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CASE REPORT |
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| Year : 2006 | Volume
: 1
| Issue : 1 | Page : 33-35 |
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Germinoma of fourth ventricle: A case report and review of literature
Anand B Kakani1, Vikram S Karmarkar1, Chandrashekhar E Deopujari1, Rajan M Shah1, Nadir E Bharucha2, Girish Muzumdar3
1 Departments of Neurosurgery, Bombay Hospital and Institute of Medical Sciences, India
2 Departments of Neurology, Bombay Hospital and Institute of Medical Sciences, India
3 Departments of Pathology, Bombay Hospital and Institute of Medical Sciences, India
Correspondence Address:
Anand B Kakani
Room No. 126B, MRC Building, Bombay Hospital, 12, New Marine Lines, Mumbai - 400 020
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/1817-1745.22947
How to cite this article:
Kakani AB, Karmarkar VS, Deopujari CE, Shah RM, Bharucha NE, Muzumdar G. Germinoma of fourth ventricle: A case report and review of literature. J Pediatr Neurosci 2006;1:33-5 |
| Introduction | |  |
Intracranial Germ Cell Tumors are relatively rare. They appear mainly in the Pineal and Suprasellar regions in children, adolescents and young adults.[1] Primary intracranial Germ Cell Tumour in the fourth ventricle is an extremely rare entity with only few such cases on record. One such case, that of a young girl, with the attendant difficulties in diagnosis and management, is reported.
| Case Report | | |
This sixteen year old female, on treatment for miliary pulmonary tuberculosis, presented with a two and half month history of difficulty in swallowing, imbalance while walking, slurring of speech and vertigo. On examination, she was emaciated, had severe bilateral cerebellar signs. Palatal movements were weak and the gag reflex was depressed.
A non-contrast CT Brain done during initial investigations was reported normal. The possibility of basal meningitis was suggested, though not confirmed on a CSF examination. An MRI Brain done when she came to us showed a mildly hyperintense heterogeneously enhancing lesion in the floor of the fourth ventricle, protruding out of the foramen of Magendie [Figure - 1]. The pineal, suprasellar regions as well as the ventricular system were normal.. Differential diagnoses of fourth ventricular ependymoma, brain stem glioma, medulloblastoma or a tuberculoma were considered.
Due to her poor general condition and miliary tuberculosis, she was started on steroids and antituberculous chemotherapy was intensified. This produced a marginal improvement over the next two weeks. However, she developed Diabetes Insipidus and then rapidly, impaired sensorium, quadriparesis and respiratory insufficiency, for which she was intubated and mechanically ventilated. After stabilizing her, an urgent CT scan was done to look for increase in ventricular size, which was negative, but showed a nodular growth in the region of the pituitary stalk [Figure - 2]. At this point a germinoma with metastasis or some other disseminated tumor was suspected and serum tumor markers were sent. After controlling her Diabetes Insipidus with appropriate fluid management and DDAVP, she was taken up for surgery. A sub-occipital craniotomy with subtotal excision of the lesion was done. Frozen section and histopathology revealed it to be a Germinoma. Immunohistochemistry was positive for Placental Alkaline Phosphatase (PLAP). [Figure - 3]
Serum tumor markers showed a beta hCG level of 6.5 mIU/ml while AFP was undetectable. Though her sensorium and limb power improved, she could not be weaned off the ventilator in the postoperative period and died of a sudden cardiac arrest on the twelfth postoperative day.
| Discussion | | |
Intracranial Germ Cell Tumors (GCT's) are relatively rare neoplasms with a variable incidence worldwide. The western literature quotes an incidence of 0.3-0.5% of all primary intracranial neoplasms and approximately 3% of tumors encountered in children and adolescents.[1] Series from Japan and Taiwan show that these neoplasms are far more common in the Asian continent, accounting for 2-5% of all primary intracranial neoplasm and for 9-15% of pediatric primary intracranial neoplasms.[1] The biological basis of this variation in tumour incidence is not known. These tumors have a peak incidence during the second decade of life and are frequently encountered in childhood rather than in adult life.
Intracranial Germ Cell Tumor is a diagnosis that includes Germinoma, Embryonal carcinoma, Yolk sac tumor, Teratomas and the mixed variety. Germinomas are the most common variety of GCT's accounting for 60% of all intracranial Germ Cell Tumors.[2] The germinomas commonly arise in the midline, like other extragonadal GCT's, typically in the pineal region and the suprasellar compartment, including the area of third ventricle and the hypothalamus. Other locations include the basal ganglia and the thalamus, the cerebral hemispheres and cerebellopontine angle.
