Abstract
Teratomas are true neoplasms that contain tissues foreign to the site in which they arise. They are derived from germ cells with components of the three embryonic layers (ectoderm, mesoderm and endoderm). The present report is about a 10 day old neonate with teratoma arising from the nasopharyngeal surface of the soft palate, which was successfully removed.
Keywords: Nasopharynx, Soft palate, Teratoma, Neonate
Introduction
Teratomas are the most common extragonadal germ cell tumors of childhood. They arise most commonly in a midline or paraxial location from the brain to the sacral area and involve the head and neck in 1 of every 40,000 births [1]. In this region, sites of involvement include the soft tissues of the neck (cervical teratoma), superficial facial structures in and about the temporal fossa and zygomatic area, oral cavity, oronasopharynx, and orbit [2].
Teratomas grow aggressively, and in the head and neck, they most commonly occur in the cervical area, followed by the nasopharynx. Overall, teratomas of the head and neck comprise approximately 2–9% of all teratomas [3, 4].
Four basic types of teratoma are recognized: (1) dermoid cyst, the most common form, composed of ectoderm and mesoderm arising as an epithelium-lined cavity with variable numbers of skin appendages; (2) teratoid cyst, derived from all three germ layers but poorly differentiated; (3) true teratoma, composed of ectoderm, mesoderm, and endoderm, with specific tissue and organ differentiation; and (4) epignathus, in which well-developed fetal parts are recognizable.
Case Report
A 10 day old male child presented with noisy breathing and bluish discolouration of skin while crying after 2 days of delivery. Child was admitted in NICU for 1 day, suspected to have upper airway obstruction and referred to our tertiary care center for further management. The baby was born by cesarian section at 35 weeks of gestation, with birth weight 2.75 kg.
At the time of admission child was on hood oxygen with 3 lt maintaining saturation 95%, child was active, pink and warm. There was no obvious, visible congenital abnormality.
Neurological examination was normal. Suprasternal retraction was seen, bilateral air entry was equal. Hemodynamically stable. The mother’s antenatal history was uneventful.
Flexible nasopharyngolaryngoscopy was done which showed mass in the nasopharynx, firm to hard consistency protruding into the oropharynx.
MRI neck without contrast showed lobulated abnormal signal soft tissue intensity mass lesion occupying the nasopharynx and extending inferiorly in the upper part of oropharynx, measuring 1.5 × 1.7 × 2.4 cm AP, transverse and craniocaudal dimension respectively resulting in complete effacement of nasopharyngeal and upper oropharyngeal airway. Upper extent of the lesion is seen in the level of soft palate, and inferiorly reaching up to the level of base of the tongue.
After complete preoperative and preanesthetic evaluation, child was intubated. Mass was visualised clearly with nasal endoscope and tranorally, its attachment to the nasopharyngeal surface of the soft palate was delineated and mass was excised completely till its attachment. The root of attachment was severed by radiofrequency and hemostasis achieved. Intraoperative and post operative period was uneventful. The mass measured 2.5 × 2 × 1 cm approximately.
Histopathology revealed mature teratoma from the soft palate. Which was composed of polypoidal tumor with surface ulceration and areas with dilated cystic spaces lined by ciliated glandular epithelium. Glial tissue component noted along with mature adipocytes and epithelial clusters.
Discussion
Teratomas are developmental lesions, clinically, dermoids are more common than true teratomas, and in the nasopharynx, the dermoid cyst is the most common developmental anomaly found known as “hairy polyps”, these lesions appear at birth as a pedunculated mass filling the nasopharynx, often with oropharyngeal extension.
Patients with teratomas have nasal obstruction, dysphagia, and copious secretions. Infants with nasopharyngeal teratomas have a higher incidence of preterm birth, neonatal airway distress, and associated congenital anomalies along with polyhydramnios than do infants with dermoids [3].
Most cervical teratomas occurring in the neonate are benign. Surgical removal must be carefully planned to ensure a controlled airway throughout the intraoperative and postoperative periods. Tracheostomy is generally not necessary if orotracheal intubation is combined with a transoral removal of pedunculated nasopharyngeal teratomas or with early excision of cervical teratomas. Operative and postoperative bleeding in patients with nasopharyngeal teratomas is usually only slight because these tumors are poorly vascularized, and if removal is complete, recurrences are rare.
More sessile tumors arising in the nasopharynx require a transpalatal approach. Malignant metastasizing cervical teratoma is extremely rare [3]. More than 75% of cervical teratomas are diagnosed in neonates, two-thirds of whom have respiratory distress significant enough to cause stridor [5]. Hydramnios, stillbirth, and premature labor are other frequent accompaniments [2].
The EXIT procedure (Ex utero intrapartum treatment) was initially described for the resolution of temporary tracheal occlusion used in the congenital diaphragmatic hernia while maintaining uteroplacental circulation [6].
In utero diagnosis of a large cervicofacial teratoma will allow time for perinatal planning of the EXIT procedure, to secure the infant’s airway before the maternal–fetal circulation is divided [7].
Although there is a female-to-male preponderance of 6:1 in other body regions, the female-to-male ratio is equal in the head and neck [8]. The differential diagnoses for the nasopharyngeal teratomas include the lesions that can also present with oropharyngeal mass like encephalocoele, meningocoele, nasal glioma, congenital rhabdomyosarcoma, hemangiomas, neurofibromatosis, congenital epulis, congenital epignathus and lymphatic malformation. Other differential diagnoses for nasopharyngeal cause for airway obstruction are choanal atresia, choanal stenosis, mid-nasal stenosis, piriform aperture stenosis, and craniofacial abnormalities.
