Pineoblastoma

A.Prof Frank Gaillard et al.

Pineoblastomas are tumours that are best thought of small round blue cell
tumours located in the pineal region and thus, they closely resemble (both on
imaging and on histology) medulloblastomas and retinoblastomas. They are the
most aggressive and highest grade tumour among pineal parenchymal tumours,
and considered WHO grade IV tumours.

On imaging, they usually present as large lobulated and enhancing tumours

(more than 3 cm), hyperattenuating on CT (highly cellular), with heterogeneous
signal intensities on MRI, sometimes with evident necrotic and haemorrhage
regions. Restricted diffusion is commonly evident and, in almost all cases,
obstructive hydrocephalus is observed at the presentation.

Epidemiology
Pineoblastomas are the most aggressive pineal parenchymal tumour and
account for a substantial proportion of such tumours (24-50%) . They are
7

typically found in young children, with only a slight female predilection (M:F 0.7:1;
similar to other pineal parenchymal tumours), which is in contrast to the male
predominance seen in pineal germinomas) . 12

There is a well-established association with hereditary retinoblastomas. Patients

with hereditary (bilateral) retinoblastoma no more than 5% develop midline
(suprasellar or pineal) neuroblastic tumours . Such cases are sometimes
6,10

referred to as trilateral retinoblastoma.

Clinical presentation
Pineoblastomas are typically large and almost always associated with obstructive
hydrocephalus, due to compression of the cerebral aqueduct. Compression of
the tectal plate may also result in the Parinaud syndrome.

They are highly malignant tumours prone to CSF seeding, which is present in
15% of patients at the time of diagnosis.

Pathology
Pineoblastomas originate from pinealocytes and/or their precursors . They are
11

the least differentiated pineal cell tumours, with pineocytomas and pineal
parenchymal tumour with intermediate differentiation representing better-
differentiated tumours along the same spectrum. Pineoblastomas are considered
WHO grade IV tumours . 12

Macroscopic appearance
These tumours are poorly defined, often invading adjacent brain parenchyma.
Their cut surface is pink and soft, with areas of necrosis and haemorrhage not
infrequently seen .12

Microscopic appearance
Pineoblastomas are composed of tightly packed small round blue cells (high
nuclear to cytoplasmic ratio) which in turn determines their imaging appearances
(see below) . The growth pattern is largely featureless with the pineocytomatous
7

rossettes seen in pineocytomas not present . In contrast, Homer Wright

12

rosettes and Flexner-Wintersteiner rosettes are occasionally seen . 12

Areas of necrosis are frequently encountered . Mitotic rate is usually high.

12

Immunophenotype
Immunophenotype is similar to other pineal parenchymal tumours . 12

synaptophysin: positive
SMARCB1: positive
other neuronal markers (e.g. neuron-specific enolase, neurofilament
protein and chromogranin-A): variable

Radiographic features
Pineoblastomas tend to be large poorly defined masses, with frequent CSF
seeding at presentation. They have a tendency to involve directly adjacent brain
structures, which helps distinguish them from other pineal tumours that tend to
be better circumscribed.

CT
The solid component tends to be slightly hyperdense compared to the adjacent
brain due to high cellularity. This is a characteristic shared by other small round
blue cell tumours such as PNET and medulloblastoma.
Classically, they are described as having peripherally dispersed or "exploded"
calcification (mnemonic: blasted calcification), similar to pineocytomas. In
contrast, pineal germinomas tend to engulf pineal calcification.

MRI
Pineoblastomas tend to appear as sizable (>4 cm) irregular masses often with
evidence of invasion into adjacent brain . Typical signal characteristics include :
6,9 9

T1: isointense to hypointense to adjacent brain

T2
o isointense to adjacent brain
o areas of cyst formation or necrosis may be present
T1 C+ (Gd): vivid heterogeneous enhancement
DWI/ADC
o restricted diffusion due to dense cellular packing
o ADC values are typically ~400-800 mm /s 2 7

Central necrosis is sometimes present which can make the mass appear
centrally cystic and thus can roughly mimic a pineal cyst, although the latter
should have a smooth, thin wall . 6

