Neuroradiology (2000) 42: 509±514

Ó Springer-Verlag 2000 D IA G N O S T I C NE UR OR A DI O LO G Y

M. Nakamura Neuroradiological characteristics

N. Saeki
Y. Iwadate of pineocytoma and pineoblastoma
K. Sunami
K. Osato
A. Yamaura

Received: 30 July 1999

Accepted: 12 October 1999
)
M. Nakamura ´ N. Saeki ( ) ´ Y. Iwadate ´
A. Yamaura
Department of Neurosurgery,
Chiba University School of Medicine,
1±8-1 Inohana, Chuo-ku, Chiba,
260±8670 Japan
e-mail: saeki@med.m.chiba-u.ac.jp
Tel.: + 81-43-2 21 21 58
Fax: + 81-43-2 26 21 59
K. Sunami
Department of Neurosurgery,
Kawatetsu Hospital, Japan
K. Osato
Department of Neurosurgery,
Chiba Cancer Centre, Japan
Abstract We reviewed neuroradio-
logical images in two histologically
proven cases of pineocytoma and
three of pineoblastoma to delineate
the characteristic features of these
rare tumours. CT revealed isodense
or slightly hyperdense masses with
central or peripheral calcification;
enhancement with contrast medium
tended to be homogeneous in
pineocytomas and heterogeneous in
pineoblastomas. In the pineocyto-
mas, T1-weighted images revealed
rounded, sometimes or slightly lob-
ulated low-signal masses with
strong, homogeneous contrast en-
hancement. Their margin was clear,
without invasion of adjacent struc-
tures. In the pineoblastomas, how-
ever, T1-weighted images revealed
multilobulated tumours with heter-
ogeneous contrast enhancement.
All three pineoblastomas had poor-
ly defined margins with adjacent
structures such as the posterior thal-
amus or corpus callosum, suggesting
a more invasive nature. T2-weighted
images revealed nonspecific high
signal lesions in all five case.
Key words Pineal, tumours ´
Pineocytoma ´ Pineoblastoma ´
Computed tomography ´ Magnetic
resonance imaging

with advances in CT and MRI, there have been few re-

Introduction
Pineal region tumours are very rare, accounting for
0.4±1.0 % of intracranial tumours in adults [1]. About
40 % of pineal region tumours are germinomas, the
most common tumours of this region [2]. The neuro-
imaging characteristics including CT and MRI of ger-
minomas have been widely reported [3,4,5,6,7], and
these contribute to preoperative diagnosis and thera-
peutic decisions, including whole-brain radiation with-
out pathological confirmation [8,9]. Pineocytomas (PC),
or the more malignant pineoblastomas (PB), on the
other hand, are extremely rare, and have been estimat-
ed as less than 15 % of all pineal-origin tumours [10].
Since these two tumours are distinct from the more
common germ-cell tumours as regards prognosis and
treatment, including surgery, radiation, chemotherapy
[10,11], accurate diagnosis may be helpful before treat-
ment. Although preoperative diagnosis has improved
ports on the characteristic features with CT or MRI of
PC or PB, because of their rarity. We therefore reviewed
the preoperative CT and MRI of five patients with his-
tologically proven PC and PB to analyse the diagnostic
value of preoperative neuroimaging.
Methods
We reviewed two patients with PC and three with PB, all histolog-
ically confirmed by haematoxylin and eosin (H&E), Nissl, and
immunohistological staining, including neurone-specific enolase
(NSE), neurofilament (NFL), synaptophysin, and glial fibrillary
acidic protein (GFAP) antibodies. There were three men and two
women, mean age 42.8 years. All underwent both CT and MRI
prior to surgery.
Imaging included CT before and after iodinated contrast me-
dium and MRI. The latter MRI was performed at 1.5 T in four
cases and 0.5 T in one. T1-weighted spin-echo (SE) sequences
510

