© Borgis - New Medicine 3/2006, s. 60-62
Anna Bielicka, Lidia Zawadzka-Głos, Mieczysław Chmielik, Eliza Brożek-Mądry
Teratomas of the nasopharynx in children. A report of three cases
Department of Paediatric Otorhinolaryngology, Medical University of Warsaw, Poland
Head of Department: Prof. Mieczysław Chmielik, MD, PhD
Teratomas are neoplasms composed of tissues from all three germ layers with varying degrees of differentiation. They are most common in the sacrococcygeal and gonadal region and are rare in the head and neck region. These tumours are usually benign, but they have a high incidence of malignancy when found in adults. The treatment of choice for head and neck teratomas is surgical excision. The authors describe three cases of nasopharyngeal teratomas diagnosed and treated in the Department of Paediatric Otorhinolaryngology of the Medical University of Warsaw. In all children the tumours were removed surgically. All excised tumours were subjected to histopathological examination to confirm their teratomatous nature. Follow-up did not show any recurrence in the operated children.
The term teratoma is often used to describe a tumour that constitutes tissue foreign to the area in which it is located . These tumours are generally rare, with an incidence of one in 4000 live births , show a female predominance, and have an 18% risk of other congenital malformations . 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 . Teratoids (non-hairy polyps) are rarely seen and are composed of all three germ layers, but are poorly differentiated . 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 . 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 .
Prognosis in the case of the pharynx and oral cavity is good. Malignant transformation has been described only in adults . 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.
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