Okcu M F, Despa S, Choroszy M, Berrak S G, Cangir A, Jaffe N, Raney R B
Division of Pediatrics, The University of Texas M.D. Anderson Cancer Center, Houston, TX 77030, USA.
Med Pediatr Oncol. 2001 Aug;37(2):90-6. doi: 10.1002/mpo.1175.
Synovial sarcoma (SS) is the most common type of non-rhabdomyosarcoma soft tissue sarcoma in childhood, with controversies about its prognosis and treatment.
We reviewed medical records of 42 children and adolescents with SS treated at our institution between 1966 and 1999 to determine treatment results and assess prognostic factors.
With a median follow-up duration of 7.8 years (range 0.2-22.4 years), 5-year progression free survival (PFS) and overall survival (OS) rates were 75.6% (95% Confidence Interval [CI] 62-89.2%) and 87.7% (95% CI 77.3-98.1%) respectively. Eleven patients were dead and four others had progressed but were alive without evidence of disease after further therapy. IRS grouping and tumor invasiveness were found to be significant prognostic indicators (P < 0.01 and = 0.02, respectively). Patients with initial gross total resection (IRS I and II) and non-invasive tumors (T1) were most likely to have prolonged PFS and OS. Patients with small tumors (< 5 cm) (P = 0.09) or with monophasic histology (P = 0.14) had better PFS and OS.
Achieving a complete resection or gross total resection with microscopic residual disease is vital for survival of patients with localized SS. Patients with localized disease who received radiotherapy had improved local control. Chemotherapy did not seem to impact PFS or OS. Future large multi-institutional trials are needed to address whether post-operative chemotherapy is necessary for patients with localized, surgically removed tumors, whether radiotherapy is necessary for patients with completely resected tumors, and to ascertain the order of importance of all the candidate prognostic markers. Med Pediatr Oncol 2001;37:90-96.
滑膜肉瘤（SS）是儿童期最常见的非横纹肌肉瘤软组织肉瘤类型，其预后和治疗存在争议。
我们回顾了1966年至1999年间在我院接受治疗的42例儿童和青少年滑膜肉瘤患者的病历，以确定治疗结果并评估预后因素。
中位随访时间为7.8年（范围0.2 - 22.4年），5年无进展生存率（PFS）和总生存率（OS）分别为75.6%（95%置信区间[CI] 62 - 89.2%）和87.7%（95% CI 77.3 - 98.1%）。11例患者死亡，另外4例病情进展，但在进一步治疗后仍存活且无疾病证据。国际横纹肌肉瘤研究协作组（IRS）分组和肿瘤侵袭性被发现是重要的预后指标（分别为P < 0.01和 = 0.02）。初始行大体肿瘤全切（IRS I和II）且肿瘤无侵袭性（T1）的患者最有可能获得较长的无进展生存期和总生存期。肿瘤较小（< 5 cm）（P = 0.09）或组织学类型为单相型（P = 0.14）的患者无进展生存期和总生存期较好。
对于局限性滑膜肉瘤患者，实现完全切除或带有镜下残留病灶的大体肿瘤全切对生存至关重要。接受放疗的局限性疾病患者局部控制情况有所改善。化疗似乎并未影响无进展生存期或总生存期。未来需要开展大型多机构试验，以解决局限性、经手术切除肿瘤的患者术后是否需要化疗，完全切除肿瘤的患者是否需要放疗，并确定所有候选预后标志物的重要性顺序。《医学与儿科肿瘤学》2001年；37:90 - 96。
