Pineal germinoma [Neurosurgery Wiki]

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====== Pineal germinoma ======
[[Pineal]] [[germinoma]] is a [[intracranial germinoma]] in the [[pineal region]].

===== Epidemiology =====
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 tumor]]s
((Schmalisch K, Pantazis G, Ebner FH, et al. Pineal germinoma with granulomatous reaction, often a pitfall in endoscopic biopsy. Report of two cases and review of the literature. Clin Neurol Neurosurg 2012;2013:741–5))
((Blakeley JO, Grossman SA. Management of pineal region tumors. Curr Treat Options Oncol 2006;2013:505–16)).

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. 

===== Clinical features =====
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.

===== Diagnosis =====

see [[Pineal germinoma diagnosis]].

====Differential 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.

===== Treatment =====
In general, pineal germinomas associated with hydrocephalus are commonly managed by endoscopic biopsy and ETV, followed by chemotherapy and/or radiotherapy
((Oppido PA, Fiorindi A, Benvenuti L, et al. Neuroendoscopic biopsy of ventricular tumors: a multicentric experience. Neurosurg Focus 2011. http://thejns.org/doi/full/10.3171/2011.1.FOCUS10326DOI))
((Talamonti G, D'Aliberti G, Debernardi A. Intracranial tumors: treatment with endoscopic neurosurgery. In: Hayat E, editor. ed. Tumors of the central nervous system. Berlin: Springer, in press. doi:10.1007/978-94-007-7217-5_19))
((Rogers SJ, Mosleh-Shirazi MA, Saran FH. Radiotherapy of localised intracranial germinoma: time to sever historical ties? Lancet Oncol 2005;2013:509–19))
((Choi BK, Cha SH, Song GS, et al. Recurrent intracranial germinoma along the endoscopic ventriculostomy tract. J Neurosurg (Pediatrics) 2007;2013:62–5)).

The non-surgical standard of care is cisplatin-based chemotherapy followed by focal radiotherapy
(( Nicholson JC, Punt J, Hale J, et al. Germ Cell Tumour Working Groups of the United Kingdom Children's Cancer Study Group and International Society of Paediatric Oncology: neurosurgical management of paediatric germ cell tumours of the central nervous system—a multi-disciplinary team approach for the new millennium. Br J Neurosurg 2002;2013:93–5))
((Souweidane MM, Krieger MD, Weiner HL, et al. Surgical management of primary central nervous system germ cell tumors. J Neurosurg Pediatrics 2010;2013:125–30))
((Rogers SJ, Mosleh-Shirazi MA, Saran FH. Radiotherapy of localised intracranial germinoma: time to sever historical ties? Lancet Oncol 2005;2013:509–19))
((Brown JH, Saran FH. Defining the optimal radiation therapy for secreting CNS germ cell tumors (SGCTS): a critical review of the literature. In: abstracts for the second International Symposium on central nervous system germ cell tumors. Neuro Oncol 2005;2013:513–33)).

===Radiation therapy===
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
((Brown JH, Saran FH. Defining the optimal radiation therapy for secreting CNS germ cell tumors (SGCTS): a critical review of the literature. In: abstracts for the second International Symposium on central nervous system germ cell tumors. Neuro Oncol 2005;2013:513–33)).

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
((Schmalisch K, Pantazis G, Ebner FH, et al. Pineal germinoma with granulomatous reaction, often a pitfall in endoscopic biopsy. Report of two cases and review of the literature. Clin Neurol Neurosurg 2012;2013:741–5))
((Mueller W, Schneider GH, Hoffmann KT, et al. Granulomatous tissue response in germinoma, a diagnostic pitfall in endoscopic biopsy. Neuropathology 2007;2013:127–32)).

====Prognosis====
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% 
((Jorsal T, Rørth M. Intracranial germ cell tumours: a review with special reference to endocrine manifestations. Acta Oncologica. 2012;51(1):3–9.)).

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

===== Case reports =====

[[Pineal Germinoma Case Reports]].