An unusual lead point: jejunal leiomyosarcoma causing jejuno-jejunal intussusception - a case report.

Abstract

Introduction: Adult intussusception is rare. Unlike in pediatric cases, adult intussusception typically arises from an underlying pathology. Primary jejunal leiomyosarcoma is an exceptionally rare malignant tumor originating from the smooth muscle cells of the small intestine.

Case presentation: A 52-year-old man presented with signs and symptoms of acute intestinal obstruction, including abdominal pain, constipation, vomiting, and abdominal distension of 2 days duration. Contrast-enhanced computed tomography revealed a jejunojejunal intussusception with a lead-point intraluminal mass. Exploratory laparotomy showed a jejuno-jejunal intussusception 40 cm distal to the duodenojejunal flexure. Segmental resection with primary anastomosis was performed. The postoperative period was uneventful. Histopathology demonstrated a spindle-cell neoplasm consistent with leiomyosarcoma. Immunohistochemistry showed strong positivity for smooth muscle actin and desmin, and negativity for CD117, DOG-1, and S-100.

Discussion: Primary jejunal leiomyosarcomas often present with nonspecific symptoms. Imaging, especially CT, is critical for diagnosis, but confirmation requires histopathology and immunohistochemistry. Complete surgical resection remains the treatment of choice. This case contributes a complete immunohistochemical panel that distinguishes leiomyosarcoma from gastrointestinal stromal tumors, the formal AJCC 8th edition staging, and a rationale for adjuvant chemotherapy based on soft tissue sarcoma protocols - thereby providing a more standardized diagnostic and management template than existing jejunal leiomyosarcoma case reports.

Conclusion: Jejunal leiomyosarcoma presenting as jejuno-jejunal intussusception is exceptionally rare. Early recognition and prompt surgical intervention are essential for favorable outcomes.