The term teratoma is often used to describe a tumour that constitutes tissue foreign to the area in which it is located [1]. These tumours are generally rare, with an incidence of one in 4000 live births [2], show a female predominance, and have an 18% risk of other congenital malformations [3]. Only 10% of all teratomas occur in the head and neck region, predominantly in the cervical and nasopharyngeal areas. Naspoharyngeal teratomas arise from the skull base or the posterior pharyngeal wall and extend inferiorly to cause obstruction of the upper aerodigestive tract. Sometimes they are so large they can protrude from the oral cavity or extend along the Eustachian tube into the middle ear.

The most widely accepted terminology describes four types of teratomas: dermoids, teratoids, true teratomas and epignathi. Dermoids, the most common form, are composed of ectoderm and mesoderm and can contain hair, sebaceous and sweat glands, and squamous epithelium. Dermoid cysts containing ectomesodermal elements are usually found in the nasal midline along the dorsum, often with hair protruding from the lesion together with the fistulous tract [4]. Teratoids (non-hairy polyps) are rarely seen and are composed of all three germ layers, but are poorly differentiated [5]. True teratomas are congenital masses and have all three elements of ectoderm, endoderm and mesoderm in various degrees of maturity, and may contain cartilage, bone, thyroid or glial tissue. The term epignathus is used when teratomas are located in the oral cavity or pharyngeal region and when they arise from craniofacial bones – mainly from palatine bone, sphenoidal bone or ethmoid bone. They derive from all three germ cell layers and present the highest form of teratoma differentiation.

The suspicion of teratoma is usually based on the external appearance of the tumour and its congenital character. The radiographic diagnosis of teratoma is suggested by the presence of calcifications on the plain film or mixed echogenicity with multiloculated cystic or solid regions on ultrasonography [4]. Biopsy is not usually performed due to problems connected with the tumour´s presence and the necessity of its total removal.

Children born with massive cervical teratomas often have an immediately compromised airway that requires securement either by endotracheal intubation or by tracheotomy [6].

Prognosis in the case of the pharynx and oral cavity is good. Malignant transformation has been described only in adults [7]. Definitive treatment is complete surgical resection [1, 4, 5].

PJ (3492/2003), a 2.5-kg girl born at 37 weeks gestation, with 9 points in the Apgar scale. From the first days of life in the newborn child effort during feeding was observed. In the course of several days before hospitalisation choking during feeding occurred, sometimes with vomiting. On the eve of admission to the hospital parents noted a red tumour in the oral cavity which caused feeding and breathing problems. After the next episode of choking the parents came to the paediatric hospital. At admission to the hospital the child´s general condition was good, without dyspnoea. The only abnormality in the paediatrician´s examination was a thickening of the left palatopharyngeal arch. The child was admitted to the neonatal department and the same day a basic radiological diagnostic examination was performed. Chest x-ray, ultrasonography through the fontanel and ultrasonography of the abdominal cavity were normal. In the ultrasonography of the neck and larynx laterally and posteriorly to the larynx, on the left side an abnormal mass was present. It measured 19 x 12 x 9 mm and its echogenicity was higher than muscle tissue. This change had a smooth contour and from its superior part upwards was present a stalk which was not visible above the epiglottis. No large vessels within the change were present. The change was localised in the left piriform recess and in the oesophagus and modelled posterior contour of the larynx. Mobility of the vocal cords was normal. In the computed tomography of the parapharyngeal space, in the prevertebral region, on the posterior wall was visible an abnormal mass descending from the level of the epiglottis to the oesophagus. This mass was visible in the oesophagus lumen for a length of about 2 cm. This formation did not intensify after giving contrast. A tumour of the oesophagus or oesophageal duplication was suspected. An x-ray of the oesophagus showed normal passage of barium contrast to the stomach. In the oesophagus was visible a tuberous change dilating its lumen, reaching the level of the clavicles and dislocating the trachea anteriorly.

On the third day of hospitalisation, after feeding, choking was observed and just after this episode in the oral cavity a tumour was demonstrated. Evening episodes of choking became more frequent and falls of saturation below 86% were observed. The child was intubated and transferred to the Intensive Care Unit. Two days later endoscopy of the nasopharynx and oesophagus was performed. During examination a smooth pedunculated tumour, pink in colour, extending out from the left torus tubarius and descending to the oesophagus was found. After pulling the tumour upwards the oesophagus was controlled. Esophagoscopy revealed a normal oesophagus. The tumour was removed by cutting off the stalk. Bleeding was small. In the histopathological examination teratoma was recognised.

