Abstract
Congenital epulis is an exceedingly uncommon neoplasm that manifests in the oral cavity of neonates. The advancement in prenatal diagnostic techniques such as ultrasound and magnetic resonance imaging (MRI) allows for detection prior to birth. The most frequent site of manifestation is the anterior alveolar ridge of the maxilla, succeeded by the alveolar ridge of the mandible, and in rare cases, the tongue. There exist several theoretical proposals studying its histogenesis and potential causes, but they remain elusive. The case under discussion is a four-day-old female patient exhibiting a multilobulated, firm, pedunculated, mucosal-colored mass originating from the right anterior maxillary ridge and extending out from the mouth. The lesion posed an obstacle to normal feeding, although it fortunately did not impede the airway or respiration. The lesion was excised under general anesthesia and the specimen was forwarded to the histopathology laboratory where the diagnosis was confirmed. Seven weeks post-operation, the patient returned to our clinic exhibiting a fully healed wound. The prognosis for such a lesion is highly favorable, with no instances of recurrence documented in the literature. A multidisciplinary approach is required for the effective management of congenital epulis.
Highlights
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Congenital epulis is a rare condition that requires multidisciplinary care.
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Congenital epulis affects females more than males, with a ratio 9:1.
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The mainstay treatment for congenital epulis is surgical excision, with no recurrence reported in the literature.
1
Introduction
Tumors that are found in the oral cavity at the time of birth are rare [ ]. However, serious consequences including asphyxia as a result of airway obstruction may result from these tumors if not managed appropriately [ , ]. Congenital epulis (CE) is a rare tumor that was first reported by Neumann and hence named Neumann tumor [ ]. Other names for this lesion are congenital gingival granular cell tumor (CGCT) of the newborn, congenital granular cell lesion, and congenital myoblastoma. The estimated incidence of this lesion is 0.0006 % and is reported tenfold in females with the most common site being the anterior alveolar ridge [ , ]. The lesion can vary in size from a few millimeters to several centimeters and can be sessile or pedunculated [ ]. The treatment of choice for such cases is surgical excision [ ]. However, conservative treatment may be reserved for small lesions that do not obstruct the airway or are associated with difficulty in feeding.
2
Case report
The patient is a neonate of four days, who was birthed through normal spontaneous vaginal delivery following a full-term pregnancy, and had a birth weight of 3.4 kg. The infant was transferred to King Saud Medical City from a different facility after her primary obstetrician observed a mass emerging from the oral cavity shortly post birth. The father enjoys a sound medical status whereas the mother suffers from iron-deficiency anemia, although she attended regular prenatal check-ups with no adverse events reported throughout the term. The patient has three healthy siblings. Upon observation in the ward, the patient was breathing room air without any signs of dyspnea or cyanosis, was being fed through a nasogastric tube due to the lesion hindering normal feeding, and overall, the patient’s condition was stable. Physical examination revealed a normally colored, firm, pedunculated, multilobulated mass that protruded from the right anterior maxillary alveolar ridge. An MRI was ordered for more detailed analysis. The preliminary diagnosis pointed to CE, and the decision was made to schedule the patient for surgery to remove the lesion. The Anesthesia team was consulted and they recommended to alert the Ear, Nose, and Throat (ENT) team for a potential emergency tracheostomy in case of difficult intubation. The patient was subsequently taken to the operating room and intubation was smoothly achieved using an orotracheal tube. A pre operative photograph was captured ( Fig. 1 ). The lesion was meticulously excised from its base utilizing electrocautery and subsequently submitted to the oral histopathology laboratory. The dimensions of the lesion were 3.5 × 2.4 cm, while the base of the lesion measured 1.1 × 1.2 cm at its greatest dimensions ( Fig. 2 ). Hemostasis was achieved at the surgical site and it was packed using sutured oxidized cellulose ( Fig. 3 ). The patient was routinely monitored postoperatively in the hospital for five days and was then discharged after verification of efficient oral feeding. The histological features of the excised specimen are characterized by the presence of well-circumscribed nodular soft tissue masses with attenuated overlying epithelium ( Fig. 4 ). The tumor comprises solid sheets of large polygonal to ovoid cells with abundant granular cytoplasm and distinct cell membranes. The nuclei are small, dark, oval, and eccentric with vesicular chromatin and small nucleoli ( Fig. 5 ). The tumor shows arborizing stromal vascular channels that are large and ectatic, some with a staghorn appearance. Interestingly, nests and cords of odontogenic epithelium that exhibit palisading and clearing were seen within the tumor ( Fig. 6 ). The patient was reviewed one week post-excision, with the subsequent follow-up appointment scheduled for the 7th week post-operation. The surgical site was fully healed, with no indications of other co-morbidities ( Fig. 7 ).
