Moyamoya

Disease

Associated

with Pituitary

Report of Two Cases—

Adenoma —

Kazunori ARITA, Tohru UOZUMI, Shuichi OKI, Satoshi KUWABARA, Shinji OHBA, Toshinori NAKAHARA, Zainal MUTTAQIN, Hideaki KOHNO and Kenji YAMADA Department

of Neurosurgery,

Hiroshima

University

School of Medicine, Hiroshima

Abstract Moyamoya disease associated with prolactin (PRL)-producing pituitary adenomas occurred in two females with elevated blood PRL levels (285 and 120 ng⁄ml). Computed tomography revealed cystic tumors extending from the sella turcica to the suprasellar cistern. Carotid angiography demonstrated stenoses or obstructions of the bilateral internal carotid arteries at their end point and development of bilateral basal moyamoya vessels. Histological diagnosis in one case was PRL-producing chromophobe adenoma. No stigmata of neurofibromatosis or any history of irradiation was found. Compression of carotid arteries by the tumor was unlikely. These cases should therefore be classified as moyamoya disease accompanied by brain tumor, a very rare occurrence. The hypothalamic distur bance caused by moyamoya disease may have induced the hyperprolactinemia, resulting in secondary prolactinoma. Key words:

prolactinoma,

pituitary

adenoma,

moyamoya

disease

included moyamoya Ophthalmological

Introduction

disease diagnosed in her son. examination disclosed bitem

Previously reported cases of moyamoya vessels asso poral hemianopsia with severe impairment of visual acuity (right 0.3, left 0.1) and bilateral optic atrophy. ciated with brain tumor have almost always not been Physical and neurological examination, including classified as moyamoya disease, since the stenoses of electroencephalography, showed no abnormalities the basal cerebral arteries were caused by irradia tion,1,2,7-9,16,20,21,24-26,28,31,34) compression of internal with a regular menstrual cycle. Endocrinological ex carotid arteries by the tumor,4,11,21,27,29)or were a amination revealed hyperprolactinemia (PRL 285 manifestation of neurofibromatosis 3,6,12,14,17,23,24,28) ng/ml) with normal basal and reserve levels of the Here, we report two cases of classical moyamoya other anterior pituitary hormones. disease occurring as an idiopathic primary Computed tomographic (CT) scans demonstrated cerebrovascular lesion, associated with prolactin a cystic tumor 14 x 24 mm extending from the sella turcica to the suprasellar cistern (Fig. 1). Coronal (PRL)-producing pituitary adenomas, and discuss the etiology of moyamoya disease associated with scans showed no cavernous sinus invasion. Carotid brain tumors. angiograms demonstrated bilateral stenoses of the in ternal carotid artery at the internal carotid bifurca Case Reports tion (Fig. 2). Basal moyamoya vessels were seen bilaterally, more pronounced on the left. Vertebral Case 1: A 40-year-old female was in good health un angiograms showed collateral blood supply via the til she noticed blurring of the right temporal visual field in December, 1982. A local ophthalmologist diagnosed bitemporal hemianopsia and referred her to our department in February, 1983. Family history Received

January

7, 1992;

Accepted

April

9, 1992

posterior pericallosal artery. Left external carotid angiograms revealed transdural anastomoses via the middle meningeal artery. The angiographic diagnosis was stage 3 (after Suzuki and Takaku30)) moyamoya disease. Neither carotid artery was significantly shifted by the tumor.

Fig. 1

Case 1. Axial (left) and coronal (right) CT scans, showing a cystic tumor extending from the sella turcica to the suprasellar cistern.

The tumor was subtotally removed through the transsphenoidal route under general anesthesia. No symptoms of cerebral ischemia were evident during the perioperative period. The tumor was histological ly diagnosed as chromophobe adenoma. Postopera tively, her visual acuity and blood PRL level were normalized. Case 2: A 23-year-old female was in good health with regular menstrual cycles, apart from chorea minor when aged 12 years, until she became amenorrheal. A gynecologist discovered hyperprolactinemia. She was admitted to our department in February, 1989, aged 24. Physical examination revealed no abnormalities except for galactorrhea and amenorrhea. En docrinological examination showed hyperprolac tinemia (PRL 120 ng/ml) with normal basal and reserve levels of the other anterior pituitary hor mones. Electroencephalography was also normal. CT scans showed a hypodense intrasellar tumor, which appeared hypointense on gadolinium-en hanced magnetic resonance (MR) images (Fig. 3). The tumor had a slight extension to the suprasellar cistern and was 11 mm high. CT scans and MR im ages also showed old infarcts in the left temporal and the right occipital lobes. The tumor neither encased nor compressed the internal carotid artery on the cor onal CT scans and MR images. Carotid angiograms showed obstruction of the bilateral internal carotid arteries at their end point without displacement (Fig. 4). Basal and ethmoidal moyamoya vessels were ob served, but no opacification of the main trunks of the anterior cerebral and middle cerebral arteries. Collateral vessels, the so-called vault moyamoya vessels, arose from the branches of external carotid

Fig. 2

Case 1. Right (upper) and left (lower) internal carotid angiograms, showing bilateral stenoses of the internal carotid arteries at the carotid bifurcation. Basal moyamoya vessels are seen bilaterally, more pronounced on the left.

