Primary Jejunal Gastrointestinal Stromal Tumor: Diagnosis Delay of 3 Years but Successful Management in Early Stage (II) by Surgery and Adjuvant Therapy

Abstract

In the digestive system, mesenchymal origin of tumors is quite rare; in general, they are recognized as gastrointestinal stromal tumors (GISTs). The incidence of GISTs is very low (2 in 100,000), while jejunal GISTs are extremely rare, accounting for 0.1-3% of all gastrointestinal (GI) tumors. Small intestinal GISTs are the second most common (25%) site in the GI tract, usually occurring in the duodenum. We present the case of a 62-year-old Bangladeshi female with a history of GI bleeding 3 years earlier; the cause of the bleeding had not been found despite extensive investigations. In the meantime, the patient had developed occasional abdominal pain and lumpy feelings in the right side of the abdomen without any GI bleeding. Exploratory laparotomy was carried out in view of a small intestinal mesenteric mass in a computed tomography scan. On midline incision there was a 6 × 6 cm mass in the antimesenteric border of the jejunum approximately 30 cm from the duodenojejunal flexure, which was resected followed by anastomosis. The presentation of GISTs ranges from asymptomatic to mild abdominal pain and mass (5-50%) and mechanical obstruction (5%) as well as hemorrhage - perforation having rarely been reported (0.8%) - making the diagnosis difficult. Exophytic growth of these tumors has been noted in 18-30% of cases. In view of intermediate risk of malignancy, the patient was started with adjuvant imatinib 400 mg once daily due to probability of disease recurrence (24%).

Keywords: Adjuvant therapy; Gastrointestinal stromal tumor; Imatinib; Jejunum; Primary treatment.

Fig. 1

Computed tomography image of the abdomen and pelvis showing an inhomogeneous mass in the right iliac fossa.

Fig. 2

Peroperative specimen.

Fig. 3

Histopathology examination.

Fig. 4

Immunohistochemistry of CD117.