An Unusual Small Bowel Lesion

摘要

A 58-year-old black female, born in Angola and living in Portugal, presented with melena, occasional vomiting, and asthenia for 1 month. She also complained of anorexia and weight loss (40 kg/88 lb) in the past 2 years and reported a 7-year history of a right thigh mass that had progressively increased in size, with an inconclusive biopsy performed in Angola. Her laboratory tests revealed iron deficiency anemia (hemoglobin 4.4 g/dL). Computed tomography (CT) showed a large solid lesion in the right thigh (Fig. ​(Fig.1a),1a), multiple smaller lesions in the thyroid, lungs, pancreas, adrenal gland, uterus, and soft tissues, focal thickness of the small bowel wall and colon, as well as jejunal intussusception, distal to the angle of Treitz (Fig. 1b, c). Upper gastrointestinal endoscopy revealed multiple ulcerative mucosal lesions with elevated edges throughout the second and third portions of the duodenum, with 10–20 mm, without hemorrhagic stigmata and surrounded by normal mucosa (Fig. ​(Fig.2).2). The duodenal and thigh lesions were biopsied, and histology showed irregularly intersecting fascicles of spindled cells with indistinct cytoplasmic contour and a cigar-shaped nucleus and areas of necrosis. Immunohistochemically, the tumor cells were positive for α-smooth muscle actin, desmin, and h-caldesmon, and negative for CD117, CK AE1/AE3, and S-100 protein. Therefore, a stage IV leiomyosarcoma was diagnosed. Due to persistent melena, endoscopy was repeated and showed an ulcerative lesion in the duodenum with active oozing. Hemostasis was achieved after adrenaline injection and coagulation with argon plasma. Nausea and vomiting relieved with conservative measures and jejunal intussusception reduced spontaneously. The patient started chemotherapy with doxorubicin. However, there was disease progression and she died 8 months after the diagnosis.