Cystic
Pineocytoma Case — Report—
Nobuaki
MOMOZAKI,
Masashi
FUKUI**,
Kiyonobu
Kiyotaka
IKEZAKI,
FUJII
and
Masamitsu
Takashi
ABE,
KISHIKAWA*
Departments of Neurosurgery and *Radiology, Saga Medical School, Saga; **Department of Neurosurgery , Neurological Institute, Kyushu University Faculty of Medicine, Fukuoka
Abstract Pineocytoma
and pineoblastoma,
unusual
of totally
case
neuronal toma
cystic
differentiation
and
and pineoblastoma
Key words:
pineal
tumor,
originating pineocytoma had a good
is an useful
from
outcome.
finding
pineocytoma,
cyst,
Pineocytoma and pineoblastoma both have in cidences of 0.2% or less of all intracranial neoplasms, and originate from pineal parenchymal cells.',',') More common pineal tumors are of germ cell origin. Histopathological and radiological studies show that a cystic tumor in the pineal region is com monly an epidermoid or dermoid tumor.',') We report a rare case of pineocytoma with a large cyst. Report
This 37-year-old female complained of headache, oc casionally accompanied by nausea and vomiting 3 months before admission. After 1 month, she also de veloped diplopia. Neurological examination found bilateral papilledema and lateral gaze paresis. All laboratory tests including alpha fetoprotein, beta human chorionic gonadotropin, and carcinoem bryonic antigen were within normal limits. Precon trast computed tomographic (CT) scans disclosed a pineal cystic mass with marginal calcification and obstructive hydrocephalus. Postcontrast CT scans Received
June
3, 1991;
Accepted
July
31,
parenchyma,
Prominent
to differentiate
Introduction
Case
pineal
in a 37-year-old
computed
female
are rare and usually is reported.
calcification these
from
The
associated benign
pineal
tumor with
solid.
An
showed pineocy
cysts.
tomography
showed a ring-like enhancement (Fig. 1). Cerebral angiograms showed no tumor stain or compression of the vein of Galen. The well-defined, grayish cystic tumor was found under the splenium of the corpus callosum via a right occipital transtentorial approach. The tumor was drained of about 5 ml of yellowish, slightly viscous fluid. The collapsed tumor was almost completely removed except for a small part adhered to the tec turn of the midbrain. The tumor resembled a der moid cyst intraoperatively. Histological study of the cyst wall specimens, however, revealed no epithelial component. The tumor consisted of a highly cellular area of medullary proliferation and a less densely cellular area. The tumor cells had small round nuclei and scanty cytoplasm. Large rosettes, with nuclei group ed around a central eosinophilic area, were often observed in the less densely cellular area (Fig. 2). The cyst wall showed spongy degeneration among loosely arranged tumor cells. There were some foci of rounded calcium deposits which often aggregated to form large calcified masses. Immunohistochemical studies revealed tumor cells positive to neuron
1991
Authors' present addresses: N. Momozaki, M.D., Division of Neurosurgery, Brain Research Institute, University of Califor nia Los Angeles School of Medicine, California, U.S.A.; K. Ikezaki, M.D. and K. Fujii, M.D., Department of Neurosurgery, Neurological Institute, Kyushu University Faculty of Medicine, Fukuoka, Japan.
Fig.
3
Immunoperoxidase strating
positive
cellular
area.
nent nuclear x 100.
stain tumor Slightly
membrane
for
NSE,
demon
cells in the less densely larger are
cells
with
strongly
promi stained.
were negative for S-100 protein and glial fibrillary acidic protein (GFAP). The histological diagnosis was a transitional form between pineoblastoma and
Fig.
1
upper:
Precontrast
hydrocephalus
CT
and
scans,
a cystic
calcification
in
the
Postcontrast
CT scans,
demonstrating
mass
pineal
with
ring-like
region.
lower:
showing
enhanced
pineocytoma with neuronal differentiation. The postoperative course was uneventful. She received 50-Gy whole brain irradiation . There was no evidence of tumor recurrence or dissemination 5 years later.
cyst
wall.