Table 1 Summary of clinical features and histological diagnoses demonstrated mild to moderate noncommunicating hy-
Case Age/ Symptoms Duration Pathological drocephalus. Two of the three PB had heterogeneous
No. Sex of symptoms diagnosis but mainly isointense signal and the third heteroge-
1. 56/M headache, 3 months pineoblastoma neous low signal on T1-weighted images. PC gave ho-
urinary incon- mogeneous isointense- or low signal on T1-weighted
tinence images. Two PB and both PC gave nonspecific hetero-
2. 51/F no symptoms (±) pineoblastoma geneous high signal on T2-weighted images. All PB en-
(incidental) hanced heterogeneously, while both PC homogeneous-
3. 49/M headache, 10 days pineoblastoma ly. The enhancement was weak to moderate in PB,
double vision strong and homogeneous in the PC. The three PB were
4. 32/M double vision 3 months pineocytoma irregularly lobulated and their margins with adjacent
5. 26/F headache, 5 months pineocytoma structures were not well-defined. Two PB had unclear
vomiting margins with the corpus callosum, posterior thalamus
blurred vision and midbrain, and the third had unclear margins with
the posterior thalamus and tectum on T1-weighted im-
ages both before and after contrast medium. Both PC
were well-circumscribed tumours with a clear margin
400±500/ 11±25 ms (TR/TE) and T2-weighted SE 2000±4000/ with the thalamus and corpus callosum; the tectal plate
80±110 ms (TR/TE) images were obtained. Gadolinium contrast was compressed in both cases.
medium was used in all patients. Axial, coronal and sagittal images
The CT findings are summarised in Table 3. The tu-
were obtained both before and after injection in all patients. The
size, shape, relationship to adjacent structures (posterior thalamus, mours were isodense or hyperdense in four cases; one
tectum, midbrain and corpus callosum) and signal intensity were PB was of slightly low density. All the tumours showed
analysed in each case. This information was compared to the his- calcification, in the centre of the tumour in three and
tology (PC or PB) and the CT findings. peripheral in two. No notable difference in density, or
the degree or site of calcification was noted. All PB en-
hanced heterogeneously, and rather weakly. Both PC
demonstrated very strong, homogeneous enhancement.
Results
The clinical details are presented in Table 1. The most
common symptom on admission was headache, which Representative cases
had persisted for from10 days to 5 months. Only patient
5 presented with papilloedema. Two patients presented Case 1
with bilateral abducens nerve palsies (cases 3 and 4) and
one (case 3) with Parinaud's sign as well. Case 2 was in- A 56-year-old man had continuous occipital headache
cidentally diagnosed when MRI was taken to search for and occasional urinary incontinence for 3 months. No
brain metastases; the patient had breast cancer, with a neurological abnormality, and no papilloedema was ob-
pelvic metastasis. served at the initial examination. CT revealed a moder-
The MRI findings are summarised in Table 2. The ate degree of hydrocephalus and a pineal mass with
maximum diameter of the three PB ranged from 2.0 to dense calcification at its centre (Fig. 1A,B). The mass
3.0 cm, and the two PC measured 2.5 cm. All patients enhanced very weakly with contrast medium. MRI dis-

Table 2 Summary of MRI findings

Tumor type/ Size (cm) Shape Signal intensity Contrast Site of Invasion
case No. enhancement
T1-weighted T2-weighted
Pineoblastoma
1 2 ” 2.5 Irregular Isointense High + + Heterogeneous Corpus callosum,
thalamus, midbrain
2 2 ” 1.5 Lobulated Isointense High + Heterogeneous Thalamus, tectum
3 3”3 Lobulated Low ± + + Heterogeneous Corpus callosum,
thalamus, midbrain
Pineocytoma
4 2 ” 2.5 Rounded Low High + + Homogeneous Tectum
5 2 ” 2.5 Rounded Isointense High + + + Homogeneous Thalamus, tectum
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Table 3 Summary of CT find- Tumor, case Hydrocephalus Tumour density Contrast enhancement Calcification/site
ings
1 ++ High + Heterogeneous + + + Central
2. + Isodense + Heterogeneous + + + Central
3. ++ Low + Heterogeneous + Peripheral
Pineocytoma
4. ++ High + + + Homogeneous + + Peripheral
5. ++ Isodense + + + Homogeneous + + + Central

