DOI: 10.1148/rg.244035164
RadioGraphics 2004;24:1165-1170
© RSNA, 2004
Best Cases from the AFIP
Congenital Intracranial Teratoma1
Bruce A. Sandow, MD,
Christopher E. Dory, MD,
Maria A. Aguiar, MD and
Alfred Z. Abuhamad, MD
1 From the Departments of Radiology (B.A.S., C.E.D.), Pediatrics (M.A.A.), Pathology (M.A.A.), and Obstetrics and Gynecology (A.Z.A.), Eastern Virginia Medical School, 825 Fairfax Ave, Suite 541, Norfolk, VA 23507. Received July 14, 2003; revision requested August 28 and received October 14; accepted October 16. Address correspondence to B.A.S. (e-mail: bsandow@infionline.net).
Index Terms: Brain neoplasms, in infants and children, 10.362 • Fetus, neoplasms, 856.313 • Teratoma, 10.362, 856.313
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History
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A 38-week-gestational-age male infant was born to a 32-year-old mother after an ill-defined intracranial mass and secondary obstructive hydrocephalus were diagnosed prenatally with ultrasound (US) and magnetic resonance (MR) imaging. He was delivered by elective cesarean section, required oxygen in the delivery room, and was then transferred to the neonatal intensive care unit for evaluation of hydrocephalus and respiratory distress. The infant required intubation and placement of bilateral ventriculoperitoneal shunts. During the first week of life, the patient was diagnosed with central diabetes insipidus and was given supplemental intravenous fluids to prevent dehydration. In addition, multiple pituitary hormone deficiencies were noted, which required treatment with hydrocortisone and levothyroxine. On the sixth day of life, the infant developed seizure activity and was treated with phenobarbital. Head circumference continued to rapidly increase, and follow-up computed tomography (CT) on day 15 revealed that the ventricles were partially decompressed and that the mass had significantly increased in size. Because of the poor prognosis, the parents agreed to withdraw ventilator support, and the infant died of respiratory failure 17 days after birth.
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Imaging Findings
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Prenatal US at 28 weeks gestation demonstrated an ill-defined echogenic mass at the level of the thalamus (Fig 1), which was the location of a cyst identified on a prior scan. No ventriculomegaly was present at that time. Biparietal diameter and head circumference were normal. Fetal MR imaging was performed at 30 weeks gestation by using half-Fourier rapid acquisition with relaxation enhancement (RARE) sequences to evaluate the intracranial mass and revealed an ill-defined iso- to hypointense 3 x 2 x 2-cm mass in the region of the hypothalamus (Fig 2).
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Figure 2a. T2-weighted MR images of the fetal brain at 30 weeks gestation. (a) Axial image shows that the mass (arrows) is iso- to hypointense and located in the region of the hypothalamus. The right temporal horn is partly effaced (arrowhead). (b) Sagittal image shows that the mass (arrows) is suprasellar or sellar. It effaces the third ventricle and extends anteriorly to the frontal cortex and posteriorly to the brainstem.
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Figure 2b. T2-weighted MR images of the fetal brain at 30 weeks gestation. (a) Axial image shows that the mass (arrows) is iso- to hypointense and located in the region of the hypothalamus. The right temporal horn is partly effaced (arrowhead). (b) Sagittal image shows that the mass (arrows) is suprasellar or sellar. It effaces the third ventricle and extends anteriorly to the frontal cortex and posteriorly to the brainstem.
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Postnatal cranial US 1 day after birth demonstrated a heterogeneous 6 x 5-cm suprasellar mass (Fig 3) with extension into both lateral ventricles and effacement of the third ventricle, resulting in moderate hydrocephalus. Postnatal MR imaging of the brain 1 day after birth revealed a heterogeneous 9 x 6 x 9-cm suprasellar mass with multiple low- and high-signal-intensity components on T1- and T2-weighted images (Fig 4a, 4b) and heterogeneous enhancement (Fig 4c). The mass filled much of the supratentorial compartment, displacing the brainstem posteriorly and attenuating the third ventricle, resulting in marked obstructive hydrocephalus.
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Figure 3. Postnatal coronal US image of the brain, obtained 1 day after birth, shows that the mass (arrows) is irregular and suprasellar. It is predominantly echogenic with cystic components and has shadowing echogenic foci, which are consistent with calcifications. Normal anatomic landmarks are replaced by the large mass, which occupies most of the intracranial compartment.
