Massive Intracranial Immature Teratoma with Extracranial Extension into Oral Cavity, Nose, and Neck

Abstract

We present a case of congenital massive intracranial teratoma with extracranial extension into oral cavity, nose, and neck diagnosed by antenatal ultrasonography at 25 weeks of gestation. The fetus was delivered by cesarean section because of massive fetal head size and severe maternal pregnancy-induced hypertension. At necropsy the tumor was found to be an immature teratoma, with no recognizable normal brain tissue. An additional finding was hepatomegaly secondary to extensive extramedullary hematopoiesis. Karyotyping of both the fetus and the teratoma revealed a normal female chromosomal composition: 46,XX.

Vaginal Delivery Using Cranioclasia following Prenatal Diagnosis of a Giant Fetal Intracranial Teratoma

“…Rupture of the extremely thin skull has been reported several times [4][5][6][7][8][9] . Nonetheless, several authors have used encephalocentesis to avoid cesareans [3,9,17]. In our case, a caesarean appeared inappropriate for several reasons: in view of the fetal death we preferred to avoid creating a uterine scar, …”

“…Of brain tumors present at birth, 29-50% are teratomas [2] . Three principal forms are described: a major form, as here, which entirely replaces normal cerebral anatomic structures, a much smaller teratoma that is complicated by hydrocephaly, and a form with extracerebral extension to any combination of the eye socket, mouth, nose, and neck [3] .…”

“…In the literature review by Wakai et al [8] , 14% of cases included various malformations, such as anomalies of the heart and urinary tract, cleft palate, and hydramnios due to a fetal swallowing disorder. Congestive hepatomegaly due to increased hematopoiesis has been found at autopsy in some cases [3][4][5][6][7][8][9] . Fetal hydrops is frequent, due to high-output cardiac failure associated with the arteriovenous shunting of the large tumor [10] .…”

No chromosomal imbalances detected by comparative genomic hybridisation in a case of fetal immature teratoma

“…Chromosomal analyses were performed in 17 cases, most of them after amniocentesis [7]. Only 3 of these teratomas had a pathologic karyotype affecting both fetus and tumour [3,11,16], while in 5 studies a normal diploid karyotype was found in fetus and tumour [1,5,12,13,17]. In the remaining 9 cases karyotyping was performed on the fetus but not on tumour material [7].…”

Congenital Brain Tumors

“…They typically involve the midline structures such as pineal region, the hypothalamic area, quadrigeminal plate, and the suprasellar region. 2,7,17 In the case when the site of origin can be determined, the most common primary sites are the cerebral hemispheres, followed by the third ventricle and the pineal region. 18 The radiological appearance of teratomas depends on the contents of the tumor.…”