Fig. 3 Case 2. left: Coronal CT scan, showing a cystic tumor with slight suprasellar extension and an old infarct in the left temporal lobe. right: Midsagittal MR image, showing a round cystic tumor.

artery. The angiographic diagnosis was stage 4 moyamoya disease. Single photon emission CT scans displayed low cerebral blood flow areas in the left temporal and frontal lobes. Revascularization surgery followed by trans sphenoidal adenomectomy was scheduled, but per

cle, and

Fig. 4

Case 2. Right (upper) and left (lower) com mon carotid angiograms, showing obstruc tions of the bilateral internal carotid arteries at their end point without displacement. Basal, ethmoidal, and vault moyamoya vessels are well developed.

mission was refused. No histological diagnosis was established, but this tumor was believed to be a PRL-producing pituitary adenoma.

Discussion Moyamoya disease is a clinical entity, characterized by bilateral stenoses of the distal portion of the inter nal carotid arteries and emergence of a hazy, puff of smoke collateral circulation called moyamoya vessels at the base of the brain. The etiology of progressive stenosis of the internal carotid artery is un known. 14,22,30) Many cases of moyamoya vessels associ ated with brain tumor have been reported. However, the cerebrovascular changes were almost always sec ondary, due to the compression of bilateral internal carotid arteries by the tumor, irradiation, or the presence of neurofibromatosis. The most frequent cause was irradiation, 9,16,20,21,24-26,28,31,34) resulting in subintimal fibrosis degeneration

of internal

elastic

tissue,

smooth

and mus

endothelial

cells in the vascular

wall. In

younger children, the occlusion of major cerebral arteries and the development of collateral circulation were more pronounced than in older children and adults. In neurofibromatosis, the genesis of brain tumor and cerebrovascular occlusive changes is not excep tional. 3,6,12-14,17,23,24,28) Many clinical and pathologi cal characteristics 15)are common to moyamoya dis ease and cerebrovascular occlusive changes in neurofibromatosis. However, neurofibromatosis dem onstrates several features different from moyamoya disease. The internal carotid arteries may be in volved unilaterally, only middle cerebral arteries may be involved with normal internal carotid ar teries, or the basal vascular networks may be unilateral and develop later in life. The criteria for moyamoya disease therefore exclude patients with neurofibromatosis, since another pathological mechanism may cause the cerebrovascular occlusive changes. A parasellar tumor may obstruct the internal carotid arteries by encasement or compression of the arterial walls against surrounding structures. In some cases, especially in children, marked develop ment of collateral circulation identical to the disease sometimes occurs moyamoya . a 10,21,27,29) In our cases, the suprasellar extension was not so remarkable and no cavernous sinus invasion was ob served. Even in other cases with larger pituitary adenoma experienced by the authors, no severe inter nal carotid artery stenosis has been observed. Tu mor compression of carotid arteries was therefore unlikely in these cases. There were neither stigmata of neurofibromatosis nor any history of irradiation. Therefore, these two cases can be classified as moyamoya disease since other possible mechanisms causing bilateral carotid artery stenoses were absent and the angiographic appearance was compatible with moyamoya disease. Brain tumor and moyamoya vessels occurring coin cidentally is rare. Kaku et al.") and Mizukawa and Iwatsuki19) reported meningiomas not causing stenoses of the internal carotid arteries accompanied by moyamoya vessels. Hattori et al.') reported a 12 year-old boy with cerebellar astrocytoma, who devel oped moyamoya vessels 3 months after operation. They considered the severe bilateral stenoses of the internal carotid arteries with ample collateral circula tion were caused by chemical irritation following iophendylate ventriculography or postoperative bacterial meningitis. In our cases, is the combination of brain tumor and moyamoya disease really a coincidence? The

general incidence of moyamoya disease is 0.1/ 100,000 population/year.") Therefore, the occur rence of these two moyamoya cases among 363 pituitary adenomas experienced by us over the last 15 years is significant (p < 0.001). Hyperprolactinemia has not been reported to induce cerebrovascular oc clusive disease. The hypothalamic disturbance (the PRL-inhibiting factor) caused by moyamoya disease may have induced the hyperprolactinemia, resulting in secondary prolactinoma. However, the menstrual cycles and blood PRL concentrations of seven child bearing age female moyamoya cases now being followed by us are normal (PRL 2.9-13.0 ng/ml) ex cept for these two cases. No correlation between moyamoya disease and hypothalamopituitary dysfunction has been reported, probably because the diencephalic blood flow is usually preserved by the ample collateral circulation at the base of the brain in moyamoya disease. However, since ischemic retinopathy rarely developed in moyamoya disease, 11,13) hypoperfusion in part of the hypothalamic region