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| Year : 1999 | Volume
: 47
| Issue : 4 | Page : 321-3 |
Mixed germ cell tumour of the pineal region : a case report.
Tandon N, Chopra R, Ghoshal S, Singh P, Sharma BS
Departments of Radiotherapy and Oncology, Neurosurgery and Pathology, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India.
Correspondence Address:
Departments of Radiotherapy and Oncology, Neurosurgery and Pathology, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India.
An intracranial mixed germ cell tumour with germinoma and teratoma components is reported. The patient presented with parinaud's syndrome and precocious puberty. The treatment involved partial surgical debulking followed by whole brain radiotherapy (4500 cGY in 25 fraction over 5 weeks) and chemotherapy (consisting of cisplatin and etoposide). Post treatment MRI showed no residual lesion. The controversies in the management are discussed.
How to cite this article:
Tandon N, Chopra R, Ghoshal S, Singh P, Sharma B S. Mixed germ cell tumour of the pineal region : a case report. Neurol India 1999;47:321 |
Primary intracranial germ cell tumours are rare in patients of less than 20 year of age and constitute less than 2% of all intracranial malignancies.[1] Similar to their systemic counterparts, these tumours commonly involve the midline structures i.e. pineal and suprasellar region.[2],[3] lntracranial germ cell tumours are classified in the same way as their gonadal counter parts i.e. germinoma, nongerminomatous germ cell tumours and mixed germ cell tumours. Mixed germ cell tumours commonly have germinoma as one of its histological subtypes.[4] The other component of the group can be a choriocarcinoma, embryonal cell tumour, endodermal sinus tumour or a teratoma. The optimal treatment remains controversial. The combination of germinoma with teratoma is rare.[5] We present a case of germinoma with mature teratoma of the pineal region, successfully managed with combination of surgery, radiotherapy and chemotherapy.
[TAG:2]Case report[/TAG:2]
A 14 year old male presented with headache, diplopia, vomiting and excessive hair on the body for about two months. General physical examination revealed extensive growth of hair on the body with full grown beard, moustaches and pubic hairs. His secondary sexual characters were well developed for his age. Neurological examination revealed papilloedema and parinaud's syndrome. Cranial CT scan showed a hyperdense mass lesion, homogeneously enhancing after injection of intravenous contrast, in the pineal region with hydrocephalus. MRI of the head showed a well defined mass in the pineal region compressing the tectal plate, midbrain and the posterior third ventricle in the sagittal T1 image [Figure. 1]. Coronal T2 images revealed heterogeneously hyperintense lesion with areas of bright signal. MRI of the spine was normal. The tumour was partially excised through the transtentorial route after occipital craniotomy. A ventriculoperitoneal shunt was inserted later on, for the relief of hydrocephalus.
The histopathological examination of the excised mass showed a mixed germ cell tumour containing both germinoma and teratoma elements [Figure. 2]. The germinoma component had appearance of classical type with cells having vesicular nuclei with prominent nucleoli, abundant cytoplasm, arranged in islands separated by fibrous septae. The septae had moderate infiltrate of lymphomononuclear cells. Focal areas of nuclear atypia and high mitotic rate (approx.2-3/HPF) were present. The teratoma component contained myxoid areas of primitive mesenchyme and many squamous epithelial nests with evidence of keratinisation in some areas. No other germ cell component was present. Immunostaining for beta human chorionic gonadotrophin (HCG) and alpha foetoprotein (AFP) was negative. CSF was negative for malignant cells. CSF and serum were also negative for tumour markers. Postoperatively, he was treated with whole brain radiotherapy in the dose of 4500 cGY in 25 fraction over 5 weeks. Follow-up MRI showed a small residual enhancing mass in the pineal region. He received chemotherapy consisting of cisplatinum in dose of 20 mg/m2 for 5 days and etoposide 100 mg/m2 on days 1,3,5. This cycle was repeated every three weeks for six such cycles. The follow up MRI showed no residual growth [Figure. 3].
Twelve percent of all intracranial germ cells tumours are mixed tumours.[1],[5],[6],[7] Combination of germinoma and mature teratoma is very rare, accounting for 4% of these cases.[5] The most common presenting features of tumours of pineal region include hydrocephalus and parinaud syndromes.[8],[9] Precocious puberty is a rare symptom and is associated with less than 10% of all pineal germ cell tumours.[10] CT and MRI have an important role in documenting the extent of disease. The germinomas are homogenous and show isointensity on T1 and hypointensity on T2 images while teratomas show a more heterogenous signal because of its various components.[11] Radiology of mixed tumours shows a considerable overlap and has not been well documented. Our case showed relatively homogenous intensity and intense enhancement on contrast administration.
In the past, because of high postoperative mortality in the tumours of pineal region, the patients were often empirically treated with radiation[12] Advances in microsurgical techniques have now rendered the pineal region more accessible and surgery can be performed safely. Surgical debulking or at least a histopathological confirmation is recommended.[13],[14] The surgery also helps in relieving hydrocephalus; however, ventriculoperitoneal shunt may still be required. AFP and Beta - HCG are secreted by some mixed and non germinomatous germ cell tumours in serum and CSF.[15] This may add to diagnosis and monitoring of therapeutic response. Our patient had a mature teratoma, hence, these markers were negative.
Germinomas are exquisitely radio-sensitive with 5 year survival rates reaching 65-95%.[4] However, the optimal dose remains to be defined. Patients treated below 4500 cGy have shown high local relapse rate.[6] The optimal treatment volume is also controversial and varies from whole brain or ventricular irradiation to localized irradiation to the tumour site.[4],[12],[16] The use of craniospinal irradiation is also debatable. The incidence of spinal dissemination ranges from 3-37%.[8] This can be proved by CSF analysis or MRI of the spine for tumour deposits. Due to significant morbidity, the prophylactic spinal radiation is not recommended by most authors and is reserved for proved cases only.
Systemic germ cell tumours and their brain metastases are quite chemosensitive,[4] especially for platinum containing compounds. Keeping this in view, a number of newer trails have been designed to give chemotherapy in a neoadjuvant setting. It is to be followed later by radiotherapy.[4] In the present case chemotherapy was added later as the follow-up MRI after radiotherapy showed a small enhancing residual mass in pineal region. The optimum management of intracranial germ cell tumour is still evolving. Further studies are needed to evaluate the benefits of various modalities of treatment in this excellent responding group of brain tumours. It is concluded that histological confirmation in pineal tumours is desirable as it discloses the nature of components in mixed germ cell tumours and paves way for further treatment.
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