Giant Epignathus Teratoma Discovered at Birth: A Case Report and 7-Year Follow-Up

Abstract

Teratomas are tumors composed by tissues derived from the three germ cell layers, and they are relatively uncommon in head and neck. The term epignathus has been applied to teratomas from the oropharynx. This paper reports the case of a giant epignathus teratoma discovered at birth, which was successfully managed and followed up for 7 years. A newborn boy presented a polypoid tumor mass exteriorizing through the mouth over a length of 9 cm, with some surface areas resembling skin and others exhibiting hair. Computed tomography showed that the mass arose deep from the left hemiface. Alpha-fetoprotein (AFP) levels were high (316,000 ng/mL). Surgery was performed and microscopic analysis confirmed the diagnosis of mature teratoma. Because of residual tumor and high AFP levels, the patient was submitted to chemotherapy, resulting in complete regression of the lesion and normalization of AFP levels. Surgical repair of a cleft palate was performed at 5 years of age. At 7 years of age, the patient was in good general health and showed no clinical signs of recurrence. Although epignathus is a rare condition, it should be diagnosed in the fetus as early as possible. Prenatal care provides unquestionable benefits, providing the early diagnosis of anomalies that can jeopardize the life of the fetus and contributing to the indication of cases that require treatment before birth. G i a n t E p i g n a t h u s T e r a t o m a D i s c o v e r e d a t B i r t h : A C a s e R e p o r t a n d 7 -Ye a r F o l l o w -U p

Neonatal epignathus in the oral and pharyngeal regions: A case report

“…Surgical resection is the only effective way to treat teratomas . The published report of clinical findings in patients with giant epignathus is shown in Table . In the present case, although partial resection of the tumor by pediatric surgeons was made as a first step, this was unavoidable because the tumor was oppressing her face.…”

“…8 The published report of clinical findings in patients with giant epignathus is shown in Table 1. 2,3,6,11,[13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28] In the present case, although partial resection of the tumor by pediatric surgeons was made as a first step, this was unavoidable because the tumor was oppressing her face. It had been difficult to approach the tumor in the pharyngeal region before resection of the tumor protruding from the left nostril.…”

An autopsy case of epignathus (immature teratoma of the soft palate) with intracranial extension but without brain invasion: case report and literature review

“…Carvalho et al [13] reported a seven-year follow-up case that also involved an epignathus determined to be a mature teratoma, with no intracranial extension. Carvalho’s case was exceptional for two reasons.…”

Mature teratoma in a neonate presenting as an intraoral cystic lesion: A case report

“…The surgical repair of cleft palate was performed at 5 years of age. At 7 years of age, the patient was in good general health and showed no clinical signs of recurrence [5]. In our case, the teratoma was diagnosed antenatally and excised at birth.…”