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指状乳头状腺癌:一例罕见的小汗腺恶性皮肤肿瘤病例

Digital Papillary Adenocarcinoma: Case of a Rare Malignant Cutaneous Tumor of the Eccrine Sweat Gland.

作者信息

Jabbour Austin J, Tangoren Ibrahim A, Kanik Andrew B

机构信息

The University of Queensland-Ochsner Clinical School, New Orleans, Louisiana, USA.

Private Practice, I.A. Tangoren, PLLC, Dermatology and Dermatologic Surgery, Syracuse, New York, USA.

出版信息

Case Rep Dermatol. 2021 Aug 6;13(2):422-427. doi: 10.1159/000518442. eCollection 2021 May-Aug.

Abstract

Digital papillary adenocarcinoma (DPA) is a rare cutaneous tumor originating from the eccrine sweat glands. These lesions occur almost exclusively on the digits of the hands and feet, where there is a high concentration of eccrine glands. The diagnosis is made histologically, and the course of the malignancy tends to be very aggressive with high rates of recurrence and early metastasis at the time of diagnosis. Due to the low incidence of these lesions, there have been minimal objective data from clinical studies to recommend specific treatment strategies. Wide local excision versus digital amputation proximal to the lesion has been debated for primary treatment, while there are no data to support routine implementation of adjuvant chemotherapy or radiation, despite its metastatic nature. This article presents a case of long-standing, previously undiagnosed DPA. The lesion appeared more inconspicuous on gross examination than other reports in the literature, and diagnosis was made with punch biopsy and confirmed postsurgically. To date, the patient has not had recurrence, although she is being monitored for potential metastatic deposits in her lungs. Clinical dermatologists should be aware of the high mortality burden this lesion may inflict if left undiagnosed or mistreated.

摘要

指状乳头状腺癌(DPA)是一种罕见的起源于小汗腺的皮肤肿瘤。这些病变几乎仅发生在手和脚的指(趾)上,此处小汗腺高度集中。诊断依靠组织学检查,该恶性肿瘤的病程往往极具侵袭性,在诊断时复发率高且早期转移率高。由于这些病变的发病率低,临床研究中几乎没有客观数据来推荐具体的治疗策略。对于主要治疗方法,广泛局部切除与在病变近端进行指(趾)截肢一直存在争议,尽管其具有转移性,但没有数据支持常规实施辅助化疗或放疗。本文介绍了一例长期未被诊断出的DPA病例。该病变在大体检查时比文献中的其他报道显得更不明显,通过打孔活检进行诊断,并在手术后得到证实。迄今为止,该患者尚未复发,尽管她正在接受肺部潜在转移灶的监测。临床皮肤科医生应意识到,如果该病变未被诊断或治疗不当,可能会带来很高的死亡负担。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8f0a/8436639/a592427a869f/cde-0013-0422-g01.jpg

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