INTRODUCTION
A teratoma is defined by Willis as a true neoplasm composed of multiple tissues foreign to the part in which it arises [1]. Teratomas are one of the common extragonadal germ cell tumors (EGCTs) of childhood, consisting of tissues from at least two of the three germ cell layers [2]. The clinical manifestation depends on the size and site of the lesion. The lesions presenting in the aerodigestive tract result in nasal blockage, difficulty in breathing, breastfeeding, rhinorrhea and snoring. It can also present with occasional bouts of respiratory distress and cyanosis [3]. The teratomas of the head and neck region might pose difficulty in surgical management. We present a case report of a 46-day-old boy with a nasopharyngeal mass that was histologically defined as a mature teratoma, and summarize a review of the literature on teratomas of the nasopharynx.
CASE REPORT
A 46-day-old boy was referred to the Department of Oral and Maxillofacial Surgery regarding opinion on the mature teratoma present over the right nasopharyngeal region, which was causing difficulty in breathing. The patient is a full-term baby who developed fever at the 7th day of life and was admitted to a local hospital, where the patient developed respiratory distress along with fever. The patient was intubated and on mechanical ventilation. Blood cultures indicated candida sepsis for which anti-fungal therapy was started. Extubation was tried but failed due to persistent respiratory distress. The patient was put back on synchronized intermittent mandatory ventilation–pressure control with pressure support (SIMV-PC+PS) mode, and the tube position was confirmed with a chest X-ray.
In view of suspecting upper airway obstruction, the patient was planned for bronchoscopy. On bronchoscopic examination, a nasopharyngeal swelling was found over the right side of the nasopharynx. After the patient was stable, a magnetic resonance imaging (MRI) head and neck was performed, which showed a peripherally enhancing mass within the right side of the neck involving parapharyngeal, carotid and extending into the retropharyngeal space nearing the foramen magnum and the jugular foramen (Fig. 1). Computed tomography chest revealed sub-segmental collapse and consolidation within the superior segment of the left lower lung lobe. Anterior glottic webs, along with subglottic stenosis with granulations, were also noted. The incisional biopsy was done, which was suggestive of mature teratoma. Repeat blood cultures were negative, and the anti-fungal therapy was stopped. Blood investigations also revealed elevated alpha-fetoprotein (AFP) and β-human chorionic gonadotropin (β-hCG) levels. The patient developed polyuria along with abnormal potassium levels and elevated urine sodium levels. In kidney-ureter-bladder ultrasonography, a few echogenic foci were noted in the lower calyx of the left kidney, which were suggestive of calcifications. The patient was suspected of Bartter syndrome, and further evaluation was suggested once the baby was stable.
A debulking of the tumor mass was planned with the Department of Pediatric Surgery and the Department of Otolaryngology to relieve the respiratory distress. Routine pre-operative workup was done, and the surgery was planned.
General anesthesia was induced using fiberoptic bronchoscopy. A Weber-Ferguson incision extending till soft palatal as a Loeb incision (Fig. 2). The incision was modified to a Loeb incision with extension over the alveolus and continuing into buccal sulcus as a high vestibular incision was planned (Fig. 3). An impression of the maxillary arch was made using polyvinyl siloxane putty impression material loaded into a teaspoon compatible with size of the maxillary arch of the baby (Fig. 4). The incisions were marked and placed. A full-thickness mucoperiosteal flap was elevated using Molts No. 9 periosteal elevator, extending the flap posteriorly till the soft palate, excluding the uvula. The elevated flap gave us adequate visualization for the tumor mass. Utilizing a combination of surgical blade and electrocautery tips, the tumor mass was debulked (Fig. 5A). The excised tumor mass was sent for biopsy. The greater palatine artery was saved, which was crucial for the blood supply for the growing maxilla (Fig. 5B). The flap closure was done using 4-0 Vicryl sutures (Ethicon) (Fig. 6). The general anesthesia was reversed, and the patient was shifted to the neonatal intensive care unit for postoperative care. The histopathological report of the debulked mass was suggestive of mature teratoma.
Postoperatively, the patient required ventilator support on pressure control mode for 1 week. An attempt to wean the patient by switching to SIMV mode was unsuccessful, as the patient developed respiratory distress and was returned to ventilator support. Venous blood gas and electrolyte analysis revealed acidosis and hyperkalemia, which were managed with sodium bicarbonate and potassium correction using human insulin. Due to these complications, postoperative imaging was postponed due to which evaluation of the airway was not performed. On postoperative day 15, clinical evaluation revealed intermittent deviation of the right eye into an inferolateral (“down and out”) position (Fig. 7), with spontaneous return to primary gaze, consistent with a partial oculomotor palsy, likely reflecting impaired function of the extraocular muscles supplied by the oculomotor nerve, with unopposed action of the lateral rectus and superior oblique. The patient’s family opted for leave against medical advice and transferred care to a local hospital, preventing further evaluation of the ocular findings and the underlying etiology, which could have been due to residual lesion, surgical manipulation, or ischemic insult. We were subsequently informed that the patient had expired, and the exact cause of death could not be ascertained.
LITERATURE REVIEW
Germ cell tumors (GCTs) can be found in all age groups, from the fetal period to adulthood. GCTs account for around 3% to 4% of all tumors among children younger than 15 years. GCTs in non-gonadal sites closer to the axis of the body are common, which include sacrococcygeal region, the mediastinum or the pineal gland [4].
Two peaks of incidence are observed in the case of GCTs. The first peak includes teratomas and yolk sac tumors, arising mainly in the sacrococcygeal region, testis and less frequently in the mediastinum or retroperitoneum. The second peak occurs in the second decade of life, which includes tumors such as geminoma and nonseminomas such as embryonal carcinoma, choriocarcinoma and yolk sac tumors. Among these, ovarian cystic teratoma (dermoid cyst) is the most commonly occurring benign tumor [4].