Discussion Russell and Rubinstein16) classified pineal cysts into neoplastic and non-neoplastic cysts. Non-neoplastic small cysts without mass effect are common inciden tal findings at autopsy. Recently, magnetic resonance (MR) imaging has suggested a relatively high in cidence of benign pineal cysts (or glial cysts) which are important to distinguish from other neoplasms.9) On CT scans, pineal cysts are characteristic of der moid and epidermoid tumors.') Pineocytoma and
Fig. 2
Photomicrograph revealing densely
cellular
hyperchromatic HE
stain,
of
the
pineocytomatous area. nuclei
tumor rosettes
Tumor and
cells scanty
specimen, in the less have
round
cytoplasm.
x 50.
specific enolase (NSE) in the less densely cellular area. Slightly larger, strongly positive cells sug gesting neuronal differentiation were also found near the pineocytomatous rosettes (Fig. 3). Tumor cells
pineoblastoma are usually solid and homogeneously enhanced by contrast media.') Jooma and Kendall ,' however, reported a pineocytoma appearing as a cystic mass with marginal enhancement. Other cases of cystic pineocytoma have also been reported .",") Histological studies have demonstrated frequent cystic degeneration in this type of tumor.") Promi nent calcification is associated with pineocytoma and pineoblastoma,s,",19) although frequently present in pineal teratomas.2,6) Therefore, a cystic appearance with prominent (ring-like) calcification may be, retrospectively, characteristic of pineocytoma and pineoblastoma. Benign pineal cysts usually have no abnormal calcification or ring-like enhancement on CT scans or MR images.10°")
The histological study revealed a transitional form between pineoblastoma and pineocytoma, with features of neuronal differentiation. Pineocytoma with neuronal or neuronal and astrocytic differentia tion may indicate a relatively benign clinical course.') Malignant features such as papillary pattern and fleurettes'"'"" ) were not observed. Cystic degeneration and focal bleeding often occur in pineocytoma, 13) so the mechanism of cyst formation was probably such secondary changes. It is important to identify pineocytoma with specific markers and electron microscope studies. Okeda et al.") concluded that NSE was present in both normal pineocytes and tumor cells. S-100 and GFAP-positive cells within the tumor may indicate astrocytic differentiation or pre-existing normal astrocytes. Pineocytoma with neuronal differentia tion have crowded clear and/or dense-cored vesicles, microtubules, and microfilaments in the processes.") The effectiveness and indication for radiation therapy of pineocytomas are still controversial. Vaquero et al.") did not recommend radiation ther apy for pineocytoma because the prognosis reported
with neuronal differentiation is good. Nakatsukasa et al.")
a case of disseminated
pineocytoma
York,
pineoblastoma component, and residual tumor or dissemination is suspected. Symptomatic cystic le sions in the pineal region should be managed surgical ly and precise histological diagnosis is necessary to decide further therapy.
7)
Herrick MK, Rubinstein LJ: The cytological differen tiating potential of pineal parenchymal neoplasms (true pinealomas). A clinicopathological study of 28 tumors. Brain 102: 289-320, 1979 Jooma R, Kendall BE: Diagnosis and management of pineal tumors. J Neurosurg 58: 654-665, 1983 Klein P, Rubinstein LJ: Benign symptomatic glial cysts of the pineal gland: A report of seven cases and review of the literature. J Neurol Neurosurg Psychiatry 52: 991-995, 1989 Lee DH, Norman D, Newton TH: MR imaging of pineal cysts. J Comput Assist Tomogr 11: 586-590, 1987 Lum GB, Williums JP, Machen BC, Akkaraju V: Benign cystic pineal lesions by magnetic resonance im aging. J Comput Assist Tomogr 11: 228-235, 1987 Nakatsukasa M, Toya S, Otani M, Yoshida K, Ishida Y, Ogawa Y: Cystic pineocytoma successfully treated with synchronized chemoradiotherapy. Case report. Neurol Med Chir (Tokyo) 29: 333-337, 1989 (in Japanese) Neuwelt EA, Glasberg M, Frenkel E, Clark WK: Malignant pineal region tumors. J Neurosurg 51: 597-607, 1979 Nielsen SL, Wilson CB: Ultrastructure of a pineocytoma.” JNeuropathol Exp Neurol 34: 148 “ 158, 1975 Okeda R, Song SJ, Nakajima T, Matsutani M: Obser vation of an autopsy case by electron microscopy and cell markers. Acta Pathol Jpn 34: 911-918, 1984
8) 9)
10)
11)
12)
13)
14)
15)
16) Russell DS, Rubinstein LJ: Pathology of Tumors of the Nervous System, ed 4. Baltimore, Williams & Wilkins, 1977, pp 287-290 17) Trojanowski JQ, Tascos NA, Rorke LB: Malignant pineocytoma with prominent papillary features. Cancer 50: 1789-1793, 1982 18)
2)
3)
4)
Borit A, Blackwood W, Mair WGP: The separation of pineocytoma from pineoblastoma. Cancer 45: 1408-1418, 1980 Chang CG, Kageyama N, Kobayashi T, Soshida J, Negoro M: Pineal tumors: Clinical diagnosis with special emphasis on the significance of pineal calcifica tion. Neurosurgery 8: 656-668, 1981 The Committee of Brain Tumor Registry in Japan: Brain Tumor Registry in Japan, vol 6. 1987, p 11 (in Japanese) DeGirolami U: Pathology of tumors of the pineal region, in Schmideck HH (ed): Pineal Tumors. New
1-19
Herrick MK: Pathology of pineal tumors, in Neuwelt EA (ed): Diagnosis and Treatment of Pineal Region Tumors. Baltimore, Williams & Wilkins, 1984, pp 31-60
References 1)
pp
6)
Acknowledgments
We thank Dr. Keith L. Black (UCLA) for his critical reading of this manuscript. We also thank Mr. T. Tanamachi and Mr. Y. Tateishi for their photographic assistance.
