非典型皮内平滑肌肿瘤，通常也称为皮肤平滑肌肉瘤，是一种侵袭行为风险较低的软组织肿瘤。这些病变出现在真皮中，可能延伸至浅表皮下，表现出细胞学异型性，并且经常表现出有丝分裂活性。对患者人口统计、肿瘤特征和治疗方法进行了回顾性审查，对连续一系列就诊于 MD 安德森癌症中心的患者进行了回顾性研究（ MDACC）从 2002 年到 2021 年（n = 95）。所有病理学均经过 MDACC 病理学家审查，确定为非典型皮内平滑肌肿瘤。诊断时的中位年龄为 58 岁（范围 22-86），74% 为男性。百分之九十五 (n = 90) 的患者被确定为白人、非西班牙裔。大多数肿瘤是生长缓慢、孤立且无痛的结节。肿瘤分布在下肢（44.2%），其次是上肢（28.4%）、躯干（22.1%）和头颈部（5.2%）。所有出于诊断目的进行打孔/切开活检的患者（n = 44，46.3%）随后进行了肿瘤切除。其余 46 名 (48%) 患者进行了计划外切除或切除活检。在该子集中，上述 46 名患者中的 41 名 (89%) 具有阳性切缘并接受了再次切除。尽管最初切缘呈阳性，但 25/38 (66%) 的再切除标本的最终病理结果显示残留肿瘤呈阴性。该队列中的两名患者在初次手术后 2 年和 3 年出现局部复发。两名患者的切缘均呈阳性，并接受了复发肿瘤的切除术，并且没有患病。中位随访 6.9 年（范围 1 天至 18 年）后，整个队列的 5 年无复发生存率为 96%，总生存 (OS) 为 78%。皮内平滑肌肿瘤，我们在阴性切缘的明确手术切除（包括切缘紧密的切除活检）后发现良好的 OS 和局部控制。非典型皮内平滑肌肿瘤不易转移，预后良好。应重新审视为这些患者确定最佳监测策略的指南。© 2024。这是美国政府的作品，在美国不受版权保护；外国版权保护可能适用。

Atypical intradermal smooth muscle neoplasm, also commonly termed cutaneous leiomyosarcoma, is a soft tissue tumor with a low risk of aggressive behavior. These lesions arise in the dermis with possible superficial subcutaneous extension, demonstrate cytologic atypia, and often show mitotic activity.A retrospective review of patient demographics, tumor characteristics, and treatment methods was conducted in a consecutive series of patients presenting to MD Anderson Cancer Center (MDACC) from 2002 to 2021 (n = 95). All pathology was reviewed by MDACC pathologists and determined to be atypical intradermal smooth muscle neoplasm.Median age at diagnosis was 58 years (range 22-86), and 74% were male. Ninety-five percent (n = 90) of patients identified as White, non-Hispanic. Most tumors were slow-growing, solitary, and painless nodules. Tumors were in the lower extremities (44.2%), followed by the upper extremity (28.4%), trunk (22.1%), and head and neck (5.2%). All patients (n = 44, 46.3%) who had a punch/incisional biopsy for diagnostic purposes had a subsequent tumor excision. Unplanned excision or excisional biopsy was performed on the remaining 46 (48%) patients. Of this subset, 41 of the 46 aforementioned patients (89%) had positive margins and underwent re-excision. Final pathology in 25/38 (66%) re-excision specimens was negative for residual tumor despite an initial positive margin. Two patients in the cohort had local recurrence 2 and 3 years after initial surgery. Both patients had positive margins, underwent excision of the recurrent tumor, and remain free of disease. After median follow-up of 6.9 years (range 1 day-18 years), 5-year recurrence-free survival was 96% and overall survival (OS) of the entire cohort was 78%.In this study of consecutive patients presenting with atypical intradermal smooth muscle neoplasm, we found good OS and local control after definitive surgical excision with negative margins, including excisional biopsy with close margins. Atypical intradermal smooth muscle neoplasm is unlikely to metastasize and has an excellent prognosis. Guidelines to determine optimal surveillance strategies for these patients should be revisited.© 2024. This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply.