Case report

A 28-year-old woman, gravida 4, para 2, was referred to our department at 36 weeks’ gestation after an ultrasound examination performed at another center had shown fetal hydrocephaly and a suspected brain tumor Ultrasound revealed a biparietal diameter (BPD) of 15.5 cm, which was grossly enlarged, and a solid tumor originating from the cranium; Real-time scanning showed the tumor to be hyperechogenic with discrete hypoechogenic regions. There were also acoustic shadows interpreted as calcifications. The lateral ventricles were dilated and no normal brain architecture was identifiable. At 38 weeks of gestation, a Cesarean section was performed, owing to disproportion. At 39 + 7 weeks of gestation, a Cesarean section was performed, owing to disproportion. A girl weighing 4450 g was delivered. Apgar scores were 8 and 9 at 1 and 5 min, respectively. The infant was then immediately referred to the neonatal intensive care unit. Physical examination revealed an extreme macrocephaly with bulging and tense anterior fontanel and split cranial sutures. No associated anomalies had been found.

Discussion

The incidence of brain tumors of congenital origin has been estimated at 0.34 per million live births,approximately 26–50% of which are teratomas. Other tumors that have been found prenatally with an intracranial location include: meningeal sarcoma, craniopharyngioma, lipoma, glioblastoma, astrocytoma, oligodendroglioma, cavernous hemangioma and undifferentiated malignant tumor The progress of ultrasound imaging has led to safer and more accurate evaluation of this fetal pathological condition. Other imaging techniques such as computed tomography magnetic resonance imaging (MRI) and echo planar imaging (EPI) may improve diagnosis in the fetus. In most cases, the diagnosis of a fetal intracranial tumor does not alter the prognosis, which is uniformly dismal. There are, however, two exceptions: lipomas (usually associated with agenesis of the corpus callosum) and choroid plexus papillomasPoor prognosis is associated, in most cases, with a large size of tumor, The histological type of the tumor may also influence the prognosis, They found a relatively good prognosis in choroid plexus papillomas and the next best with astrocytomas . In contrast, the survival rate for teratomas was only 7.2%. Our collected data confirm this. As mentioned, lipomas had a good outcomeThe ultrasound features of teratomas, astrocytomas and craniopharyngiomas have a similar appearance: a complex mass distorting the cerebral architecture, possibly associated with macrocephaly, hydrocephaly and calcifications. Intracranial lipomas are well-defined echogenic masses, usually located in the midline and possibly associated with agenesis of the corpus callosum. Polyhydramnios is also frequently associated with intracranial tumors.Once the diagnosis of an intracranial tumor has been established, a thorough search for other abnormalities should be made. In addition, fetal echocardiography and determination of karyotype are recommended, since the presence of other fetal anomalies would have an important impact on the prognosis and thus the management of pregnancyObstetric management was dependent on gestational age at the time of diagnosis. Radical extirpation was possible The overall outcome after resection of tumors was poor. Although long-term survivors were reported Less is known about the efficacy or the risks of additional therapeutic strategies such as chemotherapy or radiation therapy.

Conclusion

Increasing use of diagnostic ultrasound in pregnancy has improved the prenatal diagnosis of congenital intracranial tumors. The sonographic features of this lesion are cranial enlargement and gross distortion of the normal cerebral anatomy by a tumor mass with internal echoes of varying distribution and intensity. Since these may have an important impact on the prognosis, a thorough search for secondary fetal anomalies should be made directly after diagnosis of an intracranial tumor. The overall prognosis for intracranial tumors is poor, depending on the size and histological type of the tumor. Lipomas are the only tumors associated with a good fetal outcome Obstetric management is dependent on gestational age at the time of diagnosis In spite of recent advances in neonatal surgery and the availability of additional therapeutic strategies, all cases with fetal intracranial tumors (except lipomas) still have a very poor prognosis.

References

1-Alvarez M, Chitkara U, Lynch L, Mehalek KE, Heller D, Berkowitz RL. Prenatal diagnosis of fetal brain tumors. Fetal Ther 1987;2:203–8

2-Buetow PC, Smirniotopoulos JG, Done S. Congenital brain tumors: a review of 45 cases. Am J Roentgenol 1990;155: 587–93

3-Takaku A, Kodama N, Ohara H, Hori S. Brain tumor in newborn babies. Child’s Brain 1978;4:365–75

4-Lipman SP, Pretorius DH, Rumack CM, Manco-Johnson ML. Fetal intracranial teratoma: US diagnosis of three cases and a review of the literature. Radiology 1985;157:491–4

5-Pretorius DH, Russ PD, Rumack CM, Manco-Johnson ML. Diagnosis of brain neuropathology in utero. Neuroradiology 1986;28:386–97

6-Ferreira J, Eviatar L, Schneider S, Grossman R. Prenatal diagnosis of intracranial teratoma. Prolonged survival after resection of a malignant teratoma diagnosed prenatally by ultrasound: a case report and literature review. Pediatr Neurosurg 1993;19:84–8

7-Snyder JL, Lustig-Gillman I, Milio L, Morris M, Pardes JG, Young BK. Antenatal ultrasound diagnosis of an intracranial neoplasm (craniopharyngioma). J Clin Ultrasound 1986;14: 304–6

8-Doeren M, Tercanli S, Gulotta F, Holzgreve W. Prenatal diagnosis of a highly undifferentiated brain tumor – a case report and review of the literature. Prenat Diagn 1997;17:967–71

9-Crombleholme TM, D’Alton M, Cendron M, Alman B, Goldberg MD, Klauber GT, Cohen A, Heilman C, Lewis M, Harris BH. Prenatal diagnosis and the pediatric surgeon: the impact of prenatal consultation on perinatal management. J Pediatr Surg 1996;31:156–63

10-Dolkart LA, Balcom RJ, Eisinger G. Intracranial teratoma: prolonged neonatal survival after prenatal diagnosis. Am J Obstet Gynecol 1990;162:768–9

11-Thickman D, Mintz M, Mennuti M, Kressel HY. MR imaging of cerebral abnormalities in utero. J Comput Assist Tomogr 1984;8:1058–61