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皮肤平滑肌瘤:152例患者的临床研究

Cutaneous Leiomyoma: A Clinical Study of 152 Patients.

作者信息

Marcoval Joaquim, Llobera-Ris Clàudia, Moreno-Vílchez Carlos, Penín Rosa María

机构信息

Department of Dermatology, Hospital Universitari de Bellvitge, IDIBELL, University of Barcelona, Barcelona, Spain.

Department of Pathology, Hospital Universitari de Bellvitge, IDIBELL, University of Barcelona, Barcelona, Spain.

出版信息

Dermatology. 2022;238(3):587-593. doi: 10.1159/000518542. Epub 2021 Sep 23.

Abstract

BACKGROUND

Cutaneous leiomyoma (CL) is a benign smooth muscle tumour included in painful skin tumours. Multiple CLs are cutaneous markers of hereditary leiomyomatosis and renal cell cancer (HLRCC).

OBJECTIVES

To retrospectively review our series of patients with CLs to analyse their clinical features and the association with HLRCC.

METHODS

Cases coded as CL in the database of the pathology department between 2004 and 2019 were included in the study. Medical records were retrospectively reviewed to obtain the following data: age, sex, location, number of lesions, diameter, evolution time at diagnosis, suspected clinical diagnosis, tenderness, status of resection margins, development of recurrence, follow-up time, and association with HLRCC.

RESULTS

152 patients had CLs, 89 women and 63 men, mean age 56.26, SD 16.030 years. Subtypes were piloleiomyoma in 62 patients, angioleiomyoma in 80, and genital leiomyoma in 10. All of our 11 patients with multiple lesions corresponded to piloleiomyomas, and HLRCC was confirmed in 8 of them (73%). Patients with HLRCC were younger than patients with piloleiomyomas without HLRCC (34.88 vs. 56.17 years, p = 0.009). Vascular and genital leiomyomyomas were solitary and were not associated with HLRCC.

CONCLUSION

In patients with multiple piloleiomyomas HLRCC must be ruled out as it is confirmed in a high proportion of cases. The probability of fumarate hydratase mutation is greater in multiple piloleiomyomas involving both the trunk and upper extremities in the same patient.

摘要

背景

皮肤平滑肌瘤(CL)是一种包含在疼痛性皮肤肿瘤中的良性平滑肌肿瘤。多发性CL是遗传性平滑肌瘤病和肾细胞癌(HLRCC)的皮肤标志物。

目的

回顾性分析我们收治的CL患者系列,以分析其临床特征及与HLRCC的关联。

方法

纳入2004年至2019年病理科数据库中编码为CL的病例。对病历进行回顾性分析,以获取以下数据:年龄、性别、部位、皮损数量、直径、诊断时的病程、疑似临床诊断、压痛、手术切缘情况、复发情况、随访时间以及与HLRCC的关联。

结果

152例患者患有CL,其中女性89例,男性63例,平均年龄56.26岁,标准差16.030岁。亚型包括毛平滑肌瘤62例、血管平滑肌瘤80例、生殖器平滑肌瘤10例。我们所有11例有多个皮损的患者均为毛平滑肌瘤,其中8例(73%)确诊为HLRCC。HLRCC患者比无HLRCC的毛平滑肌瘤患者年轻(34.88岁对56.17岁,p = 0.009)。血管平滑肌瘤和生殖器平滑肌瘤为单发,与HLRCC无关。

结论

对于多发性毛平滑肌瘤患者,必须排除HLRCC,因为在高比例病例中可确诊。同一患者躯干和上肢均受累的多发性毛平滑肌瘤发生富马酸水合酶突变的可能性更大。

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