WL (11661/2004), a 6-week-old female infant was transferred from the paediatric department to the Department of Paediatric Otorhinolaryngology because of a tumour in the pharynx causing difficulties during breathing and feeding. This tumour was observed from the fifth week of life, but just after birth coryza and choking during feeding were present. These symptoms intensified during several days preceding admission to the Department of Paediatric Otorhinolaryngology. At the moment of admission to our department the general child´s condition was mean severe, with inspiratory dyspnoea and saturation of about 90-93%. During the preliminary laryngological examination the dyspnoea intensified; the child required urgent intubation and was prepared for surgery. Intraoperatively a tumour covered by hairy skin with a wide stalk arising from the superior part of the left palatopharyngeal arch was found. The diameter of the tumour was 18 mm. The tumour was removed surgically without profuse bleeding. The histopathological examination revealed within the hairy polypous tumour cartilaginous, muscular, fatty and connective tissues with the presence of thin-walled vessels. Teratoma was recognised.

OS (10489/2005), a 7.5-year-old girl was admitted to the Department of Paediatric Otorhinolaryngology because of a tumour in the oral cavity. This tumour was noticed a few hours before admission to the hospital and was not observed earlier. On the admission day the girl woke up at midnight because of vomiting and diarrhoea. After an episode of vomiting the girl complained of a foreign body sensation in the oral cavity and difficulties in swallowing. Her mother noticed a red tumour in the oral cavity and small bleeding from the tumour. During the two days before hospitalisation the girl had gastroenterocolitis with vomiting, diarrhoea and fever. From early childhood obstruction of the nasal patency, snoring and nasalization were observed in the girl, but no diagnostic of the nasal obstruction was ever performed. In the laryngological examination a red tumour localised symmetrically on the base and corpus of the tongue, with transverse diameter about 3 cm and sagittal diameter about 2 cm, was found. This tumour made estimation of the oropharynx impossible. In the oral cavity saliva with fresh blood was present. The girl was in a good general condition, without breathing difficulties, but she had nausea and coffee-grounds vomiting. The tentative diagnosis was: haemangioma of the base of the tongue. The chest x-ray and ultrasonography of the abdominal cavity and neck were normal. In laboratory investigations elevated leucocytosis (26 thousand/mm) was present, but the level of haemoglobin (13.2 g%), thrombocytes (270 thousand/mm), ESR (10 mm/h), and coagulation system parameters were normal. A CT scan of the nasopharynx demonstrated a tumour extending from the inferior contour of the adenoid to the base of the tongue with diameters 41 x 18 x 30 mm, probably arising from the base of the tongue. Surgical removal of the tumour was performed a few hours after admission to the hospital. After intubation and securing airway potency direct estimation of the tumour was possible. The tumour had a long stalk extending out from the inferior part of the adenoid on the left side. The histologic examination revealed benign teratoma.

Head and neck teratomas are uncommon but can be associated with airway obstruction and high rates of mortality in untreated patients. Teratomas of the oral cavity and pharynx are most frequently pedunculated tumours, covered by hairy skin or mucous membrane and composed of tissues from three germ layers with varying degrees of differentiation [8]. Sometimes within the tumour calcifications are visible. Hairy polyps are less rare than non-hairy polyps; they are found more frequently in female infants, mainly on the left side. The tumour and its stalk have poor vascularization. In all our described patients the tumours were pedunculated, extending out from the pharyngeal wall on the left side, and bleeding during operation was small.

Teratomatous polyps are usually recognised in the first days or weeks of life, but when the tumour is small and there are no indications for laryngological examination this tumour may be recognised later, but usually in the first years of the child´s life. These tumours usually do not increase with the child´s growth; small tumours can be found by chance during pharyngeal examination. However, in most cases symptoms connected with the tumour are present from the first days of life and compel parents to seek medical aid. We suppose that the late recognition and rapid enlargement of the tumour in the described 7.5-year-old girl (case 3) could be a result of interruption of the vessel within the tumour due to persistent vomiting in the course of gastroenterocolitis.

Most nasopharyngeal teratomas are a cause of various degrees of airway obstruction. Large teratomas obstructing the airway can be a cause of asphyxiation just after birth. The majority of neonates with naspoharyngeal teratomas can be intubated without problems via the oral or nasal routes. Sometimes tracheostomy is necessary. No tracheotomy was required in our patients with nasopharyngeal teratoma.

The dislocated pedunculated polyp dislocating can be a cause of feeding difficulties. Teratomatous polyps with a long stalk can extend to the oral cavity or outside, giving changeable symptoms. It was present in the first case.

It is very important to perform the diagnosis of this nasopharyngeal tumour before birth. It allows obstruction of the neonatal airway to be prevented and makes possible prenatal planning and prompt postnatal surgical treatment. In utero the tumour occluding the foetal airway may not cause significant problems, but it may impede foetal swallowing, leading to the accumulation of amniotic fluid or maternal polyhydramnions. Antenatal diagnosis is possible when elevated maternal alpha-fetoprotein (AFP) is detected, polyhydramnios is present or when the tumour mass is visible on ultrasound scan [9].

1. Nasopharyngeal teratomas appear most frequently in the first weeks of neonatal life, giving symptoms connected with mechanical obstruction of the aerodigestive tract.

2. "Favourable” location and small size of the tumour can favour persisting of the tumour to later childhood or to the moment of its sudden enlargement.