Case of the Week

•Background:

• Sporadic, rare, benign congenital tumor arising from the gingival mucosa; also known as congenital granular cell lesion, congenital granular cell myoblastoma, and granular cell fibroblastoma
• Typically presents around the canine/incisor region of the maxillary alveolar ridge
• Most often presents with a single lesion; 10% of cases have multiple lesions
• Female predilection with a 10:1 ratio

•Clinical Presentation:

• Soft, painless, red, non-friable pedunculated or sessile mass arising from the gingival mucosa
• Seen at birth or prenatally via ultrasound
• Can interfere with breathing and feeding

•Key Diagnostic Features:

• Well-defined homogeneous mass with major portions showing isointense signal to muscle on T1WI and T2WI
• Occurs almost exclusively in fetuses/neonates

•Differential Diagnosis:

• Teratoma of the palate (Epignathus): Heterogeneous solid and cystic mass containing calcifications and infiltrating surrounding structures
• Hemangioma: Benign vascular tumor of blood vessels. Can present similarly to congenital epulis but is more prone to bleeding.
• Lymphatic malformation and dermoid cyst: Presents as cystic lesions with T2 hyperintensity
• Infantile myofibroma: Benign head and neck mesenchymal tumor affecting the tongue and buccal mucosa; the maxilla is rarely affected.
• Melanotic neuroectodermal tumor of infancy (MNTI): Rare locally aggressive neoplasm with a site predilection for anterior maxilla. Usually occurs within the first year of life and can present at or around birth.
• Rhabdomyoma: Benign tumor of striated muscle. Tumors outside of the heart are rare but most commonly occur in the head and neck. Differentiated histologically.

•Treatment:

• Immediate non-radical excision; no malignant transformation or recurrence have been reported after excision
• Lack of treatment could lead to asphyxiation, feeding difficulty, cyanosis, or dyspnea
• Observation can also be recommended for small tumors that do not interfere with feeding or breathing. Spontaneous regression has been reported.