Ann Ital Chir. 2020 Aug 28;9:S2239253X20033162.
Retroperitoneal soft-tissue sarcoma is a very rare neoplasm, the most frequent histological subtype is liposarcoma with up to 45% of all cases. Unspecific clinical presentation, late diagnosis and high local recurrence rate represent important problems in clinical practice. We present the case of an adult patient with an unusual large liposarcoma of the retroperitoneum analyzing diagnostic workup, surgical approach and therapeutic strategies.
A 68-years old female was admitted with weight gain (+12 kg) and increasing abdominal girth. Computed tomography scan imaging showed a retroperitoneal tumor with 40 cm maximum diameter. Biopsy revealed a myxoid liposarcoma. The interdisciplinary curative surgical treatment included preoperative ureteral splinting, en-bloc tumorexstirpation, ileocecal resection, right ureteral resection and vascular reconstruction of the Arteria iliaca communis. The postoperative course was uneventful. After sixteen months the patient developed multifocal local recurrence requiring extensive surgical resection of tumor and retroperitoneal fat (Figs. 3, 4). However, thirteen months later the tumor reappeared and the patient was assigned to palliative chemotherapy. The patient is still alive with stable tumor disease.
The removal of a huge retroperitoneal sarcoma is a significant challenge for the surgeon. Accurate planning, interdisciplinary treatment options, and radical surgery are essential. However, the recurrence risk is exceptionally high because of the enormous tumor dimensions and the big tumor surface, multimodal therapeutic approaches may improve the outcome in these patients.
Liposarcoma, Retroperitoneum, Surgery.
腹膜后软组织肉瘤是一种非常罕见的肿瘤，最常见的组织学亚型是脂肪肉瘤，约占所有病例的 45%。临床表现不典型、诊断较晚和局部复发率高是临床实践中的重要问题。我们报告了一例成人腹膜后罕见巨大脂肪肉瘤病例，分析了其诊断、手术方法和治疗策略。
一名 68 岁女性因体重增加（+12 公斤）和腹部周长增大而入院。计算机断层扫描显示腹膜后肿瘤最大直径为 40 厘米。活检显示为黏液样脂肪肉瘤。多学科治疗性手术包括术前输尿管支架置入、整块肿瘤切除、回盲部切除、右输尿管切除和髂总动脉血管重建。术后过程顺利。16 个月后，患者出现多灶性局部复发，需要广泛切除肿瘤和腹膜后脂肪（图 3、4）。然而，13 个月后肿瘤再次出现，患者接受了姑息性化疗。患者目前仍存活，肿瘤病情稳定。
巨大腹膜后肉瘤的切除对外科医生来说是一个重大挑战。准确的规划、多学科治疗选择和根治性手术是必要的。然而，由于肿瘤体积巨大和肿瘤表面巨大，复发风险极高，多模式治疗方法可能改善这些患者的预后。
脂肪肉瘤、腹膜后、手术。