closed heterogeneous high signal in the pineal region on

T1-weighted images, with weak, heterogeneous contrast
enhancement (Fig. 1 C-F). The margins between this
tumour and the posterior thalamus, corpus callosum and
midbrain were not well-defined. Following a ventricu-
loperitoneal shunt, CT-guided stereotaxic biopsy was
performed. H&E staining revealed medullary cell pro-
liferation without rosette formation or lobulation, indi-
cating a pineoblastoma. Because of the refusal of fur-
ther treatment by the patient and his family, no treat-
ment was given until he developed a left hemiparesis
and multiple cranial nerve palsies, due to dissemination
of the tumour. Two courses of chemotherapy (ACNU
100 mg, vincristine 1 mg/ week, procarbazine 300 mg/
day ) were followed by whole-brain radiation. The pa-
tient died 18 months after operation due to local recur-
rence and dissemination.

Case 3

A 49-year-old man had severe headache and double vi-

Fig. 1 A±F Case 1 (pineoblastoma). A,B CT before and after con-
trast medium; C MRI; D-F Contrast-enhanced MRI. Arrowheads
blurred margin with posterior thalamus (D,E) corpus callosum and
midbrain (F)
sion for 10 days. Parinaud's sign and mild abducens
palsy were noted at the initial examination. CT dis-
closed severe hydrocephalus and a slightly low density
mass in the pineal region, with heterogeneous, weak
contrast enhancement (Fig. 2 A,B). A small focus of
calcification was seen at the periphery of the tumour.
T1-weighted MRI revealed a heterogeneous low-signal
pineal mass (Fig. 2C). Contrast enhancement was heter-
ogeneous and moderate. The tumour was irregularly
lobulated, and its borders with the posterior thalamus,
corpus callosum and midbrain were not well-defined
(Fig. 2D-F). Following ventricular drainage, the tumour
was subtotally removed via an occipital transtentorial
approach. The pathological diagnosis was pineoblasto-
ma. Extended focal irradiation of 60 Gy was given fol-
lowing surgery. The patient died from dissemination of
the tumour 12 months after the initial operation.
Case 4

A 32 year-old-man had blurred vision and diplopia for

3 months before the initial examination. A mild ab-
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Fig. 2 A±F Case 3 (pineoblastoma). Images as in Fig. 1
ducens palsy and Parinaud's sign were observed. CT re-
vealed a homogeneous high-density mass with strong,
homogeneous contrast enhancement (Fig. 3A,B), with
moderate hydrocephalus. MRI disclosed a rounded,
well-circumscribed, slightly low-signal pineal mass on
T1-weighted images (Fig. 3C). This enhanced strongly
and homogeneously, and the margins between it and the
posterior thalamus and corpus callosum were clear
(Fig. 3D,E). The tectal plate appeared compressed on a
sagittal image (Fig. 3F). The tumour was partially re-
moved via an occipital transtentorial approach, and a
ventriculoperitoneal shunt was placed after the initial
operation. H&E staining revealed medullary cell pro-
liferation with obvious rosette formation. Immunohis-
tochemical staining with synaptophysin was positive.
Fig. 3A±F Case 4 (pineocytoma). Images as in Fig. 1. Arrowhead
compression of the tectal plate (F)
These histological findings were compatible with pine-
ocytoma. The patient received radiotherapy (41.4 Gy to
the whole brain and an additional 20 Gy to the tumour
area), and chemotherapy using etoposide 60 mg/m2/day
and Cisplatin 20 mg/m2/day intravenously for 5 days.
The residual pineal mass partially responded to this ad-
juvant therapy and the patient has been free of neuro-
logical symptoms during 15 months of follow-up.
Discussion
Because of its complicated anatomical structure of the
region, pineal region tumours are among of the most
difficult intracranial tumours to approach directly [12].

