We report the case of a 3-day-old male neonate. The child was born at term and was postnatally noted to have a 2-cm mass over the maxillary alveolar ridge in the midline [Fig. 1]. The mass was not picked up on the antenatal scan. The mass was causing feeding difficulty to the child following which medical attention was sought. On examination, the mass arose from the maxillary gingiva and was pink, irregular, lobulated, and rubbery. The rest of the oral cavity was normal. Excision of the tumor was planned in view of the clinical condition and symptoms. Following endotracheal intubation, the base of the lesion was infiltrated with 0.5% lidocaine and 1:100,000 epinephrine [Fig. 2]. The alveolar mucosa around the base of the lesion was elevated, and the lesion was completely excised. The alveolar mucosa was apposed with interrupted polyglactin sutures. The child was commenced breastfeeding 12 h after surgery. This was gradually built up to full feeds over the course of the next 72 h, and the child was discharged home. Follow-up visits were scheduled at 1 week and 4 weeks. On follow-up, the child was thriving well, and the alveolar mucosa had also healed up nicely. Histopathology showed squamous mucosa with large polygonal cells, round nuclei, and abundant granular cytoplasm. No evidence of malignancy was noted. The overall picture was suggestive of congenital granular cell tumor (CGCT). Congenital granular cell tumor (CGCT) is a rare lesion and frequently seen on the alveolar ridges of the maxilla or mandible. It is predominantly a solitary lesion and more prevalent in females and Caucasians [5,6,7]. It can be picked up during prenatal imaging as early as 26 weeks. It presents as an irregular, lobulated, rubbery soft tissue mass of varying sizes. A large size may be the reason for polyhydraminios affecting fetal deglutination. Postnatally, it may cause difficulty in feeding [1]. The mass is believed to originate from the undifferentiated mesenchymal cells, pericytes, fibroblasts, odontogenic epithelial, and smooth muscle cells. The diagnosis is usually clinical and confirmed by histopathology. Congenital granular cell tumor needs to be differentiated from adult granular cell tumor. The adult variant occurs in 30–60 years. It is seen in the tongue and other sites. The overlying stratified squamous epithelium shows pseudoepitheliomatous hyperplasia [1]. The adult granular cell tumor shows positivity for S100, laminin, nerve growth factor receptor, and smooth muscle actin. CGCT shows positivity for only vimentin and neuron-specific enolase. Other differentials for congenital masses in the oral cavity can be dermoid cysts, vascular malformations, encephaloceles, and malignancies like teratoma and rhabdomyosarcomas. Melanotic pigmented neuroectodermal tumor is an osteolytic pigmented infiltrating neoplasm primarily affecting the jaws of the newborns.
Management of a child with CGCT remains debatable. The tumor stops to grow at birth and spontaneous regression can occur. These lesions can be monitored to see for regression. However, surgery is indicated when the mass causes impairment in feeding as was seen in the present case or respiratory compromise. For symptomatic patients, simple excision is preferred as radical excision can damage the developing tooth buds. Malignant change or recurrence has not been reported following incomplete excision of this mass [1].
