Epidermal Choristoma Presenting as an Enlarging Tongue Mass

             http://austinpublishinggroup.com/otolaryngology/fulltext/ajo-v4-id1092.php

Epidermalchoristoma is an extremely rare entity characterized by histologically normal-appearing skin with associated hair follicles, sebaceous glands, and adnexa in an abnormal location. When localized to the oral cavity, this lesion presents most-commonly as an asymptomatic hyper pigmented macule or patch on the lingual dorsum of a male patient. Herein, we present an unusual case of an epidermal choristoma presenting as an acutely infected mass of the deep anterior tongue in a teenage female.

Choristomas are a developmental abnormality characterized by the proliferation of histologically normal tissue in an ectopic location. Choristomas of the head and neck have been noted in the tongue floor of mouth nasopharynx pharynx hypopharynx  and submandibular regions and are clinically important lesions as they may present as a cause of airway obstruction or feeding/ swallowing difficulties, especially in the pediatric population. In the oral cavity, heterotopic tissue consistent with choristoma has been reported with otherwise histologically-normal salivary gland, cartilaginous, osseous, thyroid, sebaceous, glial, gastric mucosa, and epidermal tissues although cartilaginous, osseous, and lingual thyroid choristomas are relatively more common. “Epidermal choristoma” or “cutaneous choristoma” is defined by the presence of stratified squamous epithelium (epidermis) with associated adnexal structures including sebaceous glands, apocrine glands, and hair follicles. Epidermal choristoma is a very rare lesion of the oral cavity, with only five reported examples in the literature to date. All reported cases have occurred in males, with the majority presenting as a hyper pigmented macule or plaque (80%), most commonly of the dorsal tongue. Follicular choristoma appear to be a related lesion defined by pigmented epidermis, sebaceous glands, sweat glands, and mature hair follicles with the additional finding of keratin-containing cysts. To date, there are two cases of follicular choristoma reported in the English language literature. Herein, we report a unique example of an epidermal choristoma presenting as an infected tongue mass in a female teenager.

A 14-year-old female with no significant medical history presented to the emergency room with two days of tongue swelling, painful swallowing, fever, and difficulty speaking. The patient complained of some difficulty in breathing. Physical exam revealed moderate swelling and tenderness of the dorsum of the tongue with a 1cm right paramedian mass just anterior to the circumvallate papillae. Of note, there was no pigmentation changes or ulceration reported. The patient did not have stridor.