Screening of the whole neural axis is necessary as CSF seeding is seen in 45%
of cases . 7

Treatment and prognosis

Treatment is usually a combination of surgery, chemotherapy and radiation . 7

Despite treatment, the prognosis has historically been poor, with a 5-year survival
as low as 10%. More recently 5-year survival of 58-81% have been
reported with median overall survival times of 4-8 years .
8,12 12

The most important factors predicting a favourable outcome are early detection
and treatment with at least chemotherapy, preferably a high dose regime with
stem cell rescue . 11

Differential diagnosis
General imaging differential considerations include:

other pineal parenchymal tumours

 o pineocytoma: mature well-differentiated tumour: smaller and better
circumscribed
o pineal parenchymal tumour with intermediate differentiation
o papillary tumour of the pineal region
germ cell tumours
o germinoma
marked male predominance
engulfed calcification
ADC values are typically much higher (~1000-2000 mm /s ) 2 7

o embryonal carcinoma
o choriocarcinoma
o teratoma: may contain fat
pineal cyst
o thin (<2 mm) wall
astrocytoma of the pineal gland
metastasis
medulloblastoma
o imaging is very similar
o located in the vermis rather than pineal region but can be difficult to
distinguish if very high in the vermis and very large
 References
1. Smirniotopoulos JG, Rushing EJ, Mena H. Pineal region masses: differential diagnosis. Radiographics.
1992;12 (3): 577-96. Radiographics (abstract) - Pubmed citation
2. Chang T, Teng MM, Guo WY et-al. CT of pineal tumors and intracranial germ-cell tumors. AJR Am J
Roentgenol. 1989;153 (6): 1269-74. AJR Am J Roentgenol (abstract) - Pubmed citation
3. Ganti SR, Hilal SK, Stein BM et-al. CT of pineal region tumors. AJR Am J Roentgenol. 1.86;146 (3):
451-8. AJR Am J Roentgenol (abstract) - Pubmed citation
4. Tien RD, Barkovich AJ, Edwards MS. MR imaging of pineal tumors. AJR Am J Roentgenol. 1990;155
(1): 143-51. AJR Am J Roentgenol (abstract) - Pubmed citation
5. Banks KP, Brown SJ. AJR teaching file: solid masses of the pineal region. AJR Am J Roentgenol.
2006;186 (3): S233-5. doi:10.2214/AJR.05.0519 - Pubmed citation
6. Rodjan F, De Graaf P, Moll AC et-al. Brain abnormalities on MR imaging in patients with
retinoblastoma. AJNR Am J Neuroradiol. 2010;31 (8): 1385-9. AJNR Am J Neuroradiol (full
text) - doi:10.3174/ajnr.A2102 - Pubmed citation
7. Dumrongpisutikul N, Intrapiromkul J, Yousem DM. Distinguishing between germinomas and pineal cell
tumors on MR imaging. AJNR Am J Neuroradiol. 2012;33 (3): 550-5. AJNR Am J Neuroradiol (full
text) - doi:10.3174/ajnr.A2806 - Pubmed citation
8. Zimmerman RD, Grossman RI. Neuroradiology. Mosby. (2010) ISBN:0323045219. Read it at Google
Books - Find it at Amazon
9. Griscom NT. Practical Pediatric Imaging. Lippincott Williams & Wilkins. (1998)
ISBN:0316494739. Read it at Google Books - Find it at Amazon
10. de Jong MC, Kors WA, de Graaf P et-al. The Incidence of Trilateral Retinoblastoma: A Systematic
Review and Meta-Analysis. Am. J. Ophthalmol. 2015;160 (6): 1116-
1126.e5. doi:10.1016/j.ajo.2015.09.009 - Pubmed citation
11. de Jong MC, Kors WA, de Graaf P et-al. Trilateral retinoblastoma: a systematic review and meta-
analysis. Lancet Oncol. 2014;15 (10): 1157-67. doi:10.1016/S1470-2045(14)70336-5 - Pubmed
citation
12. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK "WHO Classification of Tumours of the Central
Nervous System. 4th Edition Revised" ISBN: 9789283244929