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A teratoma (pleural: teratomata) is a germ cell tumor derived from pluripotential cells and made up of elements of different types of tissue from one or more of the three germ cell layers.  Most often found in the ovary or testis, the rare teratoma of the head and neck region arises primarily from Rathke's pouch remnants of the sphenoid bone region, from the lateral neck, and from the tongue. Rathke's pouch teratomas may extend into the mouth through a cleft palate. Although typically congenital, the teratoma is a true neoplasm of multiple tissue types foreign to the site from which it arises. Tissues derived from different embryonic germ layers are the rule rather than the exception.

This tumor varies considerably in the differentiation and maturation of its involved tissues, with some lesions containing fingers, teeth, jawbone or diminutive skeletons, while others demonstrate no more than an admixture of various tissue types with no attempt at maturation or structural development. Most paraoral cases are cystic and relatively undifferentiated. Oral lesions have, however, been known to contain tissues from all parts of the body, including brain, bone, cartilage, skin, lung, and the gastrointestinal tract.

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Clinical Features

Pathology and Differential Diagnosis

The various tissue types found in a teratoma for the most part are mature, although full differentiation is often lacking. The tissues are randomly admixed one with another, showing little or no correlation with their normal anatomic relationships. The lesion is typically encapsulated and well demarcated from the surrounding normal tissues. There may or may not be a fibrovascular background stroma separating the different tissue types.

The keratin-filled dermoid cyst of the oral floor midline has abortive sebaceous glands, perhaps even hair follicles, in its walls. Many authorities consider this to be a teratoma, going so far as to call such a lesion a teratoid cyst and equating it with the ovarian dermoid cyst. It is best, however, to refer to such a lesion as a dermoid cyst.

Malignant teratomas do occur, usually with only a single component demonstrating dysplastic changes. When a rhabdomyoblastic component is seen, a variety of heterologous or mixed tumors must be ruled out, and when multiple components appear malignant, terms such as malignant mesenchymoma and malignant ectomesenchymoma may be applied.

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Treatment and Prognosis

Treatment of a teratoma consists of conservative surgical removal, a procedure which often requires finesse and delicacy because of the close proximity to important anatomic structures of the head and neck region.  With conservative removal, occasional recurrence is to be expected, especially when portions of the teratoma must be left in place in order to preserve normal anatomic structures. Careful and long-term follow-up is recommended. Malignant teratoma is treated according to its most prominent malignant component, usually by radical surgery, with or without radiotherapy. Rarely, a benign teratoma has been reported to transform into malignancy, especially carcinoma.

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References (Chronologic Order)

Note: General references can be found by clicking on that topic to the left.

Batsakis JG, Littler ER, Oberman HA. Teratomas of the neck: a clinicopathological appraisal. Arch Otolaryngol 1964: 79:619-625.

Gorlin RJ. Developmental anomalies of the face and oral structures. In: Gorlin RJ, Goldman HM. Thoma's oral pathology, vol. 1, 6th edition. St. Louis; C.V. Mosby; 1970:21-95.

Kountakis SE, Minotti AM, Maillard A, Steinberg CM. Teratomas of the head and neck. Am J Otolaryngol Head Neck Surg 1994: 15:292-296.

McKiernan DC, Koay B, Vinayak B, et al. A case of submandibular teratoma. J Laryngol Otol 1995; 109:992-994.

Biglioli F, Gianni AB, Difrancesco A. Congenital teratoma of the cheek - report of a case. Internal J Oral Maxillofac Surg 1996; 25:208-209.

McMahon MJ, Chescheir NC, Kuller JA, et al. Perinatal management of a lingual teratoma. Obstet Gynecol 1996; 87:848-851.

Kuhn JJ, Schoem SR, Warnock GR. Squamous cell carcinoma arising in a benign teratoma of the maxilla. Otolaryngol Head Neck Surg 1996; 114:447-452.

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