Dal Vechio Aluana, Nakajima Edgar, Pinto Décio, Azevedo Luciane Hiramatsu, Migliari Dante A
Department of Stomatology, School of Dentistry, University of São Paulo, 05508-900 São Paulo, SP, Brazil.
Case Rep Dent. 2013;2013:943953. doi: 10.1155/2013/943953. Epub 2013 May 28.
Rhabdomyomatous hamartoma is a rare disease that occurs predominantly in the skin. This paper describes a congenital lesion in a 17-year-old male, who came to our clinic presenting a circumscribed swelling involving the oral mucosa and vermillion border of the upper lip, purplish in color, and blanching under pressure. The patient reported that he had had lesion since his birth. A clinical diagnosis was of congenital haemangioma, and the patient was treated by photocoagulation using diode laser. When the lesion became smaller, by having its blood content reduced, the upper portion of the lesion was sliced off with CO2 laser and the tissue was sent for microscopic analysis. Histopathological examination showed an oral mucosa fragment with proliferation of striated muscle bundles admixed with small blood vessels, collagen, and nerve fibres. A supplementary analysis with immunohistochemistry demonstrated positivity for desmin, HHF35, smooth muscle actin, S-100, and CD34. Based on these findings, the lesion was diagnosed as rhabdomyomatous hamartoma. The aesthetic result has been very satisfactory after a 14-month followup.
横纹肌瘤样错构瘤是一种主要发生于皮肤的罕见疾病。本文描述了一名17岁男性的先天性病变,该患者前来我院就诊,表现为累及口腔黏膜及上唇唇红缘的局限性肿胀,呈紫色,压迫后褪色。患者自述自出生以来就有该病变。临床诊断为先天性血管瘤,患者接受了二极管激光光凝治疗。当病变因血液含量减少而变小时,用二氧化碳激光切除病变上部,并将组织送去做显微镜分析。组织病理学检查显示口腔黏膜碎片中有横纹肌束增生,并伴有小血管、胶原纤维和神经纤维。免疫组织化学补充分析显示结蛋白、HHF35、平滑肌肌动蛋白、S-100和CD34呈阳性。基于这些发现,该病变被诊断为横纹肌瘤样错构瘤。经过14个月的随访,美学效果非常令人满意。