Gastrointestinal Stromal Tumors

The Inside Mystery of Jejunal Gastrointestinal Stromal Tumor: A ...

Abstract

Gastrointestinal stromal tumors (GISTs) are malignant and rare form of soft tissue sarcoma of the digestive tract. The incidence of gastrointestinal stromal tumors is very low Kramer et al. 2005 Jejunal GISTs are extremely rare. Here we present a rare case of jejunal GIST with unusually large size at presentation. The patient presented with severe abdomen pain, exophytic growth, and dimorphic anemia. Surgical resection of the tumor was carried out, and operative findings revealed a 15 × 10 cm growth, arising from serosal surface of jejunum, at the antimesenteric surface. Diagnosis in this case was made by subjecting the resected specimen to immunohistochemical analysis. In view of large size of the resected tumor, and high-risk histopathological features, imatinib mesylate 400 mg once daily was given as adjuvant chemotherapy. Patient is asymptomatic without any evidence of tumor recurrence after six months of postoperative followup. Imatinib as such is recommended in metastatic, residual or recurrent cases of GISTs or which are surgically not removable; however, recent recommendations suggests the use of imatinib mesylate after radical surgery in high-risk cases, because it has shown a significant decrease in the recurrence rate, and the Food and Drug Administration (FDA) has also approved the use of imatinib as adjuvant therapy after complete resection of localized, primary GIST.

Gastrointestinal stromal tumors (GISTs) are malignant and rare form of soft tissue sarcoma of the digestive tract. Most common site of presentation is stomach, but it can crop up anywhere in the digestive tract. Two-thirds of GISTs occur in the stomach [ 1 ] while about one-fourth develop in the small intestine, usually in the duodenum [ 2 ]. GISTs are uncommon mesenchymal neoplasms of the alimentary tract. The incidence of GIST is very low (i.e., 2 in 1,00,000) while jejunal GIST is extremely rare accounting for 0.1–3% of all gastrointestinal (GI) tumors [ 3 ]. Usually they are asymptomatic but can present as abdominal pain, bleeding, or mechanical obstruction. Exophytic growths of these tumors have been noted in 18–30% of cases [ 4 ]. We present a rare case of jejunal GIST with unusual size of presentation and with history of severe pain abdomen, exophytic growth, and dimorphic anemia.

A 38-year-male patient presented with two-month-old history of pain abdomen and feeling of heaviness in right lower abdomen. Pain was severe in intensity and relieved only after taking medication. Patient also gave a history of lump in the abdomen. General physical and systemic examination was normal. Local examination of abdomen and pelvis revealed a hard mass measuring around 15 × 10 cm in size in the right iliac region. Complete hemogram of the patient was suggestive of dimorphic iron deficiency anemia with thrombocytosis. Routine blood biochemistry parameters of the patient were within normal limits.

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