Objective: Congenital intracranial tumors are very rare and only account for 0.5-1.5% of all childhood brain tumors. The most common type of these tumors present at birth is teratomas, which represent 0.5% of all intracranial tumors. Most teratomas are midline tumors located predominantly in the sellar and pineal regions. In this study, we report a neonatal intracranial immature teratoma at the lateral ventricle because of its rare location.
Case Report: A 3-day-old female neonate presented with a history of irritability, vomiting, and recurrent generalized clonic seizures since birth. A head computed tomographic scan and magnetic resonance imaging disclosed a large tumor filling the right lateral ventricle and extending into the ipsilateral posterior fossa. With right parieto-occipital craniotomy, large grayish-white lobulated vascular mass was encountered and total removal of tumor was performed. Histological examination revealed the diagnosis of immature teratoma.
Conclusion: The prognosis of congenital intracranial immature teratoma is usually poor because the lesions are extensive when they are identified. Prenatal ultrasonography is necessary for the prenatal diagnosis. Fetal magnetic resonance imaging should be made for the evaluation of intracranial tumor. If the tumor is detected before the 24 week of gestation, termination of the pregnancy should be considered. [Neurol Res 2005; 27: 53-56]
Keywords: Intracranial; lateral ventricle; neonate; surgery; teratoma; tumor
Neoplasms developing in the intrauterine period and detected either at birth or in the first month of life represent 2.5% of all tumors in the pediatric age group1,2. Congenital intracranial tumors are even rarer and only account for 0.5-1.5% of all childhood brain tumors1'3'4. The most common type of these tumors present at birth are teratomas1' , which comprise between 28.8 and 50.0% of central nervous system (CNS) tumors1 and intracranial teratomas represent only about 0.5% of all intracranial tumors6.
Immature teratoma is a tumor that contains diverse cell lineages that retain an embryonal character and display phenotypic differentiation attributed to the three classical germ layers7'8. Teratomas are commonly located in or near the midline6. Immature teratomas arising within the lateral ventricle are rare neoplasms.
We present a rare case of a giant immature teratoma arising in the lateral ventricle in a 3-day-old girl. Histological, radiological and clinical features of this tumor are discussed with the base of the literature.
A 3-day-old girl admitted to Çukurova University Hospital with a history of irritability, vomiting and recurrent generalized clonic seizures since birth. She was born to a 30-year-old mother at term by Cesarean section with acute fetal distress as the fourth live child. The regular prenatal examination and ultrasonography have not been done.
On physical examination, she was 3050 g and body length was 45 cm. The head circumference was 34.5 cm. The anterior fontanel was noted to be open and to be 2 × 2 cm, with bulbing. She had split cranial sutures and prominent scalp veins. No other congenital anomaly was observed.
On neurological examination, she had spontaneous eye opening, a feeble cry to painful stimuli. She had normal tone and symmetrical movements in the extremities.
Transcranial ultrasound examination revealed a heterogeneous intracranial mass in the right parieto-occipital area. Cranial computed tomography (CT) revealed a giant, lobulated right parietal region tumor protruding into the fourth ventricle, and hydrocephalus due to the tumor. The tumor contained solid parts, cyst formation, some small calcifications and fatty tissue in very low density. Considerable enhancement was seen after contrast administration. The mass occupied nearly half of the brain and shifted the midline to the left (Figure 1a,b). Cranial magnetic resonance imaging (MRI) showed a giant, cystic tumor filling the right lateral ventricle and extending into the ipsilateral posterior fossa. MRI also demonstrated mixed signals derived from different tissue confirming the tumor. Heterogeneous enhancement was seen after infusion of contrast medium (Figure 2a,b). The presumptive diagnosis was a teratoma.
A right parieto-occipital craniotomy was performed. After the dura was opened, a large grayish-white lobulated vascular lesion occupying the parieto-occipital area and lateral ventricle was exposed. After debulking of the tumor, the extension of the cystic mass into the posterior fossa was pulled out and the tumor was radically excised macroscopically.
Different parts of the tumor were extensively sampled and formalin fixed paraffin-embedded tissue was processed. sections were stained with hematoxylin and eosin (H&E). Histological examination of the lesion showed a mixture of embryonal and adult tissues derived from all three germ layers. The tumor, composed predominantly of primitive neuroepithelial tissue, was admixed with immature and differentiating mesenchymal and epithelial structures, and immature cartilage. No foci of germinoma, endodermal sinus, choriocarcinoma or embryonal carcinoma tissue were present (Figure 3a,b). A CT scan obtained immediately after the operation demonstrated that tumor resection and pneumocephalus (Figure 4). The patient was discharged 1 week after surgery with a normal cry and better spontaneous movements.
On follow-up after 2 weeks, the child was admitted to our department with pneumonia and she was followed in the Intensive Care Unit, but she got worse and died.
Germ cell tumors comprise 0.4-3.1% of all intracranial tumors and teratoma constitutes 9-30% of them8-10. The origin and pathogenesis of these particular tumors is still a matter of debate6'11. Jennings ef a/.12, in an excellent review of the natural history and pathogenesis of intracranial germ cell tumors, concluded that a developmental etiology for these tumors is the most suitable, favored by their natural history, the histological features, and the specific sites of development in the CNS. This theory is supported by Sano13.