Teratomas are defined as true neoplasms composed of multiple tissues foreign to the part in which they arise, typically consisting of tissues from at least two of the three germ cell layers [1]. Teratomas comprise the most common EGCTs [5]. The incidence of teratomas is approximately 1 in 40,000 live births, with the sacrococcygeal region being the most common extragonadal site. Sacrococcygeal teratomas have a female predominance of up to 87%, whereas craniofacial teratomas have an overall male-to-female ratio ranging from 1:1 to 2:1 [2].
Only 5% of EGCTs of the head and neck region are benign, and around 6% are malignant. Since teraomas comprise of all three germ layers, they are capable of transforming into various carcinomas and sarcomas. Acharya et al. [5] reported a case of primitive neuroectodermal tumors in a 6-year-old boy with oral teratoma.
However, most of the literature has reported incidence ranging from 2% to 9% for teratomas, which occur in the head and neck region, the location of which often leads to significant clinical issues due to their location [1,5–10].
These lesions are classified as teratomas, dermoids, and epidermoids by Smirniotopoulos and Chiechi [11]. Epidermoids are ectoderm-derived simple inclusion cysts that are simply bordered by squamous epithelium. Although they may have hair and other dermal appendages, dermoids are also ectodermally generated. They continue by defining a teratoma as a lesion that exhibits “histologically divergent differentiation,” rather than necessarily one that originates from the ectoderm, endoderm, and mesoderm, the three primordial germ layers.
Arnold’s system of classification as follows: (1) dermoids, which are composed of two germinal layers (ectoderm and mesoderm) and are the most common forms of teratomas; (2) teratoid tumors, which are composed of all three germinal layers (ectoderm, mesoderm, and endoderm) and are poorly differentiated; (3) true teratomas, which contain histologically identifiable tissues from all three germ layers and may be solid or cystic; and (4) epignathi, in which fetal organs may be identified and limb formation can occur, representing the most highly differentiated form known as “fetus in fetu.” The World Health Organization classifies teratomas based on histology into mature teratomas (solid or cystic [dermoid cyst]), immature teratomas, teratomas with malignant transformation, and monodermal teratomas [4].
Teratomas can also be classified as benign or possibly malignant based on histology. In contrast to other GCTs, AFP is missing in benign lesions but detectable in possibly malignant teratomas. In both benign and malignant forms, β-hCG levels are of less diagnostic value. Both juvenile and mature teratomas exhibit questionable sensitivity to radiation and chemotherapy in terms of treatment sensitivity. Therefore, the primary treatment for teratomas is surgical excision [4].
Nasopharyngeal teratomas, while rare, present significant challenges due to their potential to cause severe airway obstruction in neonates. Most of the nasopharyngeal teratomas are diagnosed at birth or soon after. They might be sessile or pedunculated. Most of the neonates with teratomas in the nasopharyngeal region are diagnosed with asphyxia neonatorum [3]. The case presented here is of a 46-day-old boy with a suspected Bartter syndrome. This case aligns with the literature, highlighting the rarity and the potential severity of nasopharyngeal teratomas.
DISCUSSION
Clinical manifestations of nasopharyngeal teratomas depend on the size and location of the lesion. In this case, the infant presented with respiratory distress, a common symptom due to the mass effect on the aerodigestive tract, causing nasal blockage, difficulty in breathing, and feeding issues. Such presentations are consistent with other reported cases where airway obstruction and respiratory distress were prominent features. Teratomas in general can be histologically and oncologically benign. In cases where malignant components exist, they have the potential to exhibit aggressive behavior. As a general rule, pediatric teratomas of the head and neck tend to be oncologically benign, adult teratomas tend to be oncologically and histologically malignant [12].
The preliminary diagnosis in this case was facilitated by fiber optic bronchoscopy, which revealed a mass in the right nasopharyngeal region. This diagnostic approach is supported by the literature, which advocates for imaging and endoscopic evaluations to delineate the extent of the mass and plan for surgical intervention. The differential diagnosis for the nasopharyngeal teratomas includes the lesions that can also present with an oropharyngeal mass like encephelocele and nasal gliomas, both of which are benign congenital malformations and are a rare occurrence [10] and also dermoid cyst, lymphangioma, hemangioma and neurofibromatosis [13]. Congenital teratomas can be diagnosed through prenatal ultrasonography in the first trimester and through fetal MRI in the later stages [14]. The histological analysis confirmed the presence of a mature teratoma, consistent with previous reports where mature elements predominate in neonatal teratomas.
Surgical intervention remains the primary treatment for nasopharyngeal teratomas. In this case, a multidisciplinary team approach was employed, involving oral and maxillofacial surgery, pediatric surgery, and otolaryngology. The surgical technique used, including a Loeb incision and careful preservation of critical vascular structures, aligns with best practices highlighted in the literature for managing such complex cases. The excised mass was sent for biopsy, confirming the diagnosis and guiding further management.
Postoperatively, the patient required intensive care, including mechanical ventilation and management of complications such as metabolic imbalances and persistent respiratory distress. These postoperative challenges are not uncommon and have been documented in other case reports, emphasizing the need for vigilant postoperative monitoring and supportive care.
This case contributes to the growing body of evidence on nasopharyngeal teratomas, highlighting the importance of early diagnosis, a multidisciplinary approach to surgical management, and comprehensive postoperative care. Continued documentation and review of similar cases are essential for improving the understanding and management of this rare but serious condition.
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