1977,
Disclafani A, Hudgins RJ, Edwards MSB, Wara W, Wilson CB, Levin VA: Pineocytomas. Cancer 63: 302-304, 1989
with a
pineoblastoma component in which chemotherapy and radiation therapy were effective. We suggest that radiation therapy is indicated if the tumor contains a
Masson,
5)
19)
Vaquero J, Ramiro J, Martinez R, Coca S, Bravo G: Clinicopathological experience with pineocytomas: Report of five surgically treated cases. Neurosurgery 27: 612-619, 1990 Wood JH, Zimmerman RA, Bruce DA, Bilaniuk LT, Norris DG, Schut L: Assessment and management of pineal-region and related 192-210, 1981
tumors.
Address reprint requests to: N. Momozaki, ment of Neurosurgery, Saga Medical Nabeshima,
Saga
849,
Japan.
Surg Neurol
16:
M.D., Depart School, 5-1-1
Pineocytoma Case — Report—
Nobuaki
MOMOZAKI,
Masashi
FUKUI**,
Kiyonobu
Kiyotaka
IKEZAKI,
FUJII
and
Masamitsu
Takashi
ABE,
KISHIKAWA*
Departments of Neurosurgery and *Radiology, Saga Medical School, Saga; **Department of Neurosurgery , Neurological Institute, Kyushu University Faculty of Medicine, Fukuoka
Abstract Pineocytoma
and pineoblastoma,
unusual
of totally
case
neuronal toma
cystic
differentiation
and
and pineoblastoma
Key words:
pineal
tumor,
originating pineocytoma had a good
is an useful
from
outcome.
finding
pineocytoma,
cyst,
Pineocytoma and pineoblastoma both have in cidences of 0.2% or less of all intracranial neoplasms, and originate from pineal parenchymal cells.',',') More common pineal tumors are of germ cell origin. Histopathological and radiological studies show that a cystic tumor in the pineal region is com monly an epidermoid or dermoid tumor.',') We report a rare case of pineocytoma with a large cyst. Report
This 37-year-old female complained of headache, oc casionally accompanied by nausea and vomiting 3 months before admission. After 1 month, she also de veloped diplopia. Neurological examination found bilateral papilledema and lateral gaze paresis. All laboratory tests including alpha fetoprotein, beta human chorionic gonadotropin, and carcinoem bryonic antigen were within normal limits. Precon trast computed tomographic (CT) scans disclosed a pineal cystic mass with marginal calcification and obstructive hydrocephalus. Postcontrast CT scans Received
June
3, 1991;
Accepted
July
31,
parenchyma,
Prominent
to differentiate
Introduction
Case
pineal
in a 37-year-old
computed
female
are rare and usually is reported.
calcification these
from
The
associated benign
pineal
tumor with
solid.