In recent years, preoperative diagnosis of pineal region
tumours has been improved, with advances in CT and
MRI. Since preoperative diagnosis of germinoma is re-
portedly reliable [3±7], diagnostic irradiation without
histological confirmation has been tried in some insti-
tutions [8,9]. Pineal parenchymal tumours such as PC or
PB are very uncommon [10,13]. Although CT findings
have been reported by some workers [4, 5, 11,14], little
has been published on MRI of these tumours [11, 15,16].
The biggest series of CT and MRI findings has been re-
ported by Chiechi et al. [11], who have summarised 11
cases of PC and eight of PB. They reported that PC ap-
peared dense and round, with peripheral calcification
on CT. Of seven PC, five enhanced heterogeneously and
two homogeneously. Both PC and PB gave isointense or
to slightly low signal on T1-weighted images, with het-
erogeneous contrast enhancement in nine cases (seven
PC and two PB).
In our two PC, CT revealed homogeneous, isodense
or slightly hyperdense masses with calcification, and
strong, homogeneous contrast enhancement. MRI gave
more information about the size and shape of the tu-
mour, and its relationship to adjacent structures such as
the posterior thalamus, corpus callosum, midbrain and
tectum. On MRI the tumours were round, sometimes
slightly lobulated, and their signal intensity were isoin-
tense with or slightly lower than brain parenchyma, with
homogeneous, strong contrast enhancement. Thus, our
cases of PC showed different pattern from those of
Chiechi et al. [11]. MRI also showed compression to the
tectal plate, which was resulting in obstructed hydro-
cephalus in both cases. The margins with adjacent
structures were clear, suggesting a less invasive nature.
We were interested in differentiating PC from ger-
minomas, the most common tumour in this region.
Zimmerman et al. [5] reported that germinomas are
homogeneous isodense- to slightly hyperdense tumours
with central calcification, and tend to enhance strongly
and homogeneously on CT. Germinomas are also re-
ported to give lower signal than or be isointense with
gray matter on T1-weighted images, and slightly higher
signal on T2-weighted images, with strong, homoge-
neous contrast enhancement [6,16]. Our findings in PC
on both CT and MRI thus were very similar to those of
germinomas.
PB, on the other hand, appeared irregularly lobulat-
ed, and the margins between them and the adjacent
structures were less well-defined. Contrast enhance-
ment on MRI and CT was moderate to weak, and het-
erogeneous. The few previous reports of the MRI ap-
pearances of PB have described iso- to high intensity on
T1- and onT2-weighted images [11, 16,17]. Most, how-
ever, gave little information about contrast enhance-
ment, and failed to give precise information about their
relationship to adjacent structures [11, 15,16]. Tien et al.
[16] described unenhanced MRI findings in three PB.
513
They tended to invade the thalamus (1/3), tectum (3/3),
corpus callosum (1/3) and cerebellar vermis (1/3). In our
series, contrast-enhanced MRI revealed more invasion
of adjacent structures than previously reported [16] on
unenhanced images. Chiechi et al. [11], in an analysis of
eight cases, reported that PB are more lobulated and
larger, and tend to have a lesser degree of calcification
than PC or germinomas. In our series, all the PC and PB
had various degrees of calcification, and there was no
difference between them as regards size and degree of
calcification. Indeed, two of PB showed more marked
calcification than two PC and the smallest tumour was a
PB. Although our findings are not conclusive, due to the
relatively small number of cases, the striking difference
which we observed between PC and PB was the rela-
tionship to adjacent structures. The more lobulated
shape, more invasive character, weaker and heteroge-
neous contrast enhancement on MRI of the PB, may be
findings in common with those of previous reports and
useful for distinguishing these tumours from PC and
germinomas [11, 16,17].
