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Journal of Neuro-Oncology 54: 251–261, 2001.

© 2001 Kluwer Academic Publishers. Printed in the Netherlands.

MRI of pineal region tumors

Yukunori Korogi1 , Mutsumasa Takahashi1 and Yukitaka Ushio2


1
Department of Radiology, 2 Department of Neurosurgery, Kumamoto University School of Medicine,
Kumamoto, Japan

Key words: pineal region tumor, germ cell tumor (GCTs), MRI

Summary

The pineal region is a heterogeneous area that includes the pineal gland and several parapineal structures. Pineal
region masses include germ cell tumors (GCTs), pineal parenchymal cell tumors, glioma, meningioma, metastases,
and non-neoplastic masses such as pineal cysts, lipoma, epidermoid, vascular malformations. Although MRI has
allowed an improvement in distinguishing true pineal masses from parapineal masses impinging into the region of
the gland, it is still difficult to differentiate the pineal GCTs from pineal parenchymal tumors with only images, and
the clinical informations such as age, sex, and the tumor markers are very important.

Introduction high incidence in Japan [3]. The age-adjusted GCTs


incidence in Japan is reported to be 0.17 per 100,000
The pineal region is a histologically heterogeneous population per year (male 0.3, female 0.07) [4]. Most
area that includes the pineal gland itself, the posterior patients with GCTs are between 10 and 30 years old;
third ventricle and aqueduct, the supraclinoid cisterns the peak age of presentation is the second decade.
(quadrigeminal plate, ambient cisterns, and the velum They can be classified into six types: germinoma,
interpositium), brain (tectum and brainstem, thalami, teratoma (mature teratoma and immature teratoma),
corpus callosum splenium), dura (tentorial apex) and embryonal carcinoma, choriocarcinoma, yolk sac
vessels (internal cerebral veins and vein of Galen, and tumor (endodermal sinus tumor), and mixed GCT
posterior choroidal and posterior cerebral arteries) [1]. which contains more than two components of above.
The list of possible pineal region masses is exten- Germinomas are the least differentiated of the germ
sive and includes germ cell tumors (GCTs), pineal cell group, whereas teratomas differentiate along all
parenchymal cell tumors, glioma, meningioma, metas- three cell lines (ectoderm, mesoderm, and endoderm).
tases, and non-neoplastic masses such as pineal cysts, York sac tumor, choriocarcinoma, and embryonal cell
lipoma, epidermoid, vascular malformations. Pineal tumor represent other less common GCTs.
gland neoplasms are relatively uncommon tumors. GCTs are most commonly located in the pineal
Pineal cysts, on the other hand, are remarkably com- region; their next most frequent site of occurrence is the
mon as incidental findings. GCTs are also not so hypothalamic–neurohypophyseal region. In the latter
uncommon in the oriental countries. MRI has allowed a case, the earliest symptom is diabetes insipidus. Pineal
marked improvement in the preoperative delineation of GCTs affected males predominantly, while suprasellar
benign and malignant pineal masses and in distinguish- GCTs have no preponderance of sex.
ing true pineal masses from parapineal masses imping-
ing into the region of the gland [2]. In this chapter, Pineal GCTs
we will briefly review the radiological findings of the
pineal region tumors focusing on the MR findings. In our series, germinomas, teratomas, and mixed GCTs
occupy 36%, 36%, and 18% of all pineal GCTs, respec-
Germ cell tumors tively. Teratoma mainly locates in this region. The
pineal lesions tend to protrude anteroinferiorly to the
GCTs account for 0.5–3.2% of primary intracranial tectal plate and posterior portion of the third ventricle,
tumors in adults and 11.8% in children, with a relative leading to hydrocephalus despite of the small mass. It
252

Figure 1. Germinoma with the synchronous lesions in the pineal


and suprasellar regions in an 11-year-old boy. Postcontrast
T1-weighted midsagittal image shows enlargement and mild
homogeneous enhancement of the pituitary stalk (arrowhead) as
well as a small mass at the pineal region (arrow).

is difficult to differentiate the pineal GCTs from pineal


parenchymal tumors with only images, and age, sex,
tumor markers as well as presence of cystic components
are important. Pineal gland calcification seems to be
the common characteristic of all pineal region tumors
and not necessarily very helpful in the differential
diagnosis. B
In our series, 16% of all intracranial GCTs
are the synchronous lesions in pineal and neuro-
hypophyseal regions, the prevalence of which is
similar to the previous report [5]. This kind of
tumor often shows some similarity: the small pineal
mass and the symmetric infundibular thickening
(Figure 1). Early detection of mass may be caused
by early complaint of diabetes insipidus. There have
been two hypotheses about the synchronous masses:
dissemination theory and multicentric origination
theory [5].

