Abstract
Gastrointestinal stromal tumors (GISTs) are rare tumors of the gastrointestinal tract accounting for less than 1% of all gut tumors. GISTs occurring in the rectum are extremely rare and these usually present at an advanced stage compared with other sites. We report a case of a middle-aged female who presented with features of anemia and subacute obstruction due to a large rectal tumor and underwent abdominoperineal resection. The histopathological examination confirmed the diagnosis of high-grade malignant GIST with multiple lymph nodal metastasis. She was started on adjuvant imatinib therapy and is on follow-up without any evidence of recurrence. The authors conclude that GIST must be included in the differential diagnosis of a rectal tumor. Diagnosis is established by biopsy and immunohistochemistry studies. Surgical resection with histological negative margins is the standard curative treatment. Adjuvant targeted therapy can reduce long-term recurrence in high-risk cases.