May 2025 NeoQuest: Oral Mass in a Neonate Free

A pregnant woman at 36 weeks’ gestation has a fetal ultrasonogram for growth that demonstrates a new large mass measuring 4 x 2.7 cm protruding from the mouth (Figure 1A). The amniotic fluid level is normal, and no other fetal malformations are observed. A repeat prenatal ultrasonogram at 38 weeks’ gestation shows a stable-sized, homogeneous mass with discrete vascularity. At birth, the female newborn’s exam demonstrates a large pedunculated oral mass protruding from the mouth, lined with oral mucosa without any ulceration (Figure 1B). The mass was resected on the first day after birth. Histopathology revealed a well-circumscribed, polypoid mass composed of polygonal cells with eosinophilic granular cytoplasm.

Figure 1: A. Prenatal ultrasonogram (sagittal) of fetal face showing a homogeneous soft tissue mass protruding from the oral cavity (yellow arrow). B. Postnatal presentation of the neonate with an oral mass protruding from the oral cavity. Image from Chitkara R, Balakrishnan, K, Jayapal P, Judy A, Tan.S, Hintz S. Prenatal diagnosis of a large congenital epulis. Neoreviews. 26(5)2025;e357-e361.¹

What is the most common origin for this type of mass?

A. Anterior tongue
B. Floor of the mouth
C. Foramen cecum of tongue
D. Maxillary or mandibular alveolar ridge
E. Posterior tongue

Answer: D. Maxillary or mandibular alveolar ridge

Explanation:

The infant in this vignette had a late fetal presentation of an oral mass, which remained stable in size and had radiographic and histopathological features consistent with a congenital epulis (CE).^1,2 CE, also known as congenital granular cell epulis or Neumann’s tumor,^1,2 is a rare benign tumor with an estimated incidence of 0.0006% per live births.^1-4 The etiology of this tumor is unknown.¹ CE has a female predominance with an estimated female-to-male ratio of 8-10:1.² The higher incidence in females has led to speculation of hormonal influences on CE (estrogen and/or progesterone), but no definitive association has been established.²

CE develops in-utero during the late third trimester, with the earliest diagnosis reported at 26 weeks’ gestation.^2,5 Prenatal diagnosis is uncommon, likely due to the tumor’s late third-trimester development. However, fetal ultrasonogram and fetal or postnatal magnetic resonance imaging can aid in diagnosis and management.¹ Postnatally, CE typically presents as a solitary, smooth, multilobed, non-tender, solid mass covered by normal-appearing pink oral mucosa. It could be sessile but is most commonly pedunculated with vascularization in the stalk. ^1,4,5 Postnatal imaging is not always necessary if the presentation is classic. The most common location of origin is the gingival mucosa of the maxillary and mandibular alveolar ridge (Option D), with a three times higher likelihood of it developing in the maxilla compared to the mandible.^1-3 The size can vary from a few millimeters to up to 10 cm, and 10% of cases have multiple epulides.^1,4

CE has distinctive microscopic findings: large round cells, granular eosinophilic cytoplasm, and small basophilic eccentric non-mitotic nuclei.¹ (Figure 2) These cells are surrounded by collagen and covered with squamous epithelium.^1,4,5 Granular cell tumors have similar microscopic findings, but they are malignant, found in all age groups, and stain positive for S-100 protein.⁴

Figure 2: Image demonstrating uniform-appearing polygonal cells with distinct cell membranes, eccentrically placed nuclei, and abundant cytoplasm. Image from Chitkara R, Balakrishnan, K, Jayapal P, Judy A, Tan.S, Hintz S. Prenatal diagnosis of a large congenital epulis. Neoreviews. 2025;26(5):e357-e361.¹

Fetuses with CE can have oral and airway obstruction leading to polyhydramnios. Postnatally, affected patients can have respiratory distress and feeding issues. If the CE is large, a multidisciplinary team should be present at delivery to manage potential life-threatening airway obstruction, ensuring prompt resuscitation and stabilization before definitive treatment. A structured approach to diagnosing an oral mass in a newborn based on consistency, size, location of origin, and vascularization pattern helps narrow the broad differential (Figure 3).^6,7 The treatment of choice is resection, especially of large CEs, and diagnosis can be confirmed on pathology.^1,2 Recovery is typically short, with an extremely low risk of malignant transformation or recurrence.^1,2

Figure 3. Schematic showing the differential diagnosis of an oral mass in a neonate.

The anterior tongue is a common location for oral foregut duplication cysts and dermoid cysts (Option A).^6,7 Duplication cysts (Figure 4) can develop anywhere along the gastrointestinal tract, including the tongue (0.3% of duplication cysts).⁸ Dermoid cysts are found in embryonic fusion lines and can contain epidermal appendages such as connective tissue, hair follicles, and sweat glands.⁸ Both are cystic in consistency, can increase in size, and are not lined with oral mucosa, which differentiates them from CE.⁸

Figure 4: A. Postnatal presentation of newborn with an oral duplication cyst in the anterior tongue. B and C. Magnetic resonance imaging of a newborn with a lingual foregut duplication cyst (green arrows). Image from Akula V. A newborn has swelling under the tongue. Neoreviews. 2007;8(8):e357–e359.⁸

