Teratoma

Abstract

Teratomas are rare congenital neoplasms that arise from pluripotent cells. They consist of tissue from all three embryonic germ layers. According to most studies, teratomas of the head and neck account for only 2 to 9% of all cases. In this article, we report the first case of a teratoma of the nasal vestibule in a newborn. We also discuss methods of diagnosis and treatment of upper airway teratomas, and we include a brief review of the literature.

Introduction

Teratomas are true neoplasms made up of a wide variety of tissues that are foreign to the site where they arise. [1] Teratomas are one particular type of several different developmental malformations. It has been observed that most extragonadal teratomas are axial or paraxial to a line from the brain to the sacral area. [2] The most common locations are the sacrococcygeal, retroperitoneal, mediastinal, and head and neck regions. Teratomas of the head and neck account for 2 to 9% of all teratomas. [3]

The most common sites of teratomas of the head and neck are the cervical region and the nasopharynx. [1,4] Other sites reported in the literature include the larynx [5]; mastoid region [6]; soft palate [7]; orbit, face, and tongue [8]; eustachian tube [9]; and the middle ear, [10] but teratomas at these locations are not common. To our knowledge, this article represents the first published report of a mature teratoma that arose in the nasal vestibule.

Case report

Our patient was a full-term female infant who was born normally following an uncomplicated pregnancy. Immediately following delivery, she was noted to have a 2 x 1.5 x 1-cm mass on the right side of her nose (figure 1). The mass, which did not hinder breathing or feeding, was mobile. Its pedunculated base was attached to the right vestibular area on the nasal septum. The mass was firm, rubbery, covered with skin, and nonpulsating. It was not translucent.

Aspiration was negative for cerebrospinal fluid. A feeding tube was easily passed to the infant's stomach through the left nostril. Both nasal choanae were patent. The larynx, vocal folds, and ears were normal. There were no palpable neck mass, no retinal lesions, and no phakomatosis.

A lateral x-ray of the neck with the nasogastric tube in place showed a normal esophagus and trachea and no calcification in the soft tissue shadow created by the mass. Complete blood and platelet counts were normal.

The infant underwent surgery with local anesthesia (2% lidocaine with epinephrine) delivered to the base of the mass. The narrow stalk of the mass was attached to the superomedial aspect of the right vestibule. After the right ala was retracted, the growth was removed without difficulty. Bleeding was minimal and controlled by pressure.

The defect was primarily closed. No hair was present, but the surface appeared to be covered with lightly pigmented skin. On sectional analysis, the mass had a solid appearance, with grayish-white. and yellow areas. No cystic cavity or hair was present. A complete diagnostic nasal endoscopy ruled out any lesions in the nasal cavity and nasopharynx.

Pathologic examination revealed a light-brown polypoid mass. Microscopy showed that the mass was covered with skin on all aspects, and the fibrous stroma within contained adipose tissue, mature cartilage, smooth muscle fibers, and nerve bundles (figure 2). There was no evidence of malignancy. A histopathologic diagnosis of a mature teratoma was made.

The patient has been followed for 14 months, and no problems have been encountered thus far.

Discussion

Teratomas are primary congenital tumors made up of tissue that arises from all three embryonic germ cell layers: the ectoderm, the endoderm, and the mesoderm (although there have been reports of tumors made up of only bidermal ingredients). [1] The term originated from the Greek teraton, which translates to monster. This name reflects the lesion's capacity for uncoordinated growth, both tumoral and malformative. Teratomas occur in approximately 1 of every 4,000 live births.

Teratomas are believed to arise both from germ cells in their course of migration during embryogenesis and from nongerm embryonic cells that have escaped their regulatory influences. [2] There are four types of teratomas: dermoids, teratoids, true teratomas, and epignathi. [2,11]

Teratomas occur most often in children younger than 1 year old, particularly in newborns. Most of these growths are noted right at birth. Extracranial teratomas of the head and neck account for only 2 to 9% of all teratomas. [3] Whereas teratomas elsewhere on the body usually have a predilection to females, those of the head and neck are divided equally between the sexes. [1,12]

Teratomas involving the nasopharynx, oropharynx, larynx, and hypopharynx usually grow rapidly and cause airway obstruction. They can cause immediate dyspnea at birth; those on long pedicles can cause intermittent respiratory obstruction and dysphagia. [2]

Until now, the occurrence of a mature teratoma appearing as an asymptomatic anterior nasal mass has not been reported in the literature. The differential diagnoses of such a mass include nasal glial heterotopia (nasal glioma), nasal dermoid cyst, encephalocele, hamartoma, choristoma, hairy polyp, and fetal rhabdomyoma.

Radiography helps make the diagnosis. Calcification in the tumor is seen in 16% of cases and is pathognomonic. [13] Computed tomography helps delineate the extent of the tumor and rules out encephalocele, because teratomas characteristically do not cause bone destruction or have an intracranial connection. [14] Fine-needle aspiration cytology can also help make the diagnosis. [1]

An antenatal diagnosis of upper airway teratoma is sometimes possible, because these tumors can elevate maternal alpha-fetoprotein levels and cause polyhydramnios. These masses can also be detected on ultrasound; when they are, followup testing with magnetic resonance imaging is advisable to determine the extent of the lesion.

The treatment of teratomas of the upper airway consists of initiating immediate support of respiratory function and performing a complete resection of the tumor. Early surgical intervention is recommended because teratomas generally grow rapidly. Moreover, early surgery can avoid the small but present risk of malignant transformation. The primary management hazard is a failure to secure the airway promptly. The prognosis is excellent when the mass is completely excised, as was done in our case. The surgical mortality rate is 9%, but patients who are not treated are likely to die. [15] No recurrences or malignancies have been reported in infants.

From the Department of Otorhinolaryngology-Head and Neck Surgery (Dr. Shetty, Dr. Gupta, and Dr. Chary), the Department of Plastic and Reconstructive Surgery (Dr. Cherian), and the Department of Pathology (Dr. Shariff), St. John's Medical College Hospital, Bangalore, India.

References

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(6.) Talmi YP, Sadov R, Dulitzky F, et al. Teratoma of the mastoid region in a newborn. J Laryngol Otol 1988;102:1033-5.

(7.) Mudbhatkal NS, Kothare SN. Teratoma of soft palate: A case report. J Postgrad Med 1966;12:45-7.

(8.) Bras G, Butts D, Hoyte DA. Gliomatous teratoma of the tongue. Report of a case. Cancer 1969;24:1045-50.

(9.) Ruah CB, Cohen D, Sade J. Eustachian tube teratoma and its terminological correctness. J Laryngol Otol 1999;113:271-4.

(10.) Forrest AW, Carr SJ, Beckenham EJ. A middle ear teratoma causing acute airway obstruction. Int J Pediatr Otorhinolaryngol 1993;25:183-9.

(11.) Calcaterra T. Teratomas of the nasopharynx. Ann Otol Rhinol Laryngol 1969;78:165-71.

(12.) Gundry SR, Wesley JR, Klein MD, et al. Cervical teratomas in the newborn. J Pediatr Surg 1983;18:382-6.

(13.) Jordan RB, Gauderer MW. Cervical teratomas: An analysis. Literature review and proposed classification. J Pediatr Surg 1988;23:583-91.

(14.) Handler SD, Raney RB, Jr. Management of neoplasms of the head and neck in children. I. Benign tumors. Head Neck Surg 1981;3:395-405.

(15.) Hawkins DB, Park R. Teratoma of the pharynx and neck. Ann Otol Rhinol Laryngol 1972;81:848-53.

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