Congenital Intracranial Teratoma Yin-Hsiu Chien, MD*, Po-Nien Tsao, MD*, Wang-Tso Lee, MD*, Shinn-Feng Peng, MD † , and Kuo-Inn Tsou Yau, MD* Congenital intracranial teratoma is a rare disease. A fetus with a congenital intracranial teratoma present- ing with a disproportionately enlarged head at 27 weeks gestation is presented. Prenatal ultrasonography and fetal magnetic resonance imaging demonstrate a huge, heterogenous intracranial mass in the left supra- tentorial region, with the left cerebral hemisphere being compressed and flattened. The infant died of respiratory failure within 24 hours of birth at 28 weeks gestation. On postmortem examination the histologic report revealed an immature teratoma. Fetal MRI is helpful in the prenatal diagnosis and evaluation of intracranial tumor. © 2000 by Elsevier Science Inc. All rights reserved. Chien Y-H, Tsao P-N, Lee W-T, Peng S-F, Tsou Yau K-I. Congenital intracranial teratoma. Pediatr Neurol 2000;22: 72-74. Introduction Congenital intracranial teratoma is a rare disease. Be- cause the lesions are usually extensive when they are identified, the prognosis is usually poor. In one large series the 1-year survival rate was 7.2% [1]. Fetal intracranial teratoma is often associated with macrocephaly, bulging anterior fontanel, hydrocephalus, or polyhydramnios. Prenatal ultrasonography permits early antenatal detection of these tumors. However, com- puted tomography (CT) and magnetic resonance imaging (MRI) are the main tools of the diagnostic evaluation [2]. A fetus with a congenital intracranial tumor diagnosed by prenatal ultrasonography and fetal MRI is presented. The immature teratoma was confirmed by pathologic findings on postmortem examination. Case Report This 2-day-old preterm male infant was born to a 37-year-old gravida 6, para 4, aborta 2 mother at 28 weeks gestation via cesarean section. The regular prenatal examination was unremarkable. However, prenatal ultrasonography at 27 weeks gestation revealed a disproportionately enlarged head. An intracranial tumorlike mass without obviously normal intracranial structures was evident on ultrasonography (Fig 1). The largest transverse diameter of the head at that time was 11.3 cm (the 95 th percentile is 9.8 cm). This complex solid mass with multiple cystlike echogenicity was located in the left supratentorial region. Fetal MRI was performed and revealed a heterogeneous intraventricular or periventricu- lar mass lesion with multiple cystlike areas in the left cerebral hemi- sphere (Fig 2). Furthermore, the left cerebral hemisphere was com- pressed, but the brainstem and the cerebellum were intact. The congenital intracranial teratoma or choroid plexus papilloma was impressed. Because of the severe macrocephaly, an elective cesarean section was performed at 28 weeks gestation. The infant was born with Apgar scores of 1, 1, and 5 at 1, 5, and 10 minutes, respectively. His body weight was 2,856 gm, his body length was 47 cm, and his head circumference was 46.6 cm at birth. Physical examination revealed a huge head with wide, bulging fontanels and a bossing forehead. He was extremely hypotonic with widely separated cranial sutures. No other congenital anomaly was observed. Transcranial ultrasound examination revealed a heterogeneous intracranial mass without normal brain structure. Because of the poor prognosis, his parents refused any aggressive treatment. He died at 1 day of age. On postmortem examination the brain weighed 500 gm. A huge and well-defined tumor measuring 10  8  7 cm in size was present in the left cerebral hemisphere. The right cerebral hemisphere was compressed and flattened. Marked hydrocephalus of the bilateral ventricle was evident. The brainstem and the cerebellum were normal. Microscopic examination disclosed an immature teratoma mainly composed of the neural tube and other immature neural tissues. Immature skin, cartilage, and bone were also observed. Discussion Congenital intracranial teratoma is a rare disease. A massive congenital intracranial teratoma was first de- scribed in 1864 [3]. Congenital brain tumors were defined as tumors presenting within 60 days after birth and represented only 0.5-1.9% of all pediatric brain tumors [4]. Weyerts et al. [5] reviewed 20 cases diagnosed in utero, but only one survived, with severe intellectual impairment, after complete resection [5]. The progress made in ultra- sound instrumentation and routine ultrasound scanning From the Departments of *Pediatrics and † Medical Imaging; National Taiwan University Hospital; Taipei, Taiwan. Communications should be addressed to: Dr. Tsou Yau; Department of Pediatrics; National Taiwan University Hospital; No. 7, Chung-Shan South Road; Taipei 100, Taiwan. Received May 25, 1999; accepted August 10, 1999. 72 PEDIATRIC NEUROLOGY Vol. 22 No. 1 © 2000 by Elsevier Science Inc. All rights reserved. PII S0887-8994(99)00103-4 ● 0887-8994/00/$20.00