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腹膜后软组织肉瘤:原发性和复发性疾病的预后与治疗

Retroperitoneal soft-tissue sarcomas: prognosis and treatment of primary and recurrent disease.

作者信息

Bautista N, Su W, O'Connell T X

机构信息

Department of Surgery, Kaiser Permanente Medical Center, Los Angeles, California 90027, USA.

出版信息

Am Surg. 2000 Sep;66(9):832-6.

PMID:10993610
Abstract

Soft-tissue sarcomas of the retroperitoneum constitute a heterogeneous group of tumors with varying histology, potential for complete resection, and propensity for recurrent disease-making the development of effective treatment difficult and challenging. A retrospective review of 23 patients with retroperitoneal sarcomas from 1985 through 1998 was performed to assess the biological behavior and clinical outcomes and to identify factors that may influence prognosis and optimize treatment strategy. Liposarcomas were the most common pathology (61%); 79 per cent of these were of low grade. Leiomyosarcomas were the next most common pathology (30%); 43 per cent of these were of low grade. Low-grade sarcomas overall accounted for 62 per cent of the total group. Low-grade tumors independent of histologic type exhibited good prognosis for long-term survival with a median survival of 44 months. In contrast, intermediate- or high-grade tumors were associated with a median survival of only 9 months (P < 0.02). On the other hand, tumor histologic type independent of grade did not have a significant survival difference. Complete tumor resection was possible in 21 of 23 patients, which gives an overall resectability rate of 91 per cent. Eight patients (36%) remain disease-free after initial surgical treatment. However, local recurrence was common; this occurred in 11 of 22 patients (50%). Local recurrence, however, did not preclude long-term survival. Surgical resection of recurrent disease was done in nine patients with a median survival of 91 months (range 24-150 months). Three patients had as many as three operations for recurrent disease. With subsequent recurrences there was a decrease in interval from approximately 4 years to 2 years, and 33 per cent of these patients developed tumor dedifferentiation to high grade. An aggressive surgical approach with reoperation can produce prolonged survival in patients with low-grade retroperitoneal sarcoma.

摘要

腹膜后软组织肉瘤是一组异质性肿瘤,其组织学类型多样,完整切除的可能性不同,且复发倾向各异,这使得有效治疗方案的制定困难且具有挑战性。对1985年至1998年间23例腹膜后肉瘤患者进行了回顾性研究,以评估其生物学行为和临床结局,并确定可能影响预后的因素,从而优化治疗策略。脂肪肉瘤是最常见的病理类型(61%);其中79%为低级别。平滑肌肉瘤是第二常见的病理类型(30%);其中43%为低级别。低级别肉瘤总体占总病例数的62%。与组织学类型无关的低级别肿瘤长期生存预后良好,中位生存期为44个月。相比之下,中级或高级别肿瘤的中位生存期仅为9个月(P<0.02)。另一方面。与级别无关的肿瘤组织学类型在生存率上无显著差异。23例患者中有21例可行肿瘤完整切除,总体可切除率为91%。8例患者(36%)在初次手术治疗后无疾病复发。然而,局部复发很常见;22例患者中有11例(50%)出现局部复发。不过,局部复发并不排除长期生存。9例复发病例接受了手术切除,中位生存期为91个月(范围24 - 150个月)。3例患者因复发病例接受了多达三次手术。随着后续复发,复发间隔从约4年缩短至2年,且这些患者中有33%的肿瘤发生去分化至高级别。对于低级别腹膜后肉瘤患者,积极的手术方法及再次手术可延长生存期。

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