Pineal germinoma

Pineal germinoma is a intracranial germinoma in the pineal region.

There is a marked male predominance with a M:F of ~13:1. Most patients are 20 years or younger at the time of diagnosis.

It is the most common tumour of the pineal region but accounts for less than 1% of all intracranial tumors ^{1) 2)}.

Pineal germinomas are the most common tumour of the pineal region accounting for ~50% of all tumours, and the majority (~80%) of intracranial germ cell tumours.

They can result in mass effect and compression of the tectal plate leading to obstructive hydrocephalus and Parinaud syndrome.

Patients with pineal germinoma present with clinical features of hypothalamic-pituitary axis dysfunction.

see Pineal germinoma diagnosis.

pineal parenchymal tumours

pineocytoma

pineal parenchymal tumour with intermediate differentiation

papillary tumour of the pineal region

pineoblastoma

germ cell tumours

pineal germinoma: most common (~ 50% of all tumours)

pineal embryonal carcinoma

pineal choriocarcinoma

pineal yolk sac carcinoma: endodermal sinus tumour

pineal teratoma

astrocytoma of pineal gland

pineal metastasis

pineal cyst.

meningioma near pineal region

other rare entities

cavernoma in pineal region

aneurysm in pineal region

If invasive, a tectal plate mass may be difficult to distinguish from a pineal mass.

In general, pineal germinomas associated with hydrocephalus are commonly managed by endoscopic biopsy and ETV, followed by chemotherapy and/or radiotherapy ^{3) 4) 5) 6)}.

The non-surgical standard of care is cisplatin-based chemotherapy followed by focal radiotherapy ^{7) 8) 9) 10)}.

The optimal radiotherapeutic management still remains controversial.

For localised intracranial lesions, no differences would be evident in relapse rates or patterns of relapse when treating with localised radiotherapy alone compared with craniospinal irradiation ¹¹⁾.

It has been reported that the response to radiation therapy would be slower in granulomatous than in non-granulomatous germinomas, but the prognoses of these subtypes remain unclear owing to their rare occurrence ^{12) 13)}.

Primary germinoma of the central nervous system carries a good prognosis because of their radiosensitivity. Recurrence is rare and extraneural metastases are even more unusual.

The prognosis following pineal germinoma is very good with a reported 5-year survival rate above 90% ¹⁴⁾.

The most common tumors to metastasize via ventriculoperitoneal shunt are germinoma of the pineal gland and medulloblastoma.

Pineal Germinoma Case Reports.