Diagnosis and Management of The Congenital Cranial Teratomas: Report of Four Cases and Review

Abstract

Congenital cranial teratomas are a well-recognized but infrequent entity and are usually characterized by complete loss of the normal intracranial architecture. They contain tissues from all three germ layers. This review presents the clinical features, diagnostic methods and management of cranial teratomas based on our experience and the literature. Material and methods: Four pathologically proven cases of congenital cranial teratomas were reviewed. One case was diagnosed prenatally, and the others were diagnosed postnatally. While CT and MRI had been performed in all patients, ultrasonography was only performed in one patient. Craniofacial mass, macrocephalus and/or hydrocephalus were the prominent findings. Localized neurological deficits were not observed even with very large tumors. In all patients, the mass was totally removed, and a ventriculoperitoneal shunt was performed in two patients who had hydrocephalus. Histological examination demonstrated a variety of tissues from all three germ layers. All three forms of teratomas were observed. There was no operative mortality and morbidity, but two patients died at 2 and 3 years after the surgery. Conclusion: Teratomas can be diagnosed early with antenatal ultrasound. They are detected postnatally in the setting of hydrocephalus and macrocephaly. CT and MRI show a multiloculated, heterogeneous solid-cystic mass with both lipids and calcification. The prognosis is poor, with death usually occurring shortly after birth, although there are rare reports of prolonged survival up to 3.5 years following tumor resection. Early diagnosis is important, and it is necessary for appropriate family counseling regarding the morbidity and mortality of fetuses diagnosed with tumors.