Tien et al. [16] reported that, of the other pineal re-
gion tumours, dermoid and benign teratoma have char-
acteristic fat signal, and intratumoral haemorrhage was
rather specific, suggesting choriocarcinoma. Falcoten-
torial meningioma has been reported as an isointense,
round mass with homogeneous contrast enhancement
on both CT and MRI [18,19]. The diagnosis, however, is
usually rather easy due to tentorial attachment on MRI.
Pilocytic astrocytoma is reportedly cystic with only the
mural nodule enhancing on MRI [16]. Satoh et al. [19]
reported the glioblastoma as a multilobular and solid
mass with a poor margin on MRI, similar to our findings
in PB. Since calcification is less likely in glioblastoma
multiforme or anaplastic astrocytoma, those highly ma-
lignant gliomas in the pineal region should be differen-
tiated from PB. Alpha-fetoprotein, human chorionic
gonadotrophin-beta, and placental alkaline phos-
phatase in the cerebrospinal fluid and serum are specific
biochemical tumour markers which give useful infor-
mation for differentiating between germ-cell tumours
[20, 21,22]. Preoperative diagnosis will become more
accurate by analysing laboratory data, including assays
for these tumour markers, as well as neuroradiological
images.
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References
1. Preslock JP (1984) The pineal gland:
basic implications and clinical correla-
tions. Endocr Rev 5: 282±308
2. Smirniotopoulos JG, Rushing EJ, Mena
H (1992) Pineal region masses: differ-
ential diagnosis. Radiographics 12:
577±596
3. Brant-Zawadzki M, Norman D (1987)
Magnetic resonance imaging of the
central nervous system. Raven Press,
New York, pp 172±173
4. Ganti SR, Hilal SK, Stein BM, Silver
AJ, Mawad M, Sane P (1986) CT of pi-
neal region tumors. AJR 146: 451±458
5. Zimmeman RA, Bilaniuk LT, Wood
JH, Bruce DA, Schut L (1980) Com-
puted tomography of the pineal, para-
pineal, and histologically related tu-
mors. Radiology 137: 669±677
6. Sumida M, Uozumi T, Kiya K, et al
(1995) MRI of intracranial germ cell
tumours. Neuroradiology 37: 32±37
7. Fujimaki T, Matsutani M, Funada N
(1994) CT and MRI features of intra-
cranial germ cell tumors. J Neuro-oncol
19: 217±226
8. Sano K (1983) Pineal region tumors:
problems in pathology and treatment.
Clin Neurosug 30: 59±91
9. Appuzzo MJL (1987) Surgery of the
third ventricle. Williams and Wilkins,
Baltimore, 663±683
10. Chang SM, Lillis-Hearne PK, et al
(1995) Pineoblastoma in adults. Neuro-
surgery 37: 383±391
11. Chiechi MV, Sminotopoulos JG, Mena
H (1995) Pineal parenchymal tumors:
CT and MR features. J Comput Assist
Tomogr 19: 509±517
12. Appuzzo MJL (1993) Brain surgery, vol
1. Livingstone, Edinburgh, pp 486±540
13. Rubinstein LJ (1981) Cytogenesis and
differentiation of pineal neoplasms.
Hum Pathol 12: 441±448
14. Lin S, Crane MD, Lin Z (1978) Char-
acteristics of calcification in tumors of
the pineal gland. Radiology 126:
721±726
15. Nakagawa H, Iwasaki S, Kihikawa K,
et al (1990) MR imaging of pineocyto-
ma: report of two cases. AJNR 11;
195±198
16. Tien RD, Barkovich AJ, Edwards MSB
(1990) MR imaging of pineal tumors.
AJNR 11: 557±565
17. Zee C, Segall H, Appuzzo M (1991)
MR imaging of pineal region neo-
plasms. J Comp Assist Tomogr 15:
56±62
18. Muller-Forell W, Schroth G, Egan PJ
(1988) MR imaging of the pineal region.
Neuroradiology 30: 224±231
19. Satoh H, Uozumi T, Kiya K, et al (1995)
MRI of pineal region tumors: relation-
ship between tumours and adjacent
structures. Neuroradiology 37: 624±630
20. Kida Y, Kobayashi T, Yoshida J, Kato
K, Kageyama N (1986) Chemotherapy
with cisplatin for AFP-secreting germ-
cell tumors of the central nervous sys-
tem. J Neurosurg 65: 470±475
21. Arita N, Ushio Y, Hayakawa T (1980)
Serum levels of alpha-fetoprotein, hu-
man chorionic gonadotropin and carci-
noembryonic antigen in patients with
primary intracranial germ cell tumors.
Onc Dev Biol Med 1: 368±374
22. Shinoda J, Yamada Y, Noboru S, Ando
T, Hirata T, Miwa Y (1988) Placental
alkaline phosphatase as a tumor marker
for primary intracranial germinoma.
J Neurosurg 68: 710±720