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  • Title: [Adult myofibroma: a clinicopathological analysis of 15 cases].
    Author: Jin L, Xu S, Wang J.
    Journal: Zhonghua Bing Li Xue Za Zhi; 2021 Dec 08; 50(12):1335-1340. PubMed ID: 34865420.
    Abstract:
    Objective: To investigate the clinicopathological features and immunophenotype of adult myofibroma with emphasis on its differential diagnosis. Methods: The clinical, pathologic data and immunohistochemical profiles were analyzed in 15 cases of adult myofibroma diagnosed between 2014 and 2020 in Department of Pathology, Fudan University Shanghai Cancer Center. The literature was reviewed. Results: There were seven males and eight females, with age at presentation ranging from 22 to 74 years (mean 54 years; median 57 years). Tumor occurred principally in the extremities (n=9), less frequently involved the head and neck region (n=3) and trunk (n=2); one case was located in the vertebral canal of C6-7. Fourteen cases were solitary; one case was multifocal. Most patients presented with a slowly growing painless subcutaneous nodule, about 1 to 2 cm in size. One patient with multifocal disease and the patient with spinal lesion complained of intermittent pain. The duration of symptoms ranged from 2 months to 10 years. Microscopically, they were well circumscribed. All cases showed biphasic growth pattern, consisting of relatively well-differentiated eosinophilic nodules alternating with dark-staining primitive-appearing areas. The light-staining hypocellular nodules were composed of myofibroblast-like plump spindle cells within an eosinophilic stroma, which assumed pale blue myxochondroid appearance and hyalinization of varying degree. The dark-staining areas were composed of compact short spindled to ovoid cells with hyperchromatic nuclei and low mitotic activity, frequently showing a distinctive hemangiopericytoma-like architecture. By immunohistochemistry, the myoid spindled cells and the primitive cells stained variably for α-SMA, MSA and calponin, but were consistently negative for desmin and β-catenin. Conclusions: Adult myofibroma tends to occur in the middle to old aged patients with a predilection for the dermis or subcutis of the extremities. It is a benign neoplasm which can be cured by excision in most cases. Familiarity with its distinctive clinicopathological features helps in the distinction from other myofibroblastic neoplasms. 目的： 探讨成人肌纤维瘤的临床病理学特征、免疫表型及预后。 方法： 回顾性分析复旦大学附属肿瘤医院病理科2014—2020年诊断的15例成人肌纤维瘤的临床病理学资料，行免疫组织化学检测，并复习相关文献。 结果： 男性7例，女性8例，平均年龄和中位年龄分别为54岁和57岁（范围22~74岁）。肿瘤主要发生于四肢（9例），少数病例位于头颈部（3例）和躯干（2例），1例位于颈椎椎管内。14例为孤立性，1例为多发性。术前病程从2个月余至10年不等。发生于皮下的孤立性病变在临床上多表现为1~2 cm的无痛性结节，1例多发性病变和1例椎管内病变伴有间歇性疼痛。镜下观察肿瘤境界清楚，所有病例均显示双相性生长结构，由分化较好的淡嗜伊红色肌样结节和分化原始的深染区域组成。肌样结节中的细胞成分相对稀疏，主要由胖梭形肌样细胞组成，基质嗜伊红染，或呈淡蓝色黏液软骨样，可伴有玻璃样变；深染性区域由相对密集、核深染的短梭形细胞至卵圆形细胞组成，常显示血管外皮瘤样排列结构，核分裂活性低。免疫组织化学标记显示，两种成分程度不等表达α-平滑肌肌动蛋白、肌特异性抗原和钙调蛋白，不表达结蛋白和β-连环蛋白。 结论： 成人肌纤维瘤好发于中老年人躯体真皮和皮下，特别是下肢，生物学行为上呈良性，切除后可治愈。.
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