Imaging findings of GCTs

The solid components of the GCTs are iso- to slightly C


hypointense relative to the gray matter on T1-weighted
image (T1WI), and mixed iso- and hyperintense on Figure 2. Pineal germinoma in a 15-year-old boy. (A) Sagit-
T2-weighted image (T2WI) [6–11]. In particular, ger- tal T1WI. (B) Sagittal T2WI. (C) Sagittal T1WI with contrast
enhancement. Intrinsic pineal mass with cyst displaces superior
minomas are usually mildly hypointense on T1WI and
colliculi and obstructs the aqueduct (arrowhead). Solid compo-
mildly hyperintense on T2WI, and may be isointense nent of mass is mildly hyperintense on T2WI, and enhanced dif-
on both pulse sequence (Figures 1, 2). GCTs, espe- fusely after contrast administration (arrow).
cially germinomas, are typically demonstrated as a
253

A B

Figure 3. Mature teratoma in an 11-year-old boy. (A) Axial T1WI. (B) Axial T2WI. (C) Axial T1WI with contrast enhancement. Pineal
mass shows higher heterogeneity, a typical honeycomb-like appearance with multiple cystic components (arrowheads).
254

A
B

Figure 4. (Continued)
255

hyperdense mass on precontrast CT images. Teratomas be observed, probably representing high protein liquid.
of the pineal are strikingly heterogeneous (Figure 3), Detection of fatty tissues is relatively uncommon in the
and often have dense calcification within them. Marked intracranial teratomas. The high hemorrhagic tendency
high intensity of teratoma on both T1WI and T2WI can of choriocarcinoma has been reported. Peritumoral

A B

Figure 5. Pineoblastoma in an 11-year-old boy. (A) Axial T1WI. (B) Axial T2WI. (C) Sagittal T1WI with contrast enhancement. Pineal
mass shows low intensity on T2WI (arrow). Contrast enhancement of the mass is remarkable.


Figure 4. Immature teratoma in a 9-year-old boy. (A) Axial T1WI. (B) Axial T2WI. (C) Axial T1WI with contrast enhancement.
Heterogeneous mass with high intensity on both T1WI and T2WI is observed (arrowheads). High intensity is probably representing high
protein liquid. Peritumoral edema is also seen (arrow).
256

edema can be seen in the huge or malignant tumors our series have cystic components. The lesions reveal
(Figure 4). either multiple microcystic components or several
Contrast MR images are essential for evaluation macrocystic components. Teratomas are characteristic
of GCT; in particular, small masses may be equiv- compared with other GCTs because of its higher het-
ocal on plain MR. After IV injection of Gd-DTPA, erogeneity with honeycomb-like appearance due to
almost all lesions are enhanced markedly and multiple cystic components, high incidence of calcifi-
heterogeneously. The heterogeneity varied from mildly cation, and fatty substances [11] (see Figure 3).
to severely. Unlike the previous contrast CT and MRI
reports, which showed homogeneous enhancement
[6,7,12–14], the heterogeneous enhancement is com- Pineal parenchymal cell tumors:
mon findings. The tumors with cystic components pineocytoma and pineoblastoma
demonstrate high heterogeneity. Pathological hetero-
geneity of tumor tissue may also cause heterogeneous Tumors that arise from pineal parenchymal cell account
enhance. With a high-resolution MRI, cystic compo- for less than 15% of all pineal region neoplasms
nents are more frequently seen than reported before; [2]. Unlike germinomas these tumors may be found
about 50% of germinomas and 90% of other GCTs in in patients beyond the third decade of life. There