An
showed pineocy
cysts.
tomography
showed a ring-like enhancement (Fig. 1). Cerebral angiograms showed no tumor stain or compression of the vein of Galen. The well-defined, grayish cystic tumor was found under the splenium of the corpus callosum via a right occipital transtentorial approach. The tumor was drained of about 5 ml of yellowish, slightly viscous fluid. The collapsed tumor was almost completely removed except for a small part adhered to the tec turn of the midbrain. The tumor resembled a der moid cyst intraoperatively. Histological study of the cyst wall specimens, however, revealed no epithelial component. The tumor consisted of a highly cellular area of medullary proliferation and a less densely cellular area. The tumor cells had small round nuclei and scanty cytoplasm. Large rosettes, with nuclei group ed around a central eosinophilic area, were often observed in the less densely cellular area (Fig. 2). The cyst wall showed spongy degeneration among loosely arranged tumor cells. There were some foci of rounded calcium deposits which often aggregated to form large calcified masses. Immunohistochemical studies revealed tumor cells positive to neuron
1991
Authors' present addresses: N. Momozaki, M.D., Division of Neurosurgery, Brain Research Institute, University of Califor nia Los Angeles School of Medicine, California, U.S.A.; K. Ikezaki, M.D. and K. Fujii, M.D., Department of Neurosurgery, Neurological Institute, Kyushu University Faculty of Medicine, Fukuoka, Japan.
Fig.
3
Immunoperoxidase strating
positive
cellular
area.
nent nuclear x 100.
stain tumor Slightly
membrane
for
NSE,
demon
cells in the less densely larger are
cells
with
strongly
promi stained.
were negative for S-100 protein and glial fibrillary acidic protein (GFAP). The histological diagnosis was a transitional form between pineoblastoma and
Fig.
1
upper:
Precontrast
hydrocephalus
CT
and
scans,
a cystic
calcification
in
the
Postcontrast
CT scans,
demonstrating
mass
pineal
with
ring-like
region.
lower:
showing
enhanced
pineocytoma with neuronal differentiation. The postoperative course was uneventful. She received 50-Gy whole brain irradiation . There was no evidence of tumor recurrence or dissemination 5 years later.
cyst
wall.
Discussion Russell and Rubinstein16) classified pineal cysts into neoplastic and non-neoplastic cysts. Non-neoplastic small cysts without mass effect are common inciden tal findings at autopsy. Recently, magnetic resonance (MR) imaging has suggested a relatively high in cidence of benign pineal cysts (or glial cysts) which are important to distinguish from other neoplasms.9) On CT scans, pineal cysts are characteristic of der moid and epidermoid tumors.') Pineocytoma and
Fig. 2
Photomicrograph revealing densely
cellular
hyperchromatic HE
stain,
of
the
pineocytomatous area. nuclei
tumor rosettes
Tumor and
cells scanty
specimen, in the less have
round
cytoplasm.
x 50.
specific enolase (NSE) in the less densely cellular area. Slightly larger, strongly positive cells sug gesting neuronal differentiation were also found near the pineocytomatous rosettes (Fig. 3). Tumor cells
pineoblastoma are usually solid and homogeneously enhanced by contrast media.') Jooma and Kendall ,' however, reported a pineocytoma appearing as a cystic mass with marginal enhancement. Other cases of cystic pineocytoma have also been reported .",") Histological studies have demonstrated frequent cystic degeneration in this type of tumor.") Promi nent calcification is associated with pineocytoma and pineoblastoma,s,",19) although frequently present in pineal teratomas.2,6) Therefore, a cystic appearance with prominent (ring-like) calcification may be, retrospectively, characteristic of pineocytoma and pineoblastoma. Benign pineal cysts usually have no abnormal calcification or ring-like enhancement on CT scans or MR images.10°")
The histological study revealed a transitional form between pineoblastoma and pineocytoma, with features of neuronal differentiation. Pineocytoma with neuronal or neuronal and astrocytic differentia tion may indicate a relatively benign clinical course.') Malignant features such as papillary pattern and fleurettes'"'"" ) were not observed. Cystic degeneration and focal bleeding often occur in pineocytoma, 13) so the mechanism of cyst formation was probably such secondary changes. It is important to identify pineocytoma with specific markers and electron microscope studies. Okeda et al.") concluded that NSE was present in both normal pineocytes and tumor cells. S-100 and GFAP-positive cells within the tumor may indicate astrocytic differentiation or pre-existing normal astrocytes. Pineocytoma with neuronal differentia tion have crowded clear and/or dense-cored vesicles, microtubules, and microfilaments in the processes.") The effectiveness and indication for radiation therapy of pineocytomas are still controversial. Vaquero et al.") did not recommend radiation ther apy for pineocytoma because the prognosis reported
with neuronal differentiation is good. Nakatsukasa et al.")
a case of disseminated
pineocytoma
York,
pineoblastoma component, and residual tumor or dissemination is suspected. Symptomatic cystic le sions in the pineal region should be managed surgical ly and precise histological diagnosis is necessary to decide further therapy.
