Large congenital facial teratoma

Background

Teratomas are rare congenital neoplasms originating from all three germinal cell layers. They are even rare in the head and neck, which account for only 2%, and those with extracranial or intracranial extensions are very unusual.1 This feature confers high mortality, and those who have had surgery have mostly remained with varying degrees of morbidity.2 Moreover, one of the most frequent complications of paediatric head and neck teratomas is airway compromise.3 We present a case of a giant congenital facial teratoma that was managed with a complete surgical resection.

Case presentation

A baby boy was brought with a large right mass present since birth. He was born by caesarean section of a primiparous woman, gravida 0, para 0, at the 38th week, with one live and healthy issue at term gestation. Antenatal ultrasound reported an elongated facial appendage measuring 54×23×49 mm (L×H×W) and volume 32 mL, which was noted to be arising from the right parotid (figure 1A). It was a smooth, hypodense cystic lesion and did not concern vascularity on colour Doppler imaging (figure 1B).

Figure 1

(A) Ultrasound reported an elongated facial appendage measuring 54×23×49 mm (L×B×W) and volume 32 mL. (B) Hypodense cystic lesion not concerning vascularity on colour Doppler imaging.

The fetus followed an average overall growth, the patient was confined by elective caesarean section at term and the neonate was delivered in good overall condition.

According to the parents, the child had no pain, difficulty chewing food and opening his mouth, difficulty in hearing or other neurological deficits.

On examination, the mass occupied the lateral part of the face and measured about 6.5×5×4 cm (figure 2), extending superiorly to the right pterygoid region to the submaxillary ipsilateral.

Figure 2

The right mass on the face measures about 6.5×5×4 cm.

Investigations

Contrast-enhanced CT with the reconstruction of the head and neck showed a large extracranial well-defined rounded hypodense (12 Hu) intensity mass lesion measuring approximately 6.5×5×4 cm along the right submaxillary (figure 3).

Figure 3

Large extracranial well-defined rounded hypodense (12 Hu) intensity mass lesion measuring approximately 6.5×5×4 cm along the right submaxillary.

The three-dimensional biomodel reconstruction used in this case was performed using CT of the anatomical area. This technique allowed the doctor–patient communication since it allowed the latter to have a better understanding of their pathology and the suggested procedure (https://sketchfab.com/3d-models/contento-a812f3a347984e8e991c5f25714d0637).

Differential diagnosis

The differential diagnoses include cystic hygroma, a benign tumour that originates from the obstruction of the drainage of the lymphatic sacs4; a cyst of the thyroglossal duct, which is embryological remains of an evagination of the endoderm in the migration path of the thyroid gland; and hamartoma, which is a mature tissue structure resulting from the involvement of one of the three germ layers compared with teratoma involving all three layers.3

Treatment

The boy underwent surgical removal of the teratoma at 16 months of age. The tumour was successfully revealed using a lateral facial approach, where a preauricular and temporal incision was made to access it (figure 4A). There was a large cystic mass of about 7×5×4 cm in the right submaxillary area extending to the cheek, with no visual deficit in the skull. Posteriorly, the mass was densely adherent to the anterior wall of the parotid, which was gradually teased off. The facial muscles were thinned out and stretched over the mass to remove the tumour, integrating them into certain tumour areas.

Figure 4

(A) The right submaxillary facial incision was made over the mass, extending to the infraorbital region. (B) Excision of the mass and closure.

Furthermore, the facial nerve branches were also stretched over the tumour. The excision of the entire tumour was performed with utmost care to preserve as much muscle and nerve tissue as possible. Excess skin was excised and closed (figure 4B). We used an intraoperative device for facial nerve monitoring to identify and minimise facial nerve injury.

Postoperatively, the patient received intravenous analgesics and antibiotics. He recovered well with no neurological deficit or wound infection and was discharged on postoperative day 2.

The histopathology report showed a mature cystic teratoma lesion filled with yellow fluid with an intact capsule. The tumour had squamous, ciliated columnar, intestinal epithelium, neuronal tissue, gastric tissue, salivary gland, lymphoid follicles, adipose tissue, muscle bundles, foamy macrophages, multiple congestive capillaries and blood vessels, hyaline cartilage and nerve fillets of variable sizes, generating images reminiscent of nerve plexuses (figure 5A,B).

Figure 5

(A) The tumour had squamous, ciliated columnar, intestinal epithelium, muscle bundles and foamy macrophages. (B) The tumour had hyaline cartilage and nerve fillets of variable sizes, generating images reminiscent of nerve plexuses.

Outcome and follow-up

The patient had an unremarkable recovery period, and he has been on follow-up for 28 months now and is doing well with no recurrent growth or neurological or sensitive deficits (figure 6).

Figure 6

Patient recovery and follow-up.