The fourth ventricle or the brainstem as the primary site of GCT's is rare and very few cases are reported including one in the midbrain,[3],[4] one in the midbrain and pons and six cases in the medulla oblongata.[5],[6] Multifocal involvement of the pineal region and suprasellar compartment simultaneously or sequentially has also been reported.[7] Rare examples of intramedullary spinal germinomas[8] and cerebellar germinomas[9] have also been reported.
Of the six cases of Germ Cell Tumors in the Medulla Oblongata, five patients were female and one patient had Kleinfelter's syndrome (47/XXY). All of these tumors were histologically verified to be germinomas. As compared to germinomas in the Pineal and Suprasellar regions, which are more common in boys, germinomas in the medulla oblongata show a striking female preponderance. All the reported cases were comparatively older than those with primary intracranial Germ Cell Tumors at other sites (14-32 years). The clinical presentation in four of these cases was involvement of the lower cranial nerves, one patient presented with predominantly ocular symptoms and one case had manifested with numbness in the extremities. Our patient had presented with features of lower cranial nerve involvement.
There is no consensus on the treatment of the Intracranial Germ Cell Tumors. The traditional approach for pineal and suprasellar region germinomas was that of surgical excision followed by adjuvant radiation with or without chemotherapy.[2] However the marked efficacy of radiation and chemotherapy, with the initial response approaching almost 100%, has led to the departure from this traditional approach. Stereotactic biopsy can be used to verify the histological diagnosis. In all the reported cases of germinomas of the Medulla Oblongata various combinations of chemotherapy and radiotherapy with surgical biopsy at least, have been used with good long-term outcome and recurrence free survival (follow up of 2 months - 9 years). Radiotherapy has been recommended to be an effective treatment for Intracranial Germinomas at other sites.[10]
| Conclusion | | |
Germinoma in the fourth ventricle involving the medulla oblongata is a rare entity but is now being increasingly recognized. A high index of suspicion is necessary for timely diagnosis. It should be included in the differential diagnosis of the fourth ventricular and medulla oblongata masses, especially in young females. CSF, serum tumor marker studies and biopsy may aid early recognition of such tumors. The prognosis can be favorable if these tumors are diagnosed early, as chemo-radiation is very effective.
| References | | |
| 1. | Rosenblum MK, Matsutini M, Van Meir EG. CNS Germ Cell Tumors. In: Kleihues P, Cavenee WK, edited WHO Classification of tumors. Pathology and Genetics: Tumors of the Nervous System. IARC Press: Lyon; 2000. p. 208-14.  |
| 2. | Maher CO, Raffel C. Neurosurgical treatment of Brain tumors in children. In: Piatt JH edited Pediatr Clin North Am 2004;51:327-57. [PUBMED] [FULLTEXT] |
| 3. | Amor SB, Siddiqui K, Baessa S. Primary midbrain germinoma. Br J Neurosurg 2004;18:310-3. |
| 4. | Matsumoto K, Tabuchi A, Tamesa N, Nakashima H, Ohmoto T. Primary intracranial germinoma involving the midbrain. Clin Neurol Neurosurg 1998;100:292-5. [PUBMED] [FULLTEXT] |
| 5. | Nakajima H, Iwai Y, Yamanaka K, Yasui T, Kishi H. Primary intracranial germinoma in the Medulla Oblongata. Surg Neurol 2000:53;448-51. |
| 6. | Yen PS, Chou AS, Chen CJ, Chuang HL, Scott RM. Primary Medulla Oblongata germinoma: case report and review of literature. J Neurooncol 2003;62:339-42. [PUBMED] [FULLTEXT] |
| 7. | Aguila LA, Samuel MC, Janet WB. Primary intracranial germinoma presenting as lower cranial nerve involvement:Case report and review of literature. Neurosurgery 1984;14:475-9. |
| 8. | Yasunobu I, Katsuyoshi M, Hiroyasu S, Masyoshi K. Intramedullary spinal cord germinoma: ase report and review of the literature. Neurosurgery 1996;38:187-94. |
| 9. | Maiuri F, Cappabianca P, Del Basso De Caro M, Esposito F, de Divitiis E. Primary cerebellar germinomas of the posterior fossa. Br J Neurosurg 2004;18:284-9. [PUBMED] [FULLTEXT] |
| 10. | Hoffman JH, Otsubu H, Hendrick B, Humphreys RP, Drake JM, Becker LE, et al. Intracranial germ cell tumors in children. J Neurosurg 1991;74:545-51. |
Figures
[Figure - 1], [Figure - 2], [Figure - 3]
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