Extragonadal germ cell tumors of the head and neck region: review of 16 cases

Abstract

During the period from 1928 to 1982, 16 children were treated or seen in consultation at the Children's Hospital Medical Center for extragonadal germ cell tumors of the head and neck region. Collectively, these tumors accounted for only 5 per cent of all benign and malignant germ cell tumors. Fourteen tumors were diagnosed in newborns and were classified as pure teratomas, either mature or immature. Six of these tumors were located in the cervical region, three presented within superficial facial structures, two were retro-orbital, and three arose within oropharyngeal or nasopharyngeal tissues. There were three endodermal sinus tumors, one of which appeared in an 11-month-old child who had undergone incomplete resection of an oropharyngeal teratoma as a newborn. Endodermal sinus tumors presented within the oropharynx, nasopharynx, and floor of the mouth and affected children at older ages (6 to 11 months). The overall prognosis for infantile teratomas of the head and neck region (exclusive of brain and spinal cord) is excellent, despite the presence of immature elements; however, tumor-related deaths can result from large unresectable primary tumors. The prognosis for children with endodermal sinus tumors remains guarded, with successful management depending on early diagnosis and aggressive adjuvant therapy.