Teratomas are best defined as benign tumors that contain representative elements of the three germinal layers: ectoderm, mesoderm and endoderm6'11. Teratomas have been classified according to the histological appearance of the different cell types and tissues within the lesion. Thus, they are classified into three groups: (1) mature teratomas, consisting of full differentiated tissues representing the three germinal layers; (2) immature teratomas, which are the most frequent, are characterized by the presence of cellular populations that retain their embryological features and include more primitive components derived from all or any of the three germinal layers; and (3) malignant teratomas, which can be mature or immature teratomas and have malignant components represented by germ cells (atypical teratoma) and embryonal undifferentiated cells5'6'14. Our patient falls into the second category. Considering all age groups, teratomas predominate in males. However, in the neonatal period, the incidence is higher in females as in our case15'16.
Congenital teratomas are most commonly found in the sacrococcygeal region, but they also occur in the neck, mediastinum and, as in our case, within the intracranial region1'2. Being midline lesions, these tumors usually occupy the pineal gland, suprasellar region, quadrigeminal plate, walls of the third ventricle and cerebellar vermis15'17. Other non-midline sites that have been described are basal ganglia, cerebellopontine angle and cavernous sinus 9. Their locations in the lateral ventricle, as in the present case, are rare and probably related with the choroid plexus15'17. Rarely, they have been described in the fourth ventricle2. Fetal intracranial teratomas, including the presented case, are usually more than 5 cm in diameter and frequently fill the cranial cavity completely2. Because of the huge size of most tumors, it is often impossible to locate the exact site of origin2.
Congenital intracranial teratoma also can be classified according to radiographie feature into three main subgroups: (1) a diffuse, intracranial form often associated with extensive destruction and occasionally with replacement of the entire brain tissue and sometimes associated with hydrocephalus; (2) a more localized, less extensive form in which survival ranges from 0.5 h to 9 weeks; (3) a massive variant extending through the cranial base into the face and neck region20. Our patient falls into the first category. These lesions may be solid or cystic. Cyst formation is a common occurrence in the neonatal intracranial teratomas. In the review of the Lipman ef a/.21 multiple cystic spaces within a solid tumor were described in nine of 10 patients. This radiologie and macroscopic appearance was also present in our patient.
Prenatal ultrasonography permits early antenatal detection of these tumors. Most tumors so far diagnosed in utero have not been detected prior to the third trimester. To our knowledge, the earliest diagnosis so far described in the literature stems from the 22nd gestational week2,5. Our patient's mother did not have the chance of fetal ultrasonography. Differential diagnosis of congenital intracranial tumors by ultrasonography is difficult and often impossible2 . MRI is a valuable complementary tool when prenatal ultrasonography is incomplete, doubtful or limited. The advantages of MRI center around its ability to reveal abnormalities that may not be detectable or only poorly seen on CT. CT is superior to MRI in the detection of calcifications, but MRI, through is multiple imaging planes and absence of bone artifact, better delineates the extent of the tumor, especially within the posterior fossa23. Recently, several reports have revealed that immature teratomas without yolk sac tumor and/or embryonal carcinoma elements contain alphafetoprotein (AFP)-positive cells8'25'26. This is considered on intrauterine diagnosis of intracranial immature teratomas.
The clinical management of these lesions is unclear, due in part to their low incidence and to an incomplete understanding of their natural history. Surgical excision may be curative, but it is only limited to the smaller benign intracranial teratomas '27. We suggest that complete removal of the teratoma should be attempted. If total surgical removal cannot be achieved, combined radiotherapy and chemotherapy is recommended, especially for immature teratomas28"30.
The prognosis is usually fatal because the lesions are usually extensive when they are identified, and invasive growth of the tumors and the destruction of regular cerebral structures2'23'31. The 1-year survival rate among infants with intracranial teratomas only amounted to 7.2% in one large series5 and the condition is usually accompanied by developmental delay even if surgery is successful2. Therefore, if such a tumor is detected before the 24th week of gestation, termination of the pregnancy should be considered2.
In spite of the advances in neonatal surgery, fetal intracranial teratoma still remains a fatal condition. Most of the infants reported in the literature either have severe neurological sequel or die20'22'32'33. However, with improved methods of diagnosis and management, selected cases may be treated with more success in the future.
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Tahsin Erman, Iskender A. Göçer, Seyda Erdogan*, Yasemin Günes[dagger], Metin Tuna and Suzan Zorludemir*
Department of Neurosurgery, * Department of Pathology and [dagger] Department of Anesthesiology, Çukurova University, School of Medicine, Adana, Turkey
Correspondence and reprint requests to: Tahsin Erman M.D., Çukurova University School of Medicine, Department of Neurosurgery, Balcali-Adana/01330, Turkey, [firstname.lastname@example.org] Accepted for publication February 2004.
Copyright Maney Publishing Jan 2005
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