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微囊性附属器癌:13例报告及文献复习

Microcystic adnexal carcinoma: report of 13 cases and review of the literature.

作者信息

Snow S, Madjar D D, Hardy S, Bentz M, Lucarelli M J, Bechard R, Aughenbaugh W, McFadden T, Sharata H, Dudley C, Landeck A

机构信息

Department of Surgery, University of Wisconsin-Madison, School of Medicine, 2880 University Ave., Madison, WI 53705, USA.

出版信息

Dermatol Surg. 2001 Apr;27(4):401-8. doi: 10.1046/j.1524-4725.2001.00208.x.

DOI:10.1046/j.1524-4725.2001.00208.x
PMID:11298716
Abstract

BACKGROUND

Microcystic adnexal carcinoma (MAC) is a rare tumor of the skin. Clinically it often masquerades as a firm, subcutaneous nodule on the head and neck regions. Microscopically it extends far beyond assessed clinical margins spreading locally in the dermal, subcutaneous, and perineural tissue planes. The local recurrence rate by standard excision is about 50%. Recent preliminary reports indicate more favorable cure rates with Mohs micrographic surgery (MMS).

OBJECTIVE

To present our data on 13 cases (12 patients) of MAC treated by MMS. In addition, we reviewed the medical literature to summarize the accumulated experience of MMS treatment in the management of MAC. We also present a case of bilateral MAC of the face and describe a renal transplant recipient on immunosuppressive therapy who developed MAC of the nasal bridge.

METHODS

We reviewed and updated our series of MAC cases treated by MMS over the last 9 years. A total of 13 cases of MAC are reviewed. We also searched the literature for MAC treated by MMS with a follow-up of more than 2-years.

RESULTS

One patient had bilateral MAC of the nose and cheek. Another patient developed a MAC of the nasal bridge 20 years after renal transplantation. In this patient predisposing factors were radiation for teenage acne and immunosuppression therapy. A total of 13 cases of MAC were treated by MMS with no recurrences, with a mean follow-up of 5.0 years (range 1.1-8.0 years).

CONCLUSION

We update the medical literature with 13 MAC cases treated by MMS. To our knowledge there have been 148 cases of MAC reported in the world literature. Including our series, there have been 73 cases of MAC treated with MMS. There were only four treatment failures. Regional and/or distant metastasis from MAC is rare, with only one reported death. Following MMS, the 2-year success rate was 89.7% (35 of 39). The accumulated data continue to confirm that when MAC is discovered early and is readily accessible to excision by MMS and other subspecialty support, a favorable outcome can be expected.

摘要

背景

微囊性附属器癌(MAC)是一种罕见的皮肤肿瘤。临床上,它常表现为头颈部区域的一个质地坚硬的皮下结节。在显微镜下,它的范围远远超出评估的临床边界,在真皮、皮下和神经周围组织平面呈局部扩散。标准切除后的局部复发率约为50%。最近的初步报告表明,莫氏显微外科手术(MMS)的治愈率更高。

目的

介绍我们采用MMS治疗的13例(12名患者)MAC的数据。此外,我们回顾了医学文献,以总结MMS治疗MAC的累积经验。我们还报告了一例面部双侧MAC病例,并描述了一名接受免疫抑制治疗的肾移植受者发生鼻梁MAC的情况。

方法

我们回顾并更新了过去9年中我们采用MMS治疗的MAC病例系列。共回顾了13例MAC病例。我们还在文献中搜索了采用MMS治疗且随访超过2年的MAC病例。

结果

一名患者鼻子和脸颊出现双侧MAC。另一名患者在肾移植20年后发生鼻梁MAC。该患者的诱发因素为青少年痤疮放射治疗和免疫抑制治疗。总共13例MAC采用MMS治疗,无复发,平均随访5.0年(范围1.1 - 8.0年)。

结论

我们用13例采用MMS治疗的MAC病例更新了医学文献。据我们所知,世界文献中已报道148例MAC病例。包括我们的系列病例,共有73例MAC采用MMS治疗。仅有4例治疗失败。MAC的区域和/或远处转移罕见,仅报告1例死亡。MMS治疗后,2年成功率为89.7%(39例中的35例)。累积数据继续证实,当MAC早期发现且易于通过MMS及其他专科支持进行切除时,有望获得良好的治疗效果。

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