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Choristomas represent normal tissue in an abnormal location. Lingual choristomas are rare cystic masses that are lined with a variety of heterotopic tissue. Because they are typically lined with respiratory and/or gastric epithelium, they have also been termed foregut duplication cysts. Portions of the resected mass (Figure 3) were dermoid in appearance, with a cystic lining composed of orthokeratinzing stratified squamous epithelium of uniform thickness. Scattered sebaceous glands and hair follicles were observed within the cyst wall. Other portions of the specimen demonstrated ciliated epithelium lining the cyst wall, a finding that is more consistent with the classic description of lingual choristoma. To our knowledge, only 2 other cases of lingual choristomas with dermal appendages and respiratory epithelium have been described in the literature.1 Herein, we report a third case, with the addition of keratinization as a cyst-lining feature. The pathogenesis of lingual choristomas remains unclear; however, it has been suggested that the cysts may arise during embryogenesis, when heterotopic groups of cells are trapped within the developing tongue.2,3 The split notochord theory holds that a disturbance in the development of the notochord and surrounding structures accounts for the misplaced segments of epithelium.2
Pathology Quiz Case 2: Diagnosis. Arch Otolaryngol Head Neck Surg. 2007;133(6):623. doi:10.1001/archotol.133.6.623
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