Department of Pathology and Laboratory Medicine, University of Miami, Miami, FL.
Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA.
Hum Pathol. 2020 Mar;97:80-93. doi: 10.1016/j.humpath.2019.12.004. Epub 2019 Dec 26.
Primary gastrointestinal liposarcoma is rare, and information regarding this entity is largely based on single case studies. We report on 8 patients with primary liposarcoma of the gastrointestinal tract and review the pertinent literature. The cohort includes 6 men and 2 women who ranged in age from 51 to 81 years (median 68.5). Two tumors arose in the stomach, 4 in the small intestine, and 2 in the large intestine. Tumors ranged in size from 2.5 to 14.5 cm (median 7 cm), originated in the submucosa or muscularis propria of the intestinal wall, and frequently protruded into the bowel lumen, resulting in mucosal ulceration and luminal obstruction. Six tumors were dedifferentiated liposarcomas, and 2 were well-differentiated liposarcoma. Surgical excision was performed on all tumors except for 1 case of dedifferentiated liposarcoma. On follow-up, 1 patient with dedifferentiated liposarcoma developed a lytic sacral lesion suspicious for metastasis 4 months after resection of the primary, and another underwent marginal resection and presented with recurrence 4 years later, had tumor re-resection, and was considered disease-free at 6 weeks postsurgery. A third patient with dedifferentiated liposarcoma was alive with unknown disease status at 17 months following surgery, and another patient with dedifferentiated liposarcoma was alive without evidence of disease at 30 months following surgery. No follow-up information on the remaining patients is available. Overall, liposarcomas of the intestinal tract are most frequently high-grade dedifferentiated tumors that are biologically aggressive and require surgical excision with widely negative margins to help reduce the risk of local recurrence and dissemination. Important in the differential diagnosis is malignant gastrointestinal stromal tumor. Care must be taken not to misdiagnose one entity for the other because the correct diagnosis carries important therapeutic implications.
原发性胃肠道脂肪肉瘤较为罕见，其相关信息主要基于个案研究。我们报道了 8 例原发性胃肠道脂肪肉瘤患者，并复习了相关文献。该队列包括 6 名男性和 2 名女性，年龄 51-81 岁，中位年龄 68.5 岁。2 例肿瘤发生于胃，4 例发生于小肠，2 例发生于大肠。肿瘤大小为 2.5-14.5cm，中位大小 7cm，起源于肠壁黏膜下层或固有肌层，常向肠腔突出，导致黏膜溃疡和管腔阻塞。6 例肿瘤为去分化脂肪肉瘤，2 例为高分化脂肪肉瘤。所有肿瘤均行手术切除，除 1 例去分化脂肪肉瘤外。随访发现，1 例去分化脂肪肉瘤患者在原发肿瘤切除后 4 个月出现溶骨性骶骨病变，疑似转移；另 1 例患者行边缘性切除，4 年后复发，再次切除肿瘤，术后 6 周无疾病进展。1 例去分化脂肪肉瘤患者在手术后 17 个月仍存活，且疾病状态未知；另 1 例去分化脂肪肉瘤患者在手术后 30 个月仍存活，且无疾病证据。其余患者无随访信息。总体而言，肠道脂肪肉瘤多为高级别去分化肿瘤，生物学侵袭性强，需要行广泛的阴性切缘手术切除，以降低局部复发和播散的风险。在鉴别诊断中，需要与恶性胃肠道间质瘤相鉴别。必须谨慎避免误诊，因为正确的诊断具有重要的治疗意义。