In the literature, the major clinical difficulties reported are either obstetrical (due to premature delivery, difficult labour with malpresentation, or stillbirth), or surgical (due to potentially fatal neonatal airway obstruction) [9, 10]. Failure to operate in the neonatal period has been associated with a mortality rate of 80–100% [9].
In the review of 18 cervical and nasopharyngeal teratoma case series done by R. W. Byard et al., prematurity and complications of pregnancy such as polyhydramnios were always associated with poor clinical outcomes. The chances of survival in live-born neonates were influenced by the interplay of factors such as pulmonary hypoplasia due to in utero airway obstruction, the extent of neonatal airway obstruction and the degree of pulmonary immaturity. Specifically none of the patients with nasopharyngeal lesions had any of the reported associated anomalies such as anencephaly or hemicranium [10, 11].
In case series of head and neck teratomas consisting of 23 cases, done by Alexander et al. [12], they say that long term survival and morbidity of head and neck teratomas is excellent following surgery. However this does depend significantly on the anatomical location. Alpha fetoprotein is frequently used to monitor for the evidence of recurrent disease but its usefulness as an initial diagnostic tool is unclear. As found in this case series not all patients with teratoma present with an elevated alpha feto protein (Figs. 1, 2).
Fig. 1.
a Intraoperative photograoh showing mass in the oropharynx b Non contrast MRI neck showing soft tissue mass lesion occupying the nasopharynx with extension into oropharynx
Fig. 2.
a Photomicrograph showing cystic space lined by columnar cells, b component of glial tissue, c epithelial clusters, d vascular spaces
Conclusion
Nasopharyngeal teratoma is a rare benign entity. Teratoma must be considered in the differential diagnosis of lesions found in the nasopharynx and nasal cavity, mainly in neonates. Endoscopic and imaging studies (CT and MRI) promote early diagnosis and improve outcome.
Compliance with Ethical Standards
Conflict of interest
All authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speaker’s bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge or beliefs) in the subject matter or materials discussed in this manuscript.
Ethical Approval
All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
Informed Consent
Identifying information is not included in this article.
Footnotes
This article or any part of it has neither been published earlier nor been presented in any conference or seminar.
References
- 1.Kountakis SE, Minotti AM, Maillard A, et al. Teratomas of the head and neck. Am J Otolaryngol. 1994;15:292. doi: 10.1016/0196-0709(94)90099-X. [DOI] [PubMed] [Google Scholar]
- 2.Batsakis JG. Teratomas of the head and neck with emphasis on malignancy. Ann Otol Rhinol Laryngol. 1995;104:496. doi: 10.1177/000348949510400616. [DOI] [PubMed] [Google Scholar]
- 3.Coppit GL, Perkins JA, Manning SC. Nasopharyngeal teratomas and dermoids: a review of the literature and case series. Int J Pediatr Otorhinolaryngol. 2000;30:219–227. doi: 10.1016/S0165-5876(00)00288-3. [DOI] [PubMed] [Google Scholar]
- 4.Day LH, Arnold GE. Rare tumors of the ear, nose and throat: second series: uncommon benign tumors of the head and neck. Laryngoscope. 1971;81:1138–1174. doi: 10.1288/00005537-197107000-00015. [DOI] [PubMed] [Google Scholar]
- 5.Hayani A, Mahoney DH, Jr, Hawkins HK, et al. Soft-tissue sarcomas other than rhabdomyosarcoma in children. Med Pediatr Oncol. 1992;20:114. doi: 10.1002/mpo.2950200205. [DOI] [PubMed] [Google Scholar]
- 6.Subrahmanyam HK, Raman EV, Radhakrishnan P. EXIT (Ex Utero Intrapartum Treatment): a new challenge for the otolaryngologist. Indian J Otolaryngol Head Neck Surg (July–September 2009) 61:000–000
- 7.Ward VM, Langford K, Morrison G. Prenatal diagnosis of airway compromise: EXIT and fetal airway surgery. Int J Pediatr Otorhinolaryngol. 2000;53:137. doi: 10.1016/S0165-5876(00)00323-2. [DOI] [PubMed] [Google Scholar]
- 8.White L, Siegel SE, Quah TC. Non-Hodgkin’s lymphomas in children: I. Patterns of disease and classification. Crit Rev Oncol Hematol. 1992;13:55. doi: 10.1016/1040-8428(92)90016-J. [DOI] [PubMed] [Google Scholar]
- 9.Gundry SR, Wesley JR, Klein MD, Barr M, Covan AG. Cervical teratomas in the newborn. J Pediatr Surg. 1983;18:382–386. doi: 10.1016/S0022-3468(83)80186-9. [DOI] [PubMed] [Google Scholar]
- 10.Rowe LD. Neonatal airway obstruction secondary to nasopharyngeal teratoma. Otolaryngol Head Neck Surg. 1980;88:221–226. doi: 10.1177/019459988008800305. [DOI] [PubMed] [Google Scholar]
- 11.Holinger LD, Birnholz JC. Management of infants with prenatal ultrasound diagnosis of airway obstruction by teratoma. Ann Otol Rhinol Laryngol. 1987;96:61–64. doi: 10.1177/000348948709600115. [DOI] [PubMed] [Google Scholar]
- 12.Alexander VRC, et al. Head and neck teratomas in children—a series of 23 cases at Great Ormond Street Hospital. Int J Pediatr Otorhinolaryngol. 2015;79:2008–2014. doi: 10.1016/j.ijporl.2015.07.042. [DOI] [PubMed] [Google Scholar]