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Figure 4a. Postnatal MR images of the brain obtained 1 day after birth. (a) Sagittal T1-weighted image shows that the mass (arrows) is heterogeneous and suprasellar. It is predominantly iso- to hyperintense and has multiple low-signal-intensity cystic regions as well as numerous punctate high-signal-intensity foci, which probably represent calcifications. The mass fills much of the supratentorial compartment, causing posterior displacement of the brainstem (arrowhead). The third ventricle is attenuated, resulting in obstructive hydrocephalus. (b) Sagittal T2-weighted image shows that the mass (arrows) is heterogeneous and predominantly iso- to hypointense with multiple hyperintense cystic areas of varying sizes. (c) Axial contrast material-enhanced T1-weighted image shows heterogeneous enhancement of the mass (arrows), which is in the midline and effaces the third ventricle, resulting in marked hydrocephalus.
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Figure 4b. Postnatal MR images of the brain obtained 1 day after birth. (a) Sagittal T1-weighted image shows that the mass (arrows) is heterogeneous and suprasellar. It is predominantly iso- to hyperintense and has multiple low-signal-intensity cystic regions as well as numerous punctate high-signal-intensity foci, which probably represent calcifications. The mass fills much of the supratentorial compartment, causing posterior displacement of the brainstem (arrowhead). The third ventricle is attenuated, resulting in obstructive hydrocephalus. (b) Sagittal T2-weighted image shows that the mass (arrows) is heterogeneous and predominantly iso- to hypointense with multiple hyperintense cystic areas of varying sizes. (c) Axial contrast material-enhanced T1-weighted image shows heterogeneous enhancement of the mass (arrows), which is in the midline and effaces the third ventricle, resulting in marked hydrocephalus.
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Figure 4c. Postnatal MR images of the brain obtained 1 day after birth. (a) Sagittal T1-weighted image shows that the mass (arrows) is heterogeneous and suprasellar. It is predominantly iso- to hyperintense and has multiple low-signal-intensity cystic regions as well as numerous punctate high-signal-intensity foci, which probably represent calcifications. The mass fills much of the supratentorial compartment, causing posterior displacement of the brainstem (arrowhead). The third ventricle is attenuated, resulting in obstructive hydrocephalus. (b) Sagittal T2-weighted image shows that the mass (arrows) is heterogeneous and predominantly iso- to hypointense with multiple hyperintense cystic areas of varying sizes. (c) Axial contrast material-enhanced T1-weighted image shows heterogeneous enhancement of the mass (arrows), which is in the midline and effaces the third ventricle, resulting in marked hydrocephalus.
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Pathologic Evaluation
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Autopsy of the head was performed and revealed asymmetric enlargement of the head and an enlarged, bulging anterior fontanelle. On removal of the brain, the cerebral hemispheres were found to be edematous and fell apart to reveal a soft 10–12-cm midline mass (Fig 5a). It was not possible to determine the specific site of origin of the mass due to distortion and replacement of normal anatomic structures. The mass was predominantly solid, and sectioning showed areas of hemorrhage. Marked hydrocephalus was present, and there was evidence of brainstem herniation. Microscopic sections demonstrated a variety of tissues from all three germ layers (Fig 5b–5d), including immature as well as mature elements. The predominant tissue was neuroectodermal, in the form of neuroepithelial rosettes and tubules, resembling neuroblastoma. Mesodermally derived immature cartilage and primitive stroma were also common, and endodermally derived respiratory and enteric epithelium was present in the form of cystic structures. The diagnosis was immature teratoma.
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Figure 5a. Histopathologic photographs of the postmortem specimen. (a) Gross photograph shows the right cerebral hemisphere (anterior is to the left), the mass (arrows), and marked hydrocephalus. The mass is lobulated, tan to cream colored, and located in the midline. The cut surface of the mass demonstrates solid and cystic areas as well as evidence of hemorrhage. (b) Photomicrograph (original magnification, x100; hematoxylin-eosin stain) shows a variety of incompletely differentiated tissue components, including endodermally derived respiratory epithelium (arrow) and enteric epithelium (arrowhead), which form cystic and tubular structures. Also seen are mesodermal elements, including moderately hypercellular cartilage (*), bundles of smooth muscle, and a surrounding hypercellular stroma. (c) Photomicrograph (original magnification, x100; hematoxylin-eosin stain) shows ectodermally derived neuroepithelial rosettes (arrows), which mimic the developing neural tube. (d) High-power photomicrograph (original magnification, x400; hematoxylin-eosin stain) shows primitive neuroblastlike cells forming a neuroepithelial rosette (arrows), which is embedded in a hypercellular stroma suggestive of embryonic mesenchyme.
