Clinical Pathologic Conference Case 6: Infantile Myofibroma

Abstract

Clinical PresentationA 5-year-old female presented with facial asymmetry,which was first noted by her parents 1 month prior toconsultation. The lesion was painful and interfered withspeech and swallowing. The girl was otherwise in goodhealth with normal physical and mental development. Hermedical history was non-contributory.Extraoral examination revealed a slight facial asymme-try of the right side covered by normal-colored skin with nosigns of paresthesia. Intraoral examination revealed aswelling on the buccal aspect of the right posterior man-dible, covered by normal mucosa. Lingual displacement ofthe first molar was noted. A panoramic radiograph revealeda large well-defined multilocular radiolucent lesion mea-suring 4 9 3 cm, which involved the ramus and coronoidprocess of the right mandible, displacing dental folliclesand cortical bone, but not affecting the inferior alveolarnerve canal (Fig. 1).Differential DiagnosisConsidering the clinical history and radiographic features,the differential diagnosis included multilocular radiolucentlesions that might affect pediatric patients. The diagnosticpossibilities, therefore, would include lesions such ascentral giant cell granuloma (CGCG), central hemangioma,infantile myofibroma, aneurysmal bone cyst (ABC), andodontogenic tumors, such as ameloblastic fibroma (AF)and odontogenic myxoma (OM).CGCG is a benign lesion that occurs in the jaws ofyoung patients, usually before the age of 30 [1, 2]. About10% of the cases affect patients in the first decade of life,similar to the current case [1]. The anterior region of themaxilla is commonly affected, and mandibular lesions areequally distributed on anterior and posterior regions [1].The radiographic features of CGCG are variable, includingboth unilocular and multilocular lesions with scalloped andwell-defined borders [2]. As in the present case, CGCGmay present with aggressive clinical behavior, exhibitingrapid growth as a large multilocular radiolucent lesion,causing tooth resorption and displacement, and eventually,paresthesia, and pain [1, 2].Central hemangioma is a benign proliferation of bloodvessels that usually affects the vertebrae and skull, and isonly uncommonly seen in the jaws [2]. Hemangioma of thejaw commonly affects patients in the first three decades oflife, but only *14% of those are in the first decade [2, 3].Most central hemangiomas occur in the mandible, with23% involving the angle and ramus regions [3]. Theradiographic appearance is variable, ranging from uniloc-ular cyst-like lesions to multilocular radiolucencies, withvariable sized loculations. Occasionally, hemangiomas canpresent radiographically with spoke-like projections radi-ating from the center of the lesion [2, 3]. It is not unusual