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影响非典型皮内平滑肌肿瘤患者的临床变量(以前是皮肤平滑肌肉瘤):对95例外科手术患者的单机构研究

Clinical Variables Influencing Outcomes in Patients with Atypical Intradermal Smooth Muscle Neoplasms (Formerly Cutaneous Leiomyosarcomas): Single-Institution Study of 95 Surgical Patients

影响因子:3.50000
分区:医学2区 / 外科2区 肿瘤学3区
发表日期:2024 Nov
作者: Alicia Gingrich, Sintawat Wangsiricharoen, Madeline B Torres, Vinod Ravi, Ravin Ratan, Emily Z Keung, Christopher P Scally, Alexander J Lazar, Wei-Lien Wang, Christina L Roland, Kelly K Hunt, Wendong Yu, Keila E Torres

摘要

非典型的皮内平滑肌肿瘤,通常称为皮肤皮肤肉瘤,是一种软组织肿瘤,其侵略性行为风险较低。这些病变在真皮中出现,可能具有表面皮下延伸,证明了细胞学上的典型性,并且经常显示有丝分裂活性。对患者人口统计学,肿瘤特征和治疗方法的回顾性回顾是在2002年至2021年至2021年至2021年(N = 95)的连续系列患者中进行的。 MDACC病理学家审查了所有病理学,并确定为诊断时的非典型皮内平滑肌肿瘤。被确定为白人,非西班牙裔的患者百分之九十五(n = 90)。大多数肿瘤的生长缓慢,孤立性和无痛结节。肿瘤处于下肢(44.2%),其次是上肢(28.4%),树干(22.1%)和头颈(5.2%)。所有用于诊断目的的有打孔/切开活检的患者(n = 44,46.3%)随后进行了肿瘤切除术。对其余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名患者中有41个(89%)的边缘为正,并进行了重新分配。尽管最初的正缘,但在25/38(66%)重新测试中的最终病理对残留肿瘤为阴性。初次手术后的两名患者在局部复发2和3年。两名患者的边缘阳性,进行了复发性肿瘤的切除,并且没有疾病。在中位随访6。9年(范围1天至18年)之后,整个队列的5年无复发生存率为96%,整个队列的总生存率(OS)为78%。在这项连续的患者的研究中,我们发现了良好的OS OS和良好的对照,在与负面型号分离后,我们发现了良好的OS和局部性,包括良好的OS和局部性,包括良好的OS和局部性,包括消极的磁场,包括Codissionals coffisisssiss coffisisssiss。非典型的皮内平滑肌肿瘤不太可能转移,并且具有出色的预后。应重新审查确定这些患者最佳监视策略的准则。

Abstract

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.