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Figure 5b. Histopathologic photographs of the postmortem specimen. (a) Gross photograph shows the right cerebral hemisphere (anterior is to the left), the mass (arrows), and marked hydrocephalus. The mass is lobulated, tan to cream colored, and located in the midline. The cut surface of the mass demonstrates solid and cystic areas as well as evidence of hemorrhage. (b) Photomicrograph (original magnification, x100; hematoxylin-eosin stain) shows a variety of incompletely differentiated tissue components, including endodermally derived respiratory epithelium (arrow) and enteric epithelium (arrowhead), which form cystic and tubular structures. Also seen are mesodermal elements, including moderately hypercellular cartilage (*), bundles of smooth muscle, and a surrounding hypercellular stroma. (c) Photomicrograph (original magnification, x100; hematoxylin-eosin stain) shows ectodermally derived neuroepithelial rosettes (arrows), which mimic the developing neural tube. (d) High-power photomicrograph (original magnification, x400; hematoxylin-eosin stain) shows primitive neuroblastlike cells forming a neuroepithelial rosette (arrows), which is embedded in a hypercellular stroma suggestive of embryonic mesenchyme.
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Figure 5c. Histopathologic photographs of the postmortem specimen. (a) Gross photograph shows the right cerebral hemisphere (anterior is to the left), the mass (arrows), and marked hydrocephalus. The mass is lobulated, tan to cream colored, and located in the midline. The cut surface of the mass demonstrates solid and cystic areas as well as evidence of hemorrhage. (b) Photomicrograph (original magnification, x100; hematoxylin-eosin stain) shows a variety of incompletely differentiated tissue components, including endodermally derived respiratory epithelium (arrow) and enteric epithelium (arrowhead), which form cystic and tubular structures. Also seen are mesodermal elements, including moderately hypercellular cartilage (*), bundles of smooth muscle, and a surrounding hypercellular stroma. (c) Photomicrograph (original magnification, x100; hematoxylin-eosin stain) shows ectodermally derived neuroepithelial rosettes (arrows), which mimic the developing neural tube. (d) High-power photomicrograph (original magnification, x400; hematoxylin-eosin stain) shows primitive neuroblastlike cells forming a neuroepithelial rosette (arrows), which is embedded in a hypercellular stroma suggestive of embryonic mesenchyme.
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Figure 5d. Histopathologic photographs of the postmortem specimen. (a) Gross photograph shows the right cerebral hemisphere (anterior is to the left), the mass (arrows), and marked hydrocephalus. The mass is lobulated, tan to cream colored, and located in the midline. The cut surface of the mass demonstrates solid and cystic areas as well as evidence of hemorrhage. (b) Photomicrograph (original magnification, x100; hematoxylin-eosin stain) shows a variety of incompletely differentiated tissue components, including endodermally derived respiratory epithelium (arrow) and enteric epithelium (arrowhead), which form cystic and tubular structures. Also seen are mesodermal elements, including moderately hypercellular cartilage (*), bundles of smooth muscle, and a surrounding hypercellular stroma. (c) Photomicrograph (original magnification, x100; hematoxylin-eosin stain) shows ectodermally derived neuroepithelial rosettes (arrows), which mimic the developing neural tube. (d) High-power photomicrograph (original magnification, x400; hematoxylin-eosin stain) shows primitive neuroblastlike cells forming a neuroepithelial rosette (arrows), which is embedded in a hypercellular stroma suggestive of embryonic mesenchyme.
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Discussion
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Congenital brain tumors, defined as those presenting within the first 60 days of life, are rare, representing only about 0.5%–1.9% of all childhood brain tumors (1–3). Teratomas constitute about one-third to one-half of these and are the most common neonatal brain tumor (4,5). Previous reports have described several forms of congenital intracranial teratoma, including massive tumors replacing the intracranial contents, smaller tumors producing hydrocephalus, large intracranial tumors extending into the orbit or neck, and incidentally discovered tumors in stillborn infants (4). Although it is often impossible to determine the exact site of origin, prenatally diagnosed intracranial teratomas are predominantly supratentorial, in contrast to their infratentorial location in older children (2,3). The prevalence of congenital intracranial teratomas is reported to be nearly equal in males and females in most series (5).
Histopathologic classification of teratomas includes mature, immature, and malignant varieties, but the malignant form is reportedly rare among congenital brain teratomas (5). Mature teratomas contain several varieties of adult tissue, while immature teratomas contain incompletely differentiated components resembling fetal tissues (6). Both mature and immature forms usually contain tissues from all three germ layers, including skeletal muscle, cartilage, bone, bronchial epithelium, gut epithelium, and neural tissue. However, teratomas can occasionally be derived from a single germ layer if they show histologically divergent differentiation, such as skin and neural tissue (7). Almost all childhood teratomas contain derivatives of all three germ layers, and head and neck teratomas frequently have a predominance of both mature and immature neuroectodermal elements (7). The etiology and pathogenesis of extragonadal teratomas remain unclear, but they are thought to arise from misplaced primordial germ cells, which become embedded in or near midline structures in the head, mediastinum, or sacrococcygeal region (7).
