Rhabdomyomatous Mesenchymal Hamartoma of the Nasal Columella-A Case Report and Literature Review

鼻小柱之橫紋肌間葉型錯構瘤－案例分享及文獻回顧

賴宇荷(Yeu-Her Lai) ； 巫政霖(Cheng-Lin Wu) ； 謝式洲(Shyh-Jou Shieh)

《臺灣整形外科醫學會雜誌》 31卷3期 (2022/09) Pp. 205-212

摘要

Background: Rhabdomyomatous mesenchymal hamartoma (RMH) is a rare, benign dermal and subcutaneous lesion that composes of mesenchymal elements, including mature striated muscles, adipose tissue, collagen, blood vessels, peripheral nerves and adnexal structures. It most commonly occurs in newborns and infants with a predilection for regions of the head and neck. Though RMH is a benign lesion, it may still be associated with other congenital anomalies; thus, a full systemic evaluation is warranted. Over 70 cases of RMH have been reported in English literature. Aim and Objectives: The aim of this study is to share a rare case of RMH of the nasal columella and review previous reported cases of such a lesion in the midface. Materials and Methods: We present the case of a 1-year-5-month-old Asian girl with an enlarging, beige colored nodule located at the nasal columella. Surgical excision was done and the specimen was sent for pathological analysis, which revealed to be rhabdomyomatous mesenchymal hamartoma. Furthermore, we present a literature review of cases of RMH of the midface. Results: Histopathological examination found bundles of mature striated muscle admixed with a large portion of adipose tissue as well as fibrous tissue. One year follow-up showed a complete healed wound with no evidence of recurrence. Conclusion: The clinical patterns, morphological details, differential diagnosis and management are delineated in this case report. Furthermore, we provide a comprehensive literature review on the previous reported cases of RMH in the midface region.

關鍵字

rhabdomyomatous mesenchymal hamartoma

並列摘要

背景：橫紋肌間葉錯構瘤為一種罕見、良性的皮下腫瘤，其由間質幹細胞（例如：橫紋肌、脂肪細胞、膠原纖維、血管、神經及其他皮膚附屬結構）所組成。通常在新生兒較常見。好發部位為頭頸部。雖然是良性腫瘤，但有可能會合併其他先天性疾病，因此需要進行系統性的全身評估。目前英文文獻中有70例橫紋肌間葉錯構瘤。目的及目標：本篇案例報導一個罕見的鼻小柱延伸至鼻腔內橫紋肌間葉型錯構瘤，並整理該腫瘤位於中顏面的文獻回顧。材料及方法：一位一歲五個月大的女童於右側鼻小柱上發現一顆膚色、緩慢生長的結節。手術切除後經病理診斷為橫紋肌間葉型錯構瘤。我們藉由此案例再做其病灶位於中顏面的文獻回顧。結果：組織病理學發現橫紋肌束參雜脂肪細胞及纖維組織。術後一年傷口復原良好，並未有復發的情形。結論：此篇報導提供橫紋肌間葉型錯構瘤臨床上的特色、病理學的特徵、鑑別診斷及其病灶在中顏面的文獻回顧。