. Only a small number of cases of acute abdomen caused by spontaneous rupture of the small-intestinal gastrointestinal stromal tumors have been reported in the literature. Together with an extensive review of the literature, here we report a case of an elderly patient with peritonitis due to spontaneous perforation of a GIST of the jejunum. Literature Review: Via an extensive search in PubMed/Google Scholar, here we report 45 cases of spontaneous perforation of GIST of the small bowel with subsequent secondary peritonitis/abscess, in the period between 2002-2020. The Purpose of the literature review is to demonstrate the importance of considering GIST as a cause of acute peritonitis in any patient with a known intra-abdominal mass. Indeed, our literature review concludes that, the reported case is merely the tip of the iceberg. Our literature review suggests that spontaneous rupture/perforation of GISTs is a far more frequent first presentation of primary GISTs than previously thought. Clinicians need to have a high degree of clinical suspicion because of the absence of pathognomonic signs and symptoms. Suspect whenever there is a presentation of sudden abdominal pain in patients with an-intra abdominal mass. Prompt radical management is required in view of the high morbidity rates, especially with delay of diagnosis. Good clinical results are achievable when the diagnosis is made in a timely manner. The mainstay treatment for spontaneous rupture/perforation of GIST of small bowel is radical resection that achieves clear margins on both macroscopy and microscopy. The treatment part doesn’t end there at the operating table because once a diagnosis of GIST is confirmed by Histopathology, a multidisciplinary approach is required for starting adjuvant imatinib therapy in high risk GISTs, considering the notorious natural tendency of this tumor entity to reappear/re-occur both locally and distally.