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

Arita K, Uozumi T, Sakoda K, Okamoto H, Naito M: Hemangiopericytic meningioma with both intraand extra-cranial metastases. Case report and review of the literature. Neurol Med Chir (Tokyo) 27: 329 -335, 1987 (in Japanese)

2) Bailey P, Bucy PC: The origin and nature of me ningeal tumors. American Journal of Cancer 15: 15 -54, 1931 3) 4)

5)

Begg CF, Garret R: Hemangiopericytoma occurring in the meninges. Cancer 7: 602-606, 1954 Blank W, Spring A, Giesen H, Artmann H: In tracranial hemangiopericytoma in a child. Klin Padiatr 200: 422-425, 1988

Cushing H, Eisenhardt L: Meningiomas: Their Classification, Regional Behavior, Life History and Surgical End Results. Springfield, CC Thomas, 1938, pp 19-55

6)

Fisher ER, Davis JS, Lemmen LJ: Meningeal hemangiopericytoma. Arch Neurol Psychiat 79: 40 - 45, 1958 7) Fukui M, Kitamura K, Nakagaki H, Yamakawa Y, Kinoshita K, Hayabuchi N, Jingu K, Numaguchi Y, Matsuura K, Watanabe K: Irradiated meningiomas: A clinical evaluation. Acta Neurochir (Wien) 54: 33 - 43, 1980 8) Fukui M, Kitamura K, Ohgami S, Takaki T, Kinoshita K, Watanabe K, Mihara K: Radiosensitivi ty of meningioma. Analysis of five cases of highly vascular meningioma treated by preoperative irradia tion. Acta Neurochir (Wien) 36: 47-60, 1977

9) Fukui M, Kitamura K, Yonemasu Y, Takaki T, Soe jima T, Wakisaka S, Okudera T, Mihara K: Malig nant primary meningeal tumors. Neurol Med Chir (Tokyo) 14: 35-46, 1974 10)

Goellner JR, Laws ER, Soule EH, Okazaki H: Hemangiopericytoma of the meninges. Mayo Clinic experience. Am J Clin Pathol 70: 375-380, 1978 11) Horten BC, Urich H, Rubinstein LJ, Montague SR: The angioblastic meningioma: A reappraisal of a nosological problem. Light-, electron-microscopic, tissue, and organ culture observations. J Neurol Sci 31: 387-410, 1977 12) Inoue H, Tamura M, Koizumi H, Nakamura M, Naganuma H, Ohye C: Clinical pathology of malig nant meningiomas. Acta Neurochir (Wien) 73: 179 -191, 1984 13) Itoyama Y, Itoh Y, Fukumura A, Takamura S, Matsukado Y, Tanimura A: Light and electron

14)

15)

16)

17)

18)

19)

20)

21)

22)

microscopy of microcystic meningioma. Case report. Neurol Med Chir (Tokyo) 27: 1104-1108, 1987 (in Japanese) Jääskeläinen J, Servo A, Haltia M, Wahlström T, Valtonen S: Intracranial hemangiopericytoma: Radiology, surgery, radiotherapy, and outcome in 21 patients. Jellinger

Surg Neurol 23: 227-236, 1985 K, Slowik F: Histological subtypes

23)

24) and

prognostic problems in meningiomas. J Neurol 208: 279-298,1975 Kamitani H, Masuzawa H, Sato J, Kanazawa I, Okada M: Intracranial hemangiopericytoma. Fine structural studies in vivo and in vitro. Neurol Med Chir (Tokyo) 28: 42-48, 1988 Kleinman GM, Liszczak T, Tarlov E, Richardson E: Microcystic variant of meningioma. A light microscopic and ultrastructural study. Am J Surg Pathol 4: 383-389, 1980 Michaud J, Gagne F: Microcystic meningioma: Clinico-pathologic report of eight cases. Arch Pathol Lab Med 107: 75-80, 1983 Mira JG, Chu FCH, Fortner JG: The role of radiotherapy in the management of malignant hemangiopericytoma. Report of eleven new cases and review of the literature. Cancer 39: 1254-1259, 1977 Orita T, Abiko S, Aoki H: Meningioma with vacuolated component. A contribution to the pathogenesis of the cyst formation in meningioma: Report of two cases. Neurol Med Chir (Tokyo) 25: 479-483, 1985 (in Japanese) Osborne DR, Dubois P, Drayer B, Sage M, Burger P, Heinz ER: Primary intracranial meningeal and spinal hemangiopericytoma: Radiologic manifestations. AJNR 2: 69-74, 1981 Peña CE: Meningioma and intracranial hemangio

25)

26)

27) 28)

29)

30)

pericytoma. A comparative electron microscopic study. Acta Neuropathol (Berl) 39: 69-74, 1977 Penfield W: Tumors of the sheaths of the nervous system, in: Penfield's Cytology and Cellular Pathology of the Nervous System, vol 3. New York, PB Hoeber, 1932, p 962 Pitkethly DT, Hardman JM, Kempe LG, Earle KM: Angioblastic meningiomas. Clinicopathologic study of 81 cases. J Neurosurg 32: 539-544, 1970 Popoff NA, Malinin TI, Rosomoff HL: Fine struc ture of intracranial hemangiopericytoma and angiomatous meningioma. Cancer 34: 1187-1197, 1974 Siegel RM, Messina AV: Computed tomography: The anatomic basis of the zone of diminished density surrounding meningiomas. AJR 127: 139-141, 1976 Skullerud K, Loken C: The prognosis in menin giomas. Acta Neuropathol (Berl) 29: 337-344, 1974 Takase M, Watanabe O: Radiosensitive hemangio pericytoma of the falx. Case report. J Neurosurg 68: 640-641, 1988 Taptas J: Intracranial meningioma in a four-month - old infant simulating subdural hematoma. J Neurosurg 18: 120-121, 1961 Yamakawa Y, Kinoshita K, Fukui M, Mihara K, Koga K, Kitamura K: Radiosensitive meningioma. Surg Neurol 13: 471-475, 1980

Address reprint requests to: Y. Itoyama, M.D., Depart ment of Neurosurgery, Kumamoto University Medical School, 1-1-1 Honjo, Kumamoto 860, Japan.

Intracranial cystic hemangiopericytoma--case report.

A rare case of intracranial hemangiopericytoma associated with a large cyst was treated by gross total removal and local irradiation. The tumor has no...
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