Pineocytoma

Dr Bruno Di Muzio and A.Prof Frank Gaillard et al.

Pineocytomas are a relatively benign pineal parenchymal tumour that have a relatively
good prognosis.

On imaging, they generally appear as a well-demarcated tumour with less than 3 cm,
iso to hyperattenuating on CT, hypo to isointense on T1, and hyperintense on T2. An
avid and homogeneous enhancement is characteristic. Cystic changes may be present,
which in some cases, can make difficult the differentiation from a pineal cyst.

Epidemiology

Pineocytomas can be encountered at any age but mostly occur in young adults in the
second decade of life 5,7. Unlike pineal germinomas both genders are equally affected.

Clinical presentation

As with all other pineal region masses, clinical presentation is mainly from obstructive
hydrocephalus secondary to compression of the tectum of the midbrain and obstruction
of the aqueduct. Compression of the superior colliculi can also lead to a characteristic
gaze palsy, known as Parinaud syndrome.

Pathology

Pineocytomas are well-differentiated tumours and considered a WHO grade I tumours

(or sometimes grade II lesions - depending on the source 7 - see WHO classification of
CNS tumours). As is the case with the rest of the pineal gland, pineocytomas do not
have a well-formed blood brain barrier and as such enhance vividly with contrast 6.
Under light microscopy, pineocytomatous pseudorosettes are characteristic.

Radiographic features

Typically pineocytomas are slow growing and well-circumscribed tumours (compared to

pineoblastomas that tend to be larger, and less well circumscribed). They tend to be
solid, although focal areas of cystic change, or haemorrhage do occur. When the cystic
component is large, distinguishing them from pineal cysts can be difficult, although it
has been postulated that this is not so much the case if complete and accurate imaging
is obtained (i.e. good quality thin section MRI with contrast) 6.

CT

CT demonstrates the mass to be of intermediate density, similar to the adjacent brain.

Pineal calcifications tend to be dispersed peripherally. This is the same pattern seen in
other pineal parenchymal tumours, which is helpful in distinguishing these tumours from
pineal germinomas that tend to 'engulf' pineal calcification.

MRI

MRI is the modality of choice for examining tumours of the pineal region.

T1: hypo to isointense to brain parenchyma

T2

o solid components are isointense to brain parenchyma

o areas of cystic change are common

o sometimes the majority of the tumour is cystic

T1 C+ (Gd): solid components vividly enhance

Treatment and prognosis

Pineocytomas are treated surgically and have an excellent prognosis when a complete
resection is achieved (which is most of the time as they are well-circumscribed lesions).
A 5-year survival of 86% has been reported 7.

Local recurrent and even CSF metastases are reported, but rare 5.

Differential diagnosis

On CT or MRI consider:

pineal cyst

o at most thin smooth (<2 mm) peripheral enhancement

other pineal parenchymal tumours

o pineal parenchymal tumour with intermediate differentiation

o pineoblastoma: larger, poorly defined

o papillary tumour of the pineal region

germ cell tumours

 o germinoma

marked male predominance

engulfed calcification

o embryonal carcinoma

o choriocarcinoma

o teratoma: may contain fat

astrocytoma of the pineal gland

metastasis

Related articles

Pineal region masses

The pineal region is anatomically complex and plays host to a number of unique
masses and tumours as well as potentially affected by many entities seen more
frequently elsewhere in the brain.

General reading

pineal gland

pineal region mass

pineal region - an approach

Pathology

cystic non-neoplastic lesions[+]

pineal parenchymal tumours

o pineocytoma

o pineal parenchymal tumour with intermediate differentiation

o pineoblastoma
 o papillary tumour of the pineal region

germ cell tumours[+]

tumours also encountered in the pineal region[+]

pineal gland metastases

vascular lesions[+]

References

Article Information

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Cases and Figures

Figure 1: illustration - pineal calcification

Case 1

Case 2

Case 3

Case 4

Case 5

Imaging Differential Diagnosis