Prenatal US has been used in diagnosing intracranial teratoma in more than 40 reported cases to date (1,8–12). The diagnosis is made between 20 and 40 weeks gestation and is usually suspected because of a sudden increase in uterine size resulting from tumor growth and polyhydramnios due to impaired fetal swallowing (4). The most common initial sonographic finding in the fetus is macrocephaly, and additional features typically include gross distortion or replacement of normal brain tissue by an echogenic mass with multiple cystic components, as well as hydrocephalus secondary to obstruction (2,4). At prenatal US, the diagnosis of teratoma should be considered for a complex intracranial mass with calcifications. However, the differential diagnosis for a sonographically diagnosed intracranial mass also includes astrocytoma, ependymoma, craniopharyngioma, choroid plexus cyst, and hemorrhage.
In the present case, prenatal sonography at 28 weeks gestation showed an ill-defined echogenic mass without an apparent cystic component (Fig 1), although a cyst had been noted on a prior scan, likely reflecting early developmental changes in the histologic composition of the teratoma. Subsequent prenatal and postnatal US demonstrated a solid and cystic midline mass with calcifications (Fig 3), similar to the findings in previous reports (2,4). The cystic elements correspond to epithelium-lined cystic structures seen at pathologic evaluation (Fig 5b), and the calcifications likely reflect the presence of calcified regions of cartilage. Although calcifications were not observed on these prenatal scans, occasional reports have suggested the presence of calcifications in intracranial teratomas at prenatal sonography (9,10).
Several recent case reports have described the use of fetal MR imaging between 25 and 36 weeks gestation in helping confirm the diagnosis of intracranial teratoma, and the typical appearance is a large, heterogeneous mass with cystic components on T1- and T2-weighted images (8,13–17), with no apparent difference between mature and immature teratomas. Similar features have been described at postnatal MR imaging of mature and immature intracranial teratomas (18–20). Although MR imaging is insensitive for detecting small calcifications, CT has demonstrated regions of calcification in most teratomas (7). As the MR imaging features of teratomas are relatively nonspecific, the differential diagnosis of congenital supratentorial tumors should also include primitive neuroectodermal tumor, astrocytoma, ependymoma, glioma, craniopharyngioma, and choroid plexus papilloma.
In the present case, prenatal MR imaging performed at 30 weeks gestation showed an ill-defined suprasellar mass without discrete cystic elements (Fig 2), in contrast to the solid and cystic appearance described previously (8,13–17). This atypical appearance likely reflects histologic changes in tissue composition during early tumor development. To our knowledge, this is the first reported prenatal MR imaging description of early development in an immature intracranial teratoma. At postnatal MR imaging, a more typical heterogeneous mass with solid and cystic components was apparent (Fig 4), similar to previous descriptions of intracranial teratomas (18–20).
The patient in the present case died of respiratory failure 17 days after birth, likely as a result of brainstem compression by the enlarging intracranial teratoma. This outcome was not unexpected, as the prognosis in cases of congenital intracranial teratoma is extremely poor, with a mortality rate around 90% (4,8). In the majority of reported cases, the outcome has been either stillbirth or death shortly after birth (1). Despite the benign histopathologic features of most intracranial teratomas, tumor growth is rapid and the tumor frequently replaces all normal brain tissue, resulting in massive craniomegaly (2). The majority of cases have been delivered by cesarean section because of abnormally large head size and difficult delivery (8). However, in several cases, vaginal delivery has been possible, occasionally with prior cranial decompression (1). The few reported attempts at total or subtotal tumor resection have had poor outcomes, although there are rare reports of prolonged survival up to 3.5 years following resection of smaller tumors (8,10).
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Footnotes
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Editor's Note.—Everyone who has taken the course in radiologic pathology at the Armed Forces Institute of Pathology (AFIP) remembers bringing beautifully illustrated cases for accession to the Institute. In recent years, the staff of the Department of Radiologic Pathology has judged the "best cases" by organ system, and recognition is given to the winners on the last day of the class. With each issue of RadioGraphics, one or more of these cases are published, written by the winning resident. Radiologic-pathologic correlation is emphasized, and the causes of the imaging signs of various diseases are illustrated.
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References
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History
Imaging Findings
