Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature. Teratomas of the Head and Neck Introduction Teratomas of the head and neck are interesting because of their obscure origin, bizarre microscopic appearance, unpredictable behavior and often dramatic clinical presentation. Since teratomas of the head and neck frequently present with respiratory distress, the otolaryngologist should be aware of their clinical features, natural history, pathology and principles of management.
A teratoma is a true neoplasm composed of an assemblage of tissue often alien to the site in which it arises. The components tend to be arranged in haphazard manner with asynchronous maturation of the involved tissue types. They grow independently of the host and can destroy adjacent tissue. In the late 19th century, Dr. Arnold derived the classification system for teratoid malformations. The system consists of four groups: dermoid cysts, teratoid cysts, true teratomas and epignathi. True teratomas, the focus of this discussion, are also composed of all three germ cell layers. However, these cells are differentiated to the level of specific tissue. General Teratomas occur in 1 of 4000 live births, demonstrating a female predominance, and represent 9% of all pediatric head and neck neoplasms. Teratomas are most commonly located in the sacrococcygeal region, followed by the ovaries, testes, anterior mediastinum, retroperitoneum, and finally the head and neck, which account for less than 5%. There are several theories regarding the histogenesis of teratoma formation. Ahfeld first proposed the included twin theory in the late 1800's. Schwalbe later suggested that teratomas arise, during embryogenesis, from displaced germ cells during migration to the genital ridge. The most popular theory speculates that teratomas arise from totipotential embryonic tissues that are somehow displaced during ontogeny. For head and neck teratomas, the theory postulates that embryonic tissue in proximity to the primitive streak and notochord somehow loses its governing influence over the totipotential cells, allowing unorganized differentiation. This is presumably due to alterations in cellular membrane chemistry. Cervical Teratomas The reported incidence of cervical teratomas ranges from 2.3% to 9.3% of the total. Only 217 cases of cervical teratomas have been reported in the world literature, of which 86 were in females and 70 in males. All races have been represented and there is no obvious genetic predisposition. Ninety percent of cases are diagnosed in the pediatric population; however, an adult form does exist. Associated malformations are rare but have been described. Only one case of metastatic disease has been reported. Seventy-five percent of cervical teratomas present during the neonatal period. The mass is often midline with lateral extension and can measure more than 10 cm in diameter. Palpation usually reveals a firm, mobile, multilobular, cystic mass with well-defined margins. Respiratory and obstetrical complications are frequent. The respiratory symptoms range from mild positional dyspnea to complete apnea. Obstetrical complications include preterm labor, polyhydramnios and birth dystocia. The polyhydramnios is thought to arise from interference of the mass with normal fetal swallowing and breathing. The larger masses are more likely to cause complications, but small teratomas can grow explosively, causing precipitous decompensation. Timely and accurate diagnosis is critical so therapy is not delayed. Evaluation should precede after the airway is secured. This may require endotracheal intubation, bronchoscopy, or tracheotomy. Plain radiographs demonstrate calcifications in 16% of cases and is diagnostic when present. Tracheal deviation is seen in more than 50% of cases. Ultrasonography can be performed at the bedside without sedation or radiation, and can reveal a complex neck mass with cystic and solid components. CT reveals differing densities and scattered calcifications. In addition to defining the location and extension of the tumor, delineation of distorted normal tissues and evaluation of cervical lymph nodes and the mediastinum is also possible. The role for MRI has not been described in the evaluation of cervical teratoma. Because of the potential for rapid growth of the cystic component and airway compromise, the treatment of cervical teratomas is immediate excision. Teratomas occurring in the adult population differ in nature from pediatric disease. Twenty-three cases of cervical teratomas have been reported in adults who ranged in age from 18 to 85 years. Most cases occurred in patients under 30 years of age. Seven of the teratomas were benign without any immature elements. None recurred after resection. Sixteen cases were malignant, demonstrating aggressive growth prior to excision. Fourteen of these cases reported local recurrence or metastatic disease and ten patients died within two years of diagnosis. The remaining six patients were lost to follow-up and presumed dead of the disease. Nasopharynx The second most common head and neck site for teratoma formation is the nasopharynx. First described in medical literature by Dr. Ahfeld in 1874, only 30 cases have been reported. A marked female predominance of six to one has been cited. The significance of immature elements in the surgical specimen is unclear since malignancy and recurrence have not been reported after local excision of nasopharyngeal teratomas. The clinical presentation depends on the size and location of the mass. Clinical manifestations can range from failure to thrive and mild positional dyspnea to severe airway compromise. Nasopharyngeal teratomas may be sessile or pedunculated, and have been reported to protrude from the mouth and nares. Diagnosis is usually made early in infancy and rarely after 2 years of age. Teratomas of the nasopharynx are often associated with cranial structural anomalies. Palatal fissures are common and hemicrania and anencephaly have been reported. It is theorized that the teratoma physically interferes with the normal fusion of embryonic tissues in the early developmental period. Prior to evaluation the airway must be secured. This can be accomplished with an oral airway, oral intubation, or, if necessary, a tracheotomy. Plain radiographs and sonography are sometimes helpful. Encephalocele is probably the most important preoperative diagnosis to make. Computerized tomography helps define the extent of the lesion as well as intracranial extension. Despite CT scanning, teratomas have been misdiagnosed for menigoencephaloceles and interoperative aspiration has been recommended, with chemical evaluation of the fluid. Recently FNA has been described. Differentiation from glial tumors often requires excisional biopsy since nasopharyngeal teratomas can be predominantly composed of neural tissue. Conclusion Teratomas of the head and neck are rare neoplasms, usually presenting in the neonatal period. Though most tumors are benign, they are often malignant with regard to their location. They can enlarge rapidly, so prompt airway intervention is important. The diagnosis needs to be made promptly, usually with the aid of computerized tomography, followed by excisional biopsy. Bibliography Abemayor E, Newman A, Bergstrom L, Dudley J, Magidson JG, Ljung BM. Teratomas of the head and neck in childhood. Laryngoscope 1984;94:1489-1492. Alter AD, Cove JK. Congenital nasopharyngeal teratoma: report of a case and review of the literature. J Pediatr Surg 1987;22:179-181. Badrawy R, Fahmy SA, Taha AM. Teratoid tumours of the nasopharynx. J Laryngol Otol 1973;87:795-799. Barkovich AJ, Vandermarck P, Edwards MSB, Cogen PH. Congenital nasal masses: CT and MR imaging features in 16 cases. AJNR 1991;12:105-116. Berry CL, Keeling J, Hilton C. 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Teratoma of the soft palate with multifocal cyst formation in the tongue and the brain. Arch Otorhinolaryngol 1986;243:320-323. Todd DW, Votava HJ, Telander RL, Shoemaker CT. Giant epignathus: a case report. Minn Med 1991;74:27-28. Touran T, Applebaum H, Frost DB, Richardson R, Taber P, Rowland J. Congenital metastatic cervical teratoma: diagnostic and management considerations. J Pediatr Surg 1989;24:21-23. Ward RF, April M. Teratomas of the head and neck. Otolaryngol Clin North Am 1989;22:621-629. Weaver RG, Meyerhoff WL, Gates GA. Teratomas of the head and neck. Surg Forum 1976;27:539-542. Wiatrak BJ, Myer CM III, Bratcher GO. Report of a nasopharyngeal teratoma evaluated with magnetic resonance imaging. Otolaryngol Head Neck Surg 1990;102:186-190.
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