Mucoceles commonly occur in the oral cavity due to the collection of saliva outside of the salivary glands. They are common in the cheeks, underside of the tongue, or floor of the mouth (Option B).⁴ Ranulae are mucoceles of the sublingual and submandibular glands and are present on the floor of the mouth.⁹ The classic neonatal presentation of a mucocele is a fluctuant cystic mass with bluish discoloration, in contrast to a CE, which is solid, stable in size, and lined with pink mucosa.^7,9

A lingual thyroid is an important diagnosis to consider in a newborn with an oral mass. A lingual thyroid is typically located in the foramen cecum (Option C) but can also be found anywhere along the thyroglossal duct.^3,9,10 It results from the failure of thyroid descent from the foramen cecum to the neck. Affected neonates have a midline mass at the base of the tongue in contrast to infants with a CE that involves the alveolar ridge.^3,7,9,10 Additionally, the histology of an ectopic thyroid shows normal thyroid tissue.¹⁰ Lingual thyroid is the most common type of ectopic thyroid tissue, 33% of which are associated with hypothyroidism.¹⁰ It may be the only functioning thyroid tissue in 75% of the affected patients.¹⁰ Therefore, the initial management consists of a thyroid ultrasonogram and thyroid function tests to determine the need for thyroid replacement therapy. Complete resection should be avoided if this ectopic thyroid is the only functional thyroid tissue present.^7,9,10

The posterior tongue (Option E) is a common location for hamartomas. Hamartomas are masses composed of disorganized but native tissue.^6,11 The etiology of hamartomas (Figure 5) is unknown. These encapsulated lesions are typically present at birth, are non-malignant, and undergo regression after adolescence.⁶ Imaging, such as a neck ultrasonogram to rule out lingual thyroid, magnetic resonance imaging, or a computed tomography scan to assess mass extent, can be obtained. Management in most cases is surgical resection, and recurrence is rare. ^6,11,12

Figure 5: A: Postnatal presentation of a neonate with an oral hamartoma. B: Magnetic resonance imaging of the head and neck of a neonate with a hamartoma (white arrow) in the tonsillar fossa, causing oropharyngeal obstruction. Image from Harpale P, Consul M, Kumar A, Sinha A. A rare case of an oropharyngeal mass causing respiratory distress. NeoReviews. 2025;26(1):e53-56.⁶

Did you know?  

• CE is typically similar in color to the oral mucosa. However, if pigmented, a melanotic neuroectodermal tumor of infancy needs to be considered.
• Melanocytic neuroectodermal tumors are rare, benign, but locally aggressive tumors, with a predilection for the maxillary bone. Affected infants present with a bluish-black, lobulated, sessile, and firm oral mass in the alveolar process of the maxilla.¹³

Which neonatal oral lesions can lead to airway obstruction?
To read more about how to differentiate various lesions at various oropharyngeal locations and treatment modalities, please see Chinnadurai S, Goudy SL. Neonatal airway obstruction: an overview of diagnosis and treatment. Neoreviews. 2013;14(3):e128-137.⁹

NeoQuest May 2025 Authors:
Ambika Bhatnagar, MD, Division of Neonatal-Perinatal Medicine, Yale School of Medicine, New Haven, CT
Srirupa Hari Gopal, MBBS, FAAP, Division of Neonatal-Perinatal Medicine, SSM Health Cardinal Glennon Pediatrics/St Louis University, St. Louis, MO

References:

1. Chitkara R, Balakrishnan K, Jayapal P, Judy A, Tan.S, Hintz S. Prenatal diagnosis of a large congenital epulis. Neoreviews. 2025;26(5):e357-e361
2. Anisowicz SK, Tolisano AM, Golden JB. Newborn with a large oral mass. Pediatr Rev. 2017;38(3):e10-e12
3. Rai VK, Kumar A. Oral cavity masses in a newborn. Arch Dis Child Fetal Neonatal Ed. 2022;107(4):451
4. Koltai P, Lan WJ, Chan D, Hoff S. Masses in the oral cavity. Neoreviews. 2011;12(9):e545–e549
5. Suzuki M, Marrow-Corwin M. A female infant who has a pink, smooth soft-tissue mass. Neoreviews. 2012;13(2):e132–e135
6. Harpale P, Consul M, Kumar A, Sinha A. A rare case of an oropharyngeal mass causing respiratory distress. Neoreviews. 2025;26(1):e53-56
7. Pahuja A, James H, D’Costa W, Al-Jaddir G, Kaur D. Congenital epulis and differential diagnoses of a neonatal oral mass. Infant. 2021;17(3): 118-22
8. Akula V. A newborn has swelling under the tongue. Neoreviews. 2007;8(8):e357–e359
9. Chinnadurai S, Goudy SL. Neonatal airway obstruction: an overview of diagnosis and treatment. Neoreviews. 2013;14(3):e128-137
10. Bhanumathi VS, Rao SV. Diagnosis and management protocol of lingual thyroid. Journal of Evolution of Medical and Dental Sciences. 2016 Jan 4;5(1):100-3.
11. James D,  Anchan J,  Antley M,  Eighmy S. Neonate with a tongue mass. Neoreviews. 2022;23 (6):e425–e428
12. Shah M,  Mukthapuram S,  Melton K,  House M. A term infant with respiratory distress after feeding. Neoreviews. 2023;24(5):e322–e325
13. Jain P, Prasad A, Sachan S, Jain S. A rare case of neonatal oral tumor. Neoreviews. 2023;24(2):e117-119