Anaplastic lymphoma kinase-negative inflammatory myofibroblastic tumor of the appendix and ileocecal region: a rare clinical entity

Inflammatory myofibroblastic tumor (IMT), historically misclassified as inflammatory pseudotumor, is a rare, intermediate-grade neoplasm with low metastatic potential and a high recurrence rate. It primarily affects the lungs, with colorectal involvement being exceptionally uncommon. We present the case of a 32-year-old woman initially diagnosed with Stage III pelvic inflammatory disease, whose surgical exploration revealed an abscessed ileocecal mass. A right hemicolectomy was successfully performed, and histopathological examination confirmed an anaplastic lymphoma kinase (ALK)-negative IMT. The diagnosis is challenging due to the nonspecific nature of clinical and radiologic findings and significant histologic overlap with other spindle cell tumors. Immunohistochemistry and molecular profiling are essential for accurate classification, particularly in ALK-negative cases. Surgical resection remains the mainstay of treatment. This case highlights the importance of including IMT in the differential diagnosis of atypical intra-abdominal masses and supports the role of advanced molecular techniques in improving diagnostic accuracy and management.