Huge nasopharyngeal teratoma with a cleft palate and bifid tongue in a patient with pierre robin syndrome.

PURPOSE: Nasopharyngeal teratoma is a very rare neoplasm that develops on the oronasal cavity as an expanding and cavity-filling lesion. We present a rare case of nasopharyngeal teratoma associated with a cleft palate and bifid tongue in a patient with Pierre Robin syndrome.

METHODS

A preterm male neonate of 33 weeks and 6 days was referred to our department. A protruding solid mass with approximately 7.0 × 4.0-cm size arising from the vomer and combined cleft palate and tongue covered with pinkish skin with lanugo were observed. In addition, there was a bandlike tissue connecting vomer and sublingual area, which divided and restricted the anterior portion of the tongue.

RESULTS

An early surgical intervention was decided because of problems with airways and nutrition caused by the mass. The mass was completely removed from the vomer. The histologic examination of the mass was consistent with mature teratoma. After his first operation, he was finally diagnosed with the Pierre Robin syndrome. After 4 months, a tongue reduction was conducted for macroglossia, and after 9 months, remnant teratoma excision and a palate repair were conducted using 2-flap palatoplasty.

CONCLUSIONS

A nasopharyngeal teratoma is a rare case and induces a cleft palate and bifid tongue in a preterm infant, leading to severe airway and nutritional problems. A teratoma interferes with the fusion of embryonic tissues in the early developmental period, in particular, with the palatal fusion severely prevented if the teratoma is accompanied by a Pierre Robin syndrome such as in this case.