影响不典型皮内平滑肌瘤（原为皮肤平滑肌肉瘤）患者结局的临床变量：单一机构95例手术患者的研究

不典型皮内平滑肌瘤，也常称为皮肤平滑肌肉瘤，是一种具有低侵袭性风险的软组织肿瘤。这些病变起源于真皮层，可能伴有浅层皮下扩展，表现为细胞异型性，并常见有有丝分裂活性。我们回顾性分析了MD安德森癌症中心（MDACC）2002年至2021年期间连续收治的95例患者的病人资料、肿瘤特征及治疗方式。所有病理样本均由MDACC病理专家复审确认为不典型皮内平滑肌瘤。诊断时的中位年龄为58岁（范围22-86），男性占74%。95%的患者为白人非西班牙裔。大部分肿瘤生长缓慢，单发，无痛性结节，常见于下肢（44.2%），次为上肢（28.4%）、躯干（22.1%）及头颈部（5.2%）。接受穿刺/切开活检以明确诊断的患者（n=44，46.3%）随后进行了肿瘤切除。其余46例（48%）患者接受了非计划性切除或切除活检，其中89%的患者（38例中）切除边缘阳性，随后再次手术切除。尽管首次手术阳性边缘，66%的二次切除标本未见残留肿瘤。两名患者在手术后2年和3年出现局部复发，均伴有阳性边缘，经过再次切除后，患者无疾病迹象。经过中位随访6.9年（范围1天至18年），5年无复发生存率为96%，整体生存率（OS）为78%。本研究显示，经根治性手术切除（包括边缘清晰的切除活检）后，患者预后良好，局部控制良好。不典型皮内平滑肌瘤不太可能转移，预后优异。应重新审视制定最佳随访策略的指导原则。

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.