Intracranial
Cystic
—
Hemangiopericytoma
Case Report—
Youichi ITOYAMA, Hiroshi SETO, Shinji NAGAHIRO, Jun-ichi KURATSU, Shozaburou UEMURA and Yukitaka USHIO Department
of Neurosurgery,
Kumamoto
University Medical School, Kumamoto
Abstract A rare total
case
removal
of
ness of radiation specimen mation.
intracranial
and local
showed
hemangiopericytoma
irradiation.
therapy
Hemangiopericytoma
Key words:
intracranial
of the microcystic should
properties.
not
Complete
with
has not recurred
for hemangiopericytoma
aggregation
plastic and cytological malignancy.
associated
The tumor
is unclear.
surgical
hemangiopericytoma,
cyst
Histological
components,
be classified
a large
for 16 months,
as meningioma
treated
examination
possibly
removal
was
although
contributing because
is essential
by gross
the effective of the tumor to the cyst for
of the different
for this tumor
because
neo of its
cyst formation
Introduction Intracranial hemangiopericytomas originating in the meninges are rare, and cystic hemangiopericytomas are even rarer. We recently encountered a heman giopericytoma associated with a large cyst origi nating in the cerebellar tentorium. The possible mechanisms of cyst formation are discussed, with a review of the literature.
Case
Report
A 38-year-old male began to suffer from syncopal at tacks in spring, 1988. He was admitted to our hospital with generalized convulsion on March 9, 1989. On admission, he showed no abnormal neurological signs. However, computed tomograph ic (CT) scans showed a supra and infratentorial brain tumor associated with a large supratentorial cyst. The tumor was highly and homogeneously enhanced, and part of the cyst wall was also en hanced (Fig. 1). Gadolinium-enhanced magnetic resonance (MR) images showed both the tumor and a portion of the cyst to be highly enhanced (Fig. 2). Intra-arterial digital subtraction angiography (DSA) revealed a tumor stain fed by the right occipital ar Received 1990
May
7,
1990;
Accepted
September
17,
Fig.
1
Postcontrast supratentorial cyst.
The
are highly
CT scans, showing tumor associated tumor
and a portion
an infra and with a large of the cyst wall
enhanced.
tery from the arterial phase (Fig. 3). The diagnosis was cystic meningioma. On March 28, an operation was performed using the combined supra and infratentorial approach. The tumor originated in the cerebellar tentorium in contact with the transverse sinus and had grown supra and infratentorially. The tumor was grossly totally removed, and the dural adhesion near the transverse sinus was electrocoagulated carefully. The cyst wall was also removed, excluding the part ad hering tightly to the brain. The cyst contained a
Fig. 2
Axial (left) and coronal (right) gadolinium enhanced MR images, showing the tumor originating in the cerebellar tentorium. The tumor and a portion of the cyst wall are highly enhanced.
xanthochromic fluid. Light microscopic examination of the tumor specimen showed fusiform-shaped cells with in distinct margins. The nuclei were clear with scanty chromatin. Formation of vascular lumens and ag gregation of microcystic components were present in some places. Gitter stain showed well-developed reticulin fibers (Fig. 4). Tumor cells were also present in the cyst wall. Postoperative postcontrast CT scans found no
Fig. 3
Intra-arterial DSA (right carotid angiogram), showing a tumor stain with the blood supply from the occipital artery.
residual tumor (Fig. 5). Local irradiation of 50 Gy was given to prevent tumor recurrence. He was discharged on May 12, but is being followed as an outpatient. No tumor recurrence was observed up to 16 months after the postoperative radiation therapy. Discussion Hemangiopericytomas are rare in the central nervous system, 14,21)with an incidence of 1-3.8% of all primary intracranial meningeal tumors. 9,14,15,24)
Fig. 4
Photomicrographs of the tumor specimen, showing fusiform-shaped cells with indistinct margins. The nuclei are clear with scanty chromatin (A), and microcystic components ag gregate in some places (B). Gitter stain reveals well-developed reticulin fibers (C). A, B: HE stain, x 200; C: Gitter stain, x 400.
the radiation therapy there was no tumor
was effective in this case, recurrence for 16 months
postoperative local removal is essential
irradiation. for treatment
since after
Complete surgical of hemangioperi
cytoma, because the effect of radiation therapy re mains unclear and the incidence of local recurrence is extremely high.',', 10,12)
References 1)
Fig. 5
Postoperative no residual
postcontrast
CT
scans,
showing
tumor.
Hemangiopericytomas originating in the intracranial meninges were first reported by Begg and Garret') and Fisher et al.') Some authors have classified this tumor as an angioblastic meningioma 5,11,24)but others consider it is distinct from meningioma and represents a type of vascular neoplasm. 10,15,21) This question remains unresolved. Hemangiopericytomas frequently recur and metastasize extracranially12,14,15,21,24,27) in contrast to ordinary meningiomas. Goellner et al.") reported local recurrence in 80% and extracranial metastasis in 23% of 26 patients with hemangiopericytomas. We consider that hemangiopericytomas should not be classified as meningiomas, because the electron microscopic appearance is very different to menin giomas16,22,25>and the incidence of recurrence and extracranial metastasis is much higher. The incidence of cystic hemangiopericytoma is unclear, because of few definite diagnoses of this tumor by CT scanning. Blank et al.4' reported a pediatric case of cystic hemangiopericytoma, and Arita et al.') reported a hemangiopericytoma con taining necrotic, microcystic components and a macrocyst containing xanthochromic fluid. Possible mechanisms of cyst formation in meningioma in clude: 1) central degeneration and necrosis of the tumor due to insufficient blood flow, "'23) 2) exuda tion of plasma components,") 3) intratumoral bleed ing, 2,29)4) reforming process of the subarachnoid spaces '16) and 5) aggregation and enlargement of microcysts.13'20' In our case, some microcystic com ponents were present, so we speculate that the ag gregation and enlargement of microcysts were the contributing factors. The effectiveness of radiation therapy for he mangiopericytoma has not been established. Some authors believed it to be efficacious,', 12,28,30) while others questioned its effectiveness.",",") We consider
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Address reprint requests to: Y. Itoyama, M.D., Depart ment of Neurosurgery, Kumamoto University Medical School, 1-1-1 Honjo, Kumamoto 860, Japan.