Malignant peripheral nerve sheath tumor of the ileocecal region: a case report

Malignant peripheral nerve sheath tumor (MPNST) is a rare and aggressive soft tissue sarcoma that rarely occurs in the gastrointestinal tract. To date, fewer than ten cases of primary colonic MPNST have been reported, and this case may represent the first case report of an MPNST arising in the ileocecal region.

A 62-year-old male patient presented with right lower abdominal pain for two days. A firm, fixed mass approximately 10 cm in diameter was palpable in his right lower abdomen. Auxiliary examinations including abdominal CT and colonoscopy revealed various features suggestive of malignant neoplasm, such as: a large, irregularly shaped tumor with infiltrative growth, accompanied by hemorrhagic erosion and enlarged regional lymph nodes. Although endoscopic biopsy suggested high-grade mucosal intraepithelial neoplasia, clinical suspicion remained strongly focused on a malignant tumor of the ileocecal region. Due to concomitant intestinal obstruction symptoms, the patient underwent laparoscopic radical right hemicolectomy following multidisciplinary consultation. Ultimately, multiple pathological examinations combined with immunohistochemistry confirmed the diagnosis of MPNST of the ileocecal region. Follow-up examination six months later revealed no evidence of recurrence or metastasis.

This case provides novel anatomical and clinical information regarding MPNST, a rare and aggressive neoplasm. Due to its atypical clinical manifestations and overlapping histopathological features with other colonic tumors, preoperative definitive diagnosis is difficult and misdiagnosis is common. A confirmed diagnosis relies on postoperative pathological examination and immunohistochemistry. Surgical resection with confirmation of tumor-free margins represents the preferred therapeutic approach.