Department of Sarcoma, Moffitt Cancer Center, Tampa, FL, USA.
Cutaneous Oncology Department, Moffitt Cancer Center, Tampa, FL, USA.
Cancer Control. 2023 Jan-Dec;30:10732748231206957. doi: 10.1177/10732748231206957.
Primary cutaneous leiomyosarcoma (cLMS), a rare, typically intradermal tumor, has previously been reported to exhibit an indolent course of disease with zero-to-low risk of local recurrence or distant metastasis. This study seeks to evaluate recurrence and survival of cLMS patients through study of its clinicopathologic and treatment characteristics.
All patients included underwent resection of primary cLMS at this institution between 2006 and 2019. A retrospective cohort study analysis of clinicopathologic characteristics, treatment, recurrence, and overall survival was performed. Data was assessed through descriptive statistics and outcome measures assessed by Cox proportional models and log-rank tests.
Eighty-eight patients with cLMS were evaluated. The majority were men (n = 68, 77%) and Caucasian (n = 85, 97%), with median age at diagnosis of 66 years (range 20-96). 65% of tumors were located on the extremities, with a median size of 1.3 cm (range .3-15). Assessment revealed low (n = 41, 47%), intermediate (n = 29, 33%), and high (n = 18, 20%) grade tumors, demonstrating extension into subcutaneous tissue in 38/60 (60%), with 3 patients exhibiting extension into muscle (3%). All underwent resection as primary treatment with median 1 cm margins (range .5-2). With median follow-up of 27.5 months (IQR 8-51; range 1-131), no low-grade cases had recurrence or death while there was a recurrence rate of 19.1% (9/47) and death rate of 8.5% (4/47) in intermediate- to high-grade cases.
Primary tumor resection of cLMS provides excellent local control for low-grade tumors as no low-grade cases experienced recurrence. For patients with intermediate- to high-grade tumors, there is potential for local recurrence, distant metastasis, and death, and therefore surveillance following treatment is encouraged.
原发性皮肤平滑肌肉瘤(cLMS)是一种罕见的、典型的真皮内肿瘤,其疾病进程一直被认为是惰性的,局部复发或远处转移的风险极低。本研究旨在通过分析其临床病理和治疗特征,评估 cLMS 患者的复发和生存情况。
本机构于 2006 年至 2019 年间对所有接受原发性 cLMS 切除术的患者进行了回顾性队列研究分析。对临床病理特征、治疗、复发和总生存进行了回顾性分析。采用描述性统计学方法对数据进行评估,并采用 Cox 比例模型和对数秩检验对结局指标进行评估。
共评估了 88 例 cLMS 患者。患者中男性居多(n=68,77%),白人(n=85,97%),中位诊断年龄为 66 岁(范围 20-96 岁)。65%的肿瘤位于四肢,中位大小为 1.3cm(范围 0.3-15cm)。评估结果显示,低级别(n=41,47%)、中级别(n=29,33%)和高级别(n=18,20%)肿瘤分别有 38/60(60%)例肿瘤侵犯皮下组织,3 例(3%)肿瘤侵犯肌肉。所有患者均接受手术切除作为主要治疗手段,中位切缘为 1cm(范围 0.5-2cm)。中位随访时间为 27.5 个月(IQR 8-51;范围 1-131),低级别病例无一例复发或死亡,而中高级别病例的复发率为 19.1%(9/47),死亡率为 8.5%(4/47)。
对于低级别肿瘤,cLMS 患者行原发性肿瘤切除术可获得极好的局部控制效果,因为没有低级别病例出现复发。对于中高级别肿瘤患者,存在局部复发、远处转移和死亡的风险,因此鼓励在治疗后进行监测。