Congenital Intracranial Teratoma with Massive Macrocephaly and Skull Rupture

Abstract

Objective: Congenital intracranial tumors are rare and only account for 0.5–1.5% of all pediatric brain tumors. Teratoma is the most frequently encountered intracranial tumor at birth. Massive congenital intracranial teratoma is an extremely rare neoplasm with a poor prognosis. They grow rapidly and cause extensive destruction in the brain. Herein we report a massive intracranial teratoma causing skull rupture. Case Report: A fetus with a congenital intracranial teratoma presenting with a disproportionately enlarged head at 25 weeks of gestation is presented. Since it was the first admission of the mother to a medical expert for a prenatal examination, there was noprevious follow-up data. Prenatal ultrasonography demonstrated a huge, heterogeneous intracranial mass, and midline structures and ventricles could not be observed. No heartbeat was detected. Autopsy was perforated, and histopathologic examination of the samples taken from the intracranial mass revealed an immature teratoma. Conclusion: Although congenital intracranial teratomas are rare, they may reach enormous sizes. Regular follow-up of the fetus may lead to early diagnosis of immature intracranial teratomas and prevent the mother from having further complications either due to intrauterine fetal death orpsychological trauma of giving birth to a heavily malformed baby.

Frederik Ruysch (1638–1731): Historical perspective and contemporary analysis of his teratological legacy

“…Considering the consecutive expulsion of the child, the placenta and the tumorous tissue, we think that the latter originated from the sac‐like protrusion of the child's head, which ruptured during birth (hence the roughly edged opening), a mechanism that has been reported several times [e.g., Washburne et al, 1995; Bolat et al, 2008]. Subsequently, its contents were expelled in the uterine cavity.…”

“…The combination of multi-structured tissue lumps and several limb-like appendages, the size of which is not in accordance with the actual gestational age, is a strong indication for fetiform teratoma. Considering the consecutive expulsion of the child, the placenta and the tumorous tissue, we think that the latter originated from the sac-like protrusion of the child's head, which ruptured during birth (hence the roughly edged opening), a mechanism that has been reported several times [e.g., Washburne et al, 1995;Bolat et al, 2008]. Subsequently, its contents were expelled in the uterine cavity.…”

Acting on macrocephaly in the neonatal period: an illustrative case of congenital teratoma

“…These lesions only represent 0.5–1.5% of all paediatric brain tumours, whereby the supratentorial compartment is affected more commonly than the posterior fossa 3 4. Teratoma represents the most frequently encountered congenital brain tumour and has a poor prognosis with death usually occurring not long after birth 5 6.…”

Rapid Growth of Congenital Diffuse Brain Tumor Considered to Be Teratoma -Case Report-

“…The histological diagnosis is only achieved at autopsy in many cases following the failure of endoscopic biopsy. 2,5,6,19) Here we present a case of massive congenital brain tumor showing rapid growth which was most probably teratoma but endoscopic biopsy failed to provide definitive identification.…”

“…19) Massive congenital intracranial teratoma is a rare neoplasm with poor prognosis because the tumor tissue has already replaced most of the brain tissue before first identification on fetal ultrasonography. [1][2][3][4][5]7,10,13,15,19) Surgical treatment is seldom undertaken because of the high frequency of fatal and postnatal death. The surgical treatment of a large intracranial tumor with partial destruction and replacement of brain structure also represents an ethical dilemma.…”