Lauretti S, Cappa M, Emiliozzi P, Casareale P, Ianari A, Defidio L
Divisione di Urologia, Ospedale CTO, Roma.
Minerva Chir. 1998 Jan-Feb;53(1-2):77-81.
Retroperitoneal soft-tissue sarcomas are a heterogeneous group of rare and peculiar mesenchymal tumors. They are locally invasive and have a peak incidence in the fifth decade of life. They account for 0.1-0.2% of all solid tumors and 15% of all soft-tissue tumors. Liposarcomas are usually large and occur most frequently in the lower extremities, in the retroperitoneal, perineal and mesenteric region. In the retroperitoneum they grow slowly due to the ability of the abdominal cavity to accommodate these slowly expanding masses. They don't produce symptoms until they are very large and have invaded local tissues. The case of a 61-year old man with a retroperitoneal liposarcoma is reported. The tumor was discovered due to the association of abdominal mass, weight loss and persistent fever. The fever, especially, is present due to a wide tumor necrosis. The diagnosis was suggested by computed tomography. Normally, the interval between start of symptoms and diagnosis is included within three weeks and one year. Surgical complete resection of the mass with splenectomy and local postoperative radiotherapy were performed. The weight of the mass was 8.56 kilograms and the pathological evaluation showed a pleomorphic highly undifferentiated liposarcoma. This histological type normally presents many tumor giant cells, some of which have the features of lipoblasts. The single most important prognostic factor in patients with soft-tissue sarcomas is the histologic grade of the primary lesion. In the last AJCC Staging System the grades are assigned from grade 1 (well differentiated) to grade 3 (poorly differentiated). The present case is grade 3. In the treatment of sarcoma of the retroperitoneum or genitourinary tract, the conventional chemotherapy does not seem effective, while radiotherapy has a little improvement on survival. Local recurrences are frequent, especially in the first three years, often in the absence of distant metastases. When the tumor recurs locally, the best therapy is still to remove the mass. Sometimes, two or more operations may be necessary for the patient. Generally, the prognosis is poor with overall 5-year survival of 15-50%. The patient was admitted in our division 4 months after the first operation with poor medical condition. The patient died nine months after surgery.
腹膜后软组织肉瘤是一组罕见且特殊的异质性间充质肿瘤。它们具有局部侵袭性，发病高峰在50岁左右。它们占所有实体瘤的0.1 - 0.2%，占所有软组织肿瘤的15%。脂肪肉瘤通常体积较大，最常发生于下肢、腹膜后、会阴和肠系膜区域。在腹膜后，由于腹腔能够容纳这些缓慢生长的肿块，它们生长缓慢。直到长得非常大并侵犯局部组织时才会产生症状。本文报道了一例61岁患有腹膜后脂肪肉瘤的男性病例。该肿瘤因腹部肿块、体重减轻和持续发热而被发现。特别是发热是由于广泛的肿瘤坏死所致。通过计算机断层扫描做出了诊断。通常，症状出现到诊断的间隔时间在三周至一年之间。对肿块进行了手术完整切除并切除脾脏，术后进行了局部放疗。肿块重量为8.56千克，病理评估显示为多形性高度未分化脂肪肉瘤。这种组织学类型通常有许多肿瘤巨细胞，其中一些具有脂肪母细胞的特征。软组织肉瘤患者最重要的单一预后因素是原发灶的组织学分级。在最新的美国癌症联合委员会（AJCC）分期系统中，分级从1级（高分化）到3级（低分化）。本病例为3级。在腹膜后或泌尿生殖道肉瘤的治疗中，传统化疗似乎无效，而放疗对生存率略有改善。局部复发很常见，尤其是在头三年，且往往没有远处转移。当肿瘤局部复发时，最佳治疗方法仍然是切除肿块。有时，患者可能需要进行两次或更多次手术。一般来说，预后较差，总体5年生存率为15 - 50%。患者在首次手术后4个月因病情不佳入住我科。患者术后9个月死亡。
