Microcystic Adnexal Carcinoma at Mental Area--A Rare Case Report and Literature Review

下頦微囊腫性附屬器癌―案例報告與文獻回顧

阮智楷(Chih-Kai Juan) ； 黃傑慧(Chieh-Huei Huang) ； 李維棠(Wei-Tang Li) ； 戴浩志(Hao-Chih Tai)

《臺灣整形外科醫學會雜誌》 30卷4期 (2021/12) Pp. 250-256

摘要

Background: Microcystic adnexal carcinoma (MAC) is a rare malignant cutaneous neoplasm, which was first described by Goldstein et al. in 1982. It is a slow-growing and locally aggressive tumor, but rarely found nodal or distant metastasis. The incidence of MAC for Asian people was significantly lower than the westerns, with about 1.9 per 10 million individuals. Aim and Objectives: Owing to its rarity, we are unfamiliar with this diagnosis, risk factors, treatment, and further follow-up timeline. Here we presented a case and reviewed the current literatures about the way to approach MAC, including epidemiology, clinical manifestation, treatment, and follow-up. Material and Methods: A 48-year-old lady, who was healthy before, noticed a 1.5×1.5 plaque on her left chin in 2019. Due to the plaque is getting larger, she decided to receive incisional biopsy in mid-June, 2020. The pathological examination revealed microcystic adnexal carcinoma (MAC). Head and neck MRI revealed no obvious cervical lymphadenopathy. Tumor wide excision and free anterolateral thigh (ALT) flap reconstruction were performed smoothly in late-July, 2020. Results: In pathological exam, the tumor was characterized with infiltrative sheets of squamoid cells containing keratinous cyst and small basaloid nests. All section margins are uninvolved by tumor (＞5mm). Good oral competence, functional outcome, and aesthetic result were also achieved afterwards. No local recurrence or systemic involvement was observed up to now. Conclusion: The assessment of skin biopsy finding is sufficient for the diagnosis of MAC. As for treatment, surgical excision is the first-line choice. Adjuvant radiotherapy may improve local control of MAC in patients with margin positive or perineural invasion. However, prophylactic radiotherapy is not supported. Follow-up every 6-12 months for first 5 years is recommended.

關鍵字

microcystic adnexal carcinoma ； mental defect

並列摘要

背景：微囊腫性附屬器癌是種極罕見惡性皮膚腫瘤，首位案例報告於1982年由Goldstein等人提出。它是種局部侵犯較深，但生長緩慢且罕見淋巴或遠端轉移的腫瘤。亞洲人的疾病發生率遠較西方人低，約每千萬人中有1.9位罹病。目的及目標：微囊腫性附屬器癌是極為罕見的疾病，因此我們對於它的診斷、危險因子、治療及後續追蹤時程皆不熟悉。在此，我們提出一例個案報告，並針對微囊腫性附屬器癌的流行病學、臨床表現、治療及後續追蹤時程做最新文獻回顧。材料及方法：一位48歲女性沒有任何過去病史，她在2019年時發現下巴有一塊約1.5 × 1.5公分斑塊。因為斑塊逐漸變大，她於2020年6月中旬接受切片手術，病理報告確診為微囊腫性附屬器癌，後續安排頭頸部核磁共振檢查沒有明顯淋巴結腫大情形。病人於2020年7月下旬順利接受腫瘤廣泛性切除及大腿前外側自由皮瓣重建手術。結果：病理檢查下，腫瘤呈現浸潤性片狀之鱗狀細胞，內含角質囊腫及少量基底類巢狀特徵。手術完整把腫瘤切除，其距離腫瘤邊緣皆超過5 mm。術後口腔閉合、口部功能及外觀皆有良好結果。至目前為止，沒有局部復發或遠端轉移情形。結論：在診斷上，皮膚切片檢查就足以診斷微囊腫性附屬器癌。在治療上，手術完全切除為治療的第一選擇。針對手術切緣仍有殘餘腫瘤或腫瘤侵犯神經周圍的病人，輔助性放射治療能局部控制腫瘤；然而，目前並不建議預防性放射治療。術後前五年建議每半年至一年回診追縱一次。