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睑部多形性皮肤肉瘤采用显微外科手术及二期愈合治疗的病例

Case of Pleomorphic Dermal Sarcoma of the Eyelid Treated with Micrographic Surgery and Secondary Intention Healing.

作者信息

Kim Jung-In, Choi Young-Jun, Seo Hyun-Min, Kim Han-Saem, Lim Jae Yun, Kim Dong-Hoon, Chae Seoung Wan, Lee Ga-Young, Kim Won-Serk

机构信息

Department of Dermatology, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea.

Department of Dermatology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.

出版信息

Ann Dermatol. 2016 Oct;28(5):632-636. doi: 10.5021/ad.2016.28.5.632. Epub 2016 Sep 30.

Abstract

Pleomorphic dermal sarcoma (PDS) is a rare mesenchymal neoplasm sharing histopathological features with atypical fibroxanthoma (AFX), but has additional features of deep invasion of the superficial subcutis, tumor necrosis and vascular/perineural invasion. It is not well documented in the literature because of its rarity, and its clinical course has been debated due to the lack of homogenous criteria. We describe here the case of a 91-year-old female with a 6-month history of a solitary, asymptomatic, well-defined, 3.4-cm-sized, reddish, hard, protruding mass on the lateral aspect of the right upper eyelid. On the basis of initial punch biopsy results, storiform cellular infiltrate of pleomorphic spindle and polygonal cells with frequent atypical mitoses, the lesion was identified as AFX. Following the initial biopsy, micrographic surgery was performed and a tumor-free margin was confirmed. Considering the conservation of the periocular function and the advanced age of the patient, we planned secondary intention healing rather than primary suturing. After surgery, skeletal muscle infiltration was found and the diagnosis was revised to PDS by a pathologist based on the currently accepted criteria for PDS. There has been no evidence of recurrence or periocular functional defects during a 2-year follow-up without adjuvant therapy. Although the PDS is highly malignant, complete excision under micrographic surgery can prevent recurrence without adjuvant therapy. Also, the secondary intention healing is an effective method for closure of large defects on the face.

摘要

多形性皮肤肉瘤(PDS)是一种罕见的间叶性肿瘤,与非典型纤维黄色瘤(AFX)具有共同的组织病理学特征,但还具有侵犯浅表皮下组织深层、肿瘤坏死以及血管/神经周围侵犯等额外特征。由于其罕见性,文献中对此记载不多,且由于缺乏统一标准,其临床病程一直存在争议。我们在此描述一例91岁女性患者,其右上眼睑外侧有一个6个月病史的孤立、无症状、边界清晰、大小为3.4厘米的红色、坚硬、突出肿物。根据最初的穿刺活检结果,可见多形性梭形和多边形细胞呈束状细胞浸润,并有频繁的非典型有丝分裂,该病变被诊断为AFX。初次活检后,进行了显微外科手术并确认切缘无肿瘤残留。考虑到眼周功能的保留以及患者的高龄,我们计划采用二期愈合而非一期缝合。术后发现有骨骼肌浸润,病理学家根据目前公认的PDS标准将诊断修订为PDS。在未进行辅助治疗的2年随访期间,没有复发或眼周功能缺陷的证据。尽管PDS具有高度恶性,但在显微外科手术下完整切除可在不进行辅助治疗的情况下预防复发。此外,二期愈合是闭合面部大缺损的有效方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6262/5064195/e431fc68661c/ad-28-632-g001.jpg

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