A B

Figure 6. Pineocytoma in a 32-year-old man. (A) Sagittal T1WI. (B) Sagittal T2WI. (C) Sagittal T1WI with contrast enhancement. Intrinsic
pineal mass fills pineal cistern and compressing vermis (arrowheads). Mass shows hyperintense on T2WI, and relatively homogeneous
enhancement.
257

are two types of pineal cell neoplasms: pineocytoma a higher degree of cytoplasm, should have relatively
and pineoblastoma. Pineocytomas are benign, well- higher signal intensity on T2WI (Figure 6).
delineated tumors with mature cells that are histo-
logically almost indistinguishable from normal pineal Pineal cyst
parenchyma. Pineoblastomas are malignant neoplasms
that are composed of undifferentiated or immature Non-neoplastic pineal cysts are benign cystic lesions
pineal cells. The reports of image findings for pineal that are lined by collagenous fibers, glial cells, and
parenchymal tumors varied greatly [11,15,16]. The normal pineal parenchymal cells [1]. Small asymp-
specific diagnosis of histopathologic type in pineal tomatic non-neoplastic pineal gland cysts are common
cell tumors is often not possible with MRI [2]. MRI incidental findings, seen in up to 40% of routine
depicts pineal neoplasms as lobulated, solid tumors autopsies. Occasionally, the imaging appearance of
which enhance densely with contrast. MR signal inten- benign pineal cyst is indistinguishable from small cys-
sities vary, but generally pineoblastomas are essentially tic neoplasms. The pineal cyst is round and smoothly
isointense to gray matter on T2WI (Figure 5), possibly marginated on MRI (Figure 7). It can be small and lie
related to the known paucity of cytoplasm and over- within a small portion of the gland, or it can replace
all dense cellularity seen in these lesions. Pineoblas- the entire structure. The contents of the pineal cyst
tomas may also show brain edema or invasion in the are homogeneous, and are either isointense to cere-
surrounding brain parenchyma. Pineocytomas, with brospinal fluid (CSF) or diffusely hyperintense. The

C
B

Figure 7. Large pineal cyst in a 67-year-old women. (A) Sagittal T1WI. (B) Axial T2WI. (C) Sagittal T1WI (MPRAGE) with contrast
enhancement. Round, homogeneous mass in pineal compresses superior colliculi without obstruction of the aqueduct (arrowhead). Mass
is only slightly higher intensity than CSF on T2WI. No enhancement is seen.
258

relative hyperintensity of the cyst fluid may relate to Miscellaneous lesions


factors such as isolation form flow, high protein con-
tent, or even old hemorrhage [2]. The pineal cyst usu- Glioma
ally does not enhance, but surrounding residual pineal Pineal region glial cell tumor usually extend from brain
tissue will. parenchymal structures that lie in close proximity to

A B

Figure 8. Anaplastic glioma in a 54-year-old woman. (A) Axial T1WI. (B) Axial T2WI. (C) Sagittal T1WI with contrast enhancement.
Pineal region mass compresses superior colliculi just like an intrinsic pineal mass. However, swelling and invasion in the corpus callosum
splenium is clearly seen (arrow). Note dissemination in the posterior fossa (arrowhead).
259

B C

Figure 9. Meningioma of the tentorial apex in a 40-year-old woman. (A) Sagittal T1WI. (B) Coronal T2WI. (C) Axial T1WI with contrast
enhancement. Large tumor with typical signal intensities as meningioma markedly compresses midbrain, cerebellum, and corpus callosum
(arrowheads).

the pineal gland, such as brainstem, corpus callosum homogeneous (Figure 9). Relationship to the falx and
as well as thalamus, and only rarely originate from tentorium as well as the thickening of the adjacent dura
the stroma of the pineal gland itself (Figure 8). are important for differential diagnosis.
Tectal gliomas are usually low-grade astrocytomas that
enlarge the tectum. The aqueduct may be engulfed and Metastatic disease
occluded, resulting in obstructive hydrocephalus. The Specific locations of intracerebral metastases gener-
gliomas from thalami or corpus callosum tend to be ally coincide with the respective lobar volumes. Very
higher grade: anaplastic astrocytoma or glioblastoma. rarely, metastasis to the pineal parenchyma can occur
and mimic the primary pineal gland masses. Signal
Meningioma intensity patterns and contrast enhancement of pineal
Pineal region meningiomas usually extend from adja- metastases on MRI are usually non-specific and basi-
cent tentorial apex. Compared with white matter, cally the same as in other locations (Figure 10).
meningiomas on T1WI are almost always hypointense.
On T2WI, approximately one-half are isointense Vascular lesions
and one-half hyperintense, with a rare tumor being The most common vascular mass in the pineal region
hypointense in relationship to the cerebral cortex. is an elongated, tortuous basilar artery that elevates
Contrast enhancement is usually strong and relatively and compresses the third ventricle. Occasionally,
260

