Svarvar Catarina, Böhling Tom, Berlin Orjan, Gustafson Pelle, Follerås Gunnar, Bjerkehagen Bodil, Domanski Henryk A, Sundby Hall Kirsten, Tukiainen Erkki, Blomqvist Carl
Department of Plastic Surgery, Helsinki University Central Hospital, Helsinki, Finland.
Cancer. 2007 Jan 15;109(2):282-91. doi: 10.1002/cncr.22395.
Leiomyosarcoma of nonvisceral soft tissues is an uncommon malignant tumor; thus, only small numbers of cases have been reported. This study was based on a large series of patients from the Scandinavian Sarcoma Group Register acquired during a 15-year period (from 1986 to 2001). Follow-up information was available for all patients.
The authors analyzed the clinical features of 225 patients with cutaneous, subcutaneous, or deep-seated leiomyosarcoma of the extremities, trunk wall, and superficial parts of the head and neck region to determine the natural course of the disease. Only patients who received their treatment at a specialist sarcoma center were included. Re-evaluation of histopathology was performed.
The age of the patients (121 women and 104 men) ranged from 20 years to 98 years (median, 70 years), and the tumors ranged in size from 0.6 cm to 35 cm (median, 4.0 cm). Eighty-two percent of the tumors were classified as high grade. The median follow-up for survivors was 5.5 years. The local treatment was adequate in 154 of 206 patients (75%) who were without metastasis at presentation. At 10 years, 84% of the 206 patients with localized disease at presentation were free from local recurrence, 66% remained metastasis free, and 49% were alive. Multivariate analysis showed that higher malignancy grade (P = .006), larger tumor size (P = .003), and deeper tumor location (P = .002) were correlated significantly with decreased metastasis-free survival, inadequate local treatment was correlated with local recurrence (P = .007), and high malignancy grade was correlated with decreased overall survival (P = .007).
The long-term prognosis for patients with subcutaneous and deep-seated soft tissue leiomyosarcoma remains poor despite the ability to achieve adequate local control through nonmutilating surgery with or without radiotherapy.
非内脏软组织平滑肌肉瘤是一种罕见的恶性肿瘤，因此仅有少量病例报道。本研究基于斯堪的纳维亚肉瘤小组登记处15年期间（1986年至2001年）收集的大量患者。所有患者均有随访信息。
作者分析了225例患有四肢、躯干壁以及头颈部浅表部位皮肤、皮下或深部平滑肌肉瘤患者的临床特征，以确定该疾病的自然病程。仅纳入在肉瘤专科中心接受治疗的患者。对组织病理学进行了重新评估。
患者（121名女性和104名男性）年龄在20岁至98岁之间（中位数为70岁），肿瘤大小在0.6厘米至35厘米之间（中位数为4.0厘米）。82%的肿瘤被归类为高级别。幸存者的中位随访时间为5.5年。206例初诊时无转移的患者中，154例（75%）的局部治疗充分。初诊时患有局限性疾病的206例患者中，10年后84%无局部复发，66%无转移，49%存活。多变量分析显示，较高的恶性程度（P = .006）、较大的肿瘤大小（P = .003）和较深的肿瘤位置（P = .002）与无转移生存期缩短显著相关，局部治疗不充分与局部复发相关（P = .007），高恶性程度与总生存期缩短相关（P = .007）。
尽管通过保肢手术联合或不联合放疗能够实现充分的局部控制，但皮下和深部软组织平滑肌肉瘤患者的长期预后仍然较差。
