Gastrointestinal stromal tumor of the anus.

We report a rare case of gastrointestinal tumor of the anus with an unusual presentation as a perianal lipoma. A 65-year-old man presented with a 2-month history of a painless perianal lump clinically resembling a perianal lipoma. Endoanal ultrasonography revealed a 3 x 3 cm2 mass in the intersphincteric plane. Following initial excision of the lesion, histological analysis revealed a stromal lesion comprising fascicles of spindle cells with a mitotic count of 4 per 50 x high power field. Immunohistochemical analysis displayed positive reactivity for CD34 with focal staining for CD117; S100, smooth muscle actin and desmin were not expressed. No evidence of local or distant metastatic disease was found on computed tomography of the abdomen and pelvis. The patient subsequently underwent abdominoperineal resection. The resected specimen contained a mural nodule measuring 0.7 cm, located 5 cm from the distal margin and 2 mm from the radial margin. Histological analysis confirmed a stromal tumor composed of spindle cells with mitoses up to 2 per 10 x high power field. The patient's recovery was uneventful and he was free of recurrence at the 1-year follow-up. Gastrointestinal stromal tumors of the anal canal are an extremely rare occurrence, and may mimic benign perianal lesions. Tumor size and mitotic count are the most important factors in prognosticating outcome. Oncologic resection and protracted follow-up must factor in their predilection for late recurrence and metastatic spread. The role of adjuvant therapy with STI571 here remains to be clearly defined.