C B

Figure 10. Metastatic adenocarcinoma to the pineal gland in a 61-year-old man. (A) Sagittal T1WI. (B) Axial T2WI. (C) Sagittal T1WI
with contrast enhancement. Relatively hypointense mass on T2WI locates at pineal region (arrows). Differential diagnosis may include
the pineal cell tumor, glioma, and meningioma; in this case, however, multiple intracerebral metastases also exited.

aneurysms of the P2 or P3 segments of the posterior tumor with synchronous lesions in the pineal and suprasellar
cerebral artery occur here. regions: report of six cases and review of the literature. Surg
Neurol 38: 114–120, 1992
6. Sumida M, Uozumi T, Kiya K, Mukada K, Arita K, Kuris
References K, Sugiyama K, Onda J, Satoh H, Ikawa F, Migita K: MRI
of intracranial germ cell tumors. Neuroradiology 37: 32–37,
1. Osborn AG: Pineal region masses. In: Diagnostic Neurora- 1995
7. Fujimaki T, Matsutani M, Funada N, Kirino T, Takakura K,
diology. Mosby, St. Louis, 1994, pp 408–421
Nakamura O, Tamura A, Sano K: CT and MRI features of
2. Atlas SW, Ehud L: Intra-axial brain tumors. In: Atlas SW
intracranial germ cell tumors. J Neuro-Oncol 19: 217–226,
(ed.) Magnetic Resonance Imaging of the Brain and Spine.
1994
Lippincott-Raven, Philadelphia, 1996, pp 315–422
8. Kilgore DP, Strother CM, Strashak RJ, Haugton VM: Pineal
3. Committee of Brain Tumor Registry of Japan: The
germinoma: MR imaging. Radiology 158: 435–438, 1986
epidemiology and statistic of brain tumors in Japan, 1990
9. Zee CS, Segall H, Apuzzo M, Destian S, Colletti P, Ahmadi
(Japanese)
J, Clark C: MR imaging of pineal region neoplasms. J Com-
4. Kuratsu J, Ushio Y: Epidemiological study of primary
put Assist Tomogr 15: 156–163, 1991
intracranial tumors: a regional survey in Kumamoto prefec- 10. Chang T, Teng MMH, Guo WY, Sheng WY: CT of pineal
ture in the southern part of Japan. J Neurosurg 84: 946–950, tumors and intracranial germ-cell tumors. AJNR Am J Neu-
1996 roradiol 10: 1039–1044, 1989
5. Sugiyama K, Uozumi T, Kiya K, Mukada K, Arita K, Kurisu 11. Satoh H, Uozumi T, Kiya K, Kuris K, Arita K, Sumida M,
K, Hotta T, Ogasawaralt Sumida M: Intracranial germ-cell Ikawa F: MRI of pineal region tumors: relationship between
261

tumors and adjacent structures. Neuroradiology 37: 624– 15. Zimmerman RA, Bilaniuk LT, Wood JH, Bruce DA, Schut
630, 1995 L: Computed tomography of pineal, parapineal, and histo-
12. Tanaka R, Takeda N, Ueki K, Itoh J, Honda H: Computer- logically related tumors. Radiology 137: 669–677, 1980
ized tomography in the diagnosis and management of pineal 16. Chiechi MV, Smirniotopoulos JG, Mena H: Pineal
region tumors. Neurol Med Chir (Tokyo) 20: 1103–1114, parenchymal tumors: CT and MR features. J Comput Assist
1980 Tomogr 19: 509–517, 1995
13. Futrell NN, Osborn AG, Cheson BD: Pineal region tumors:
computed tomographic-pathologic spectrum. AJR 137: Address for offprints: Yukunori Korogi, Department of Radiology,
951–956, 1981 Kumamoto University School of Medicine, 1-1-1 Honjo,
14. Ganti SR, Hilal SK, Stein BM, Silver, AJ, Mawad M, Sane Kumamoto 860, Japan; Tel.: 81-96-373-5262; Fax: 81-96-362-4330;
P: CT of pineal region tumors. AJR 146: 451–458, 1986 E-mail: yuku@kaiju.medic.kumamoto-u.ac.jp

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