7)
Herrick MK, Rubinstein LJ: The cytological differen tiating potential of pineal parenchymal neoplasms (true pinealomas). A clinicopathological study of 28 tumors. Brain 102: 289-320, 1979 Jooma R, Kendall BE: Diagnosis and management of pineal tumors. J Neurosurg 58: 654-665, 1983 Klein P, Rubinstein LJ: Benign symptomatic glial cysts of the pineal gland: A report of seven cases and review of the literature. J Neurol Neurosurg Psychiatry 52: 991-995, 1989 Lee DH, Norman D, Newton TH: MR imaging of pineal cysts. J Comput Assist Tomogr 11: 586-590, 1987 Lum GB, Williums JP, Machen BC, Akkaraju V: Benign cystic pineal lesions by magnetic resonance im aging. J Comput Assist Tomogr 11: 228-235, 1987 Nakatsukasa M, Toya S, Otani M, Yoshida K, Ishida Y, Ogawa Y: Cystic pineocytoma successfully treated with synchronized chemoradiotherapy. Case report. Neurol Med Chir (Tokyo) 29: 333-337, 1989 (in Japanese) Neuwelt EA, Glasberg M, Frenkel E, Clark WK: Malignant pineal region tumors. J Neurosurg 51: 597-607, 1979 Nielsen SL, Wilson CB: Ultrastructure of a pineocytoma.” JNeuropathol Exp Neurol 34: 148 “ 158, 1975 Okeda R, Song SJ, Nakajima T, Matsutani M: Obser vation of an autopsy case by electron microscopy and cell markers. Acta Pathol Jpn 34: 911-918, 1984
8) 9)
10)
11)
12)
13)
14)
15)
16) Russell DS, Rubinstein LJ: Pathology of Tumors of the Nervous System, ed 4. Baltimore, Williams & Wilkins, 1977, pp 287-290 17) Trojanowski JQ, Tascos NA, Rorke LB: Malignant pineocytoma with prominent papillary features. Cancer 50: 1789-1793, 1982 18)
2)
3)
4)
Borit A, Blackwood W, Mair WGP: The separation of pineocytoma from pineoblastoma. Cancer 45: 1408-1418, 1980 Chang CG, Kageyama N, Kobayashi T, Soshida J, Negoro M: Pineal tumors: Clinical diagnosis with special emphasis on the significance of pineal calcifica tion. Neurosurgery 8: 656-668, 1981 The Committee of Brain Tumor Registry in Japan: Brain Tumor Registry in Japan, vol 6. 1987, p 11 (in Japanese) DeGirolami U: Pathology of tumors of the pineal region, in Schmideck HH (ed): Pineal Tumors. New
1-19
Herrick MK: Pathology of pineal tumors, in Neuwelt EA (ed): Diagnosis and Treatment of Pineal Region Tumors. Baltimore, Williams & Wilkins, 1984, pp 31-60
References 1)
pp
6)
Acknowledgments
We thank Dr. Keith L. Black (UCLA) for his critical reading of this manuscript. We also thank Mr. T. Tanamachi and Mr. Y. Tateishi for their photographic assistance.
1977,
Disclafani A, Hudgins RJ, Edwards MSB, Wara W, Wilson CB, Levin VA: Pineocytomas. Cancer 63: 302-304, 1989
with a
pineoblastoma component in which chemotherapy and radiation therapy were effective. We suggest that radiation therapy is indicated if the tumor contains a
Masson,
5)
19)
Vaquero J, Ramiro J, Martinez R, Coca S, Bravo G: Clinicopathological experience with pineocytomas: Report of five surgically treated cases. Neurosurgery 27: 612-619, 1990 Wood JH, Zimmerman RA, Bruce DA, Bilaniuk LT, Norris DG, Schut L: Assessment and management of pineal-region and related 192-210, 1981
tumors.
Address reprint requests to: N. Momozaki, ment of Neurosurgery, Saga Medical Nabeshima,
Saga
849,
Japan.
Surg Neurol
16:
M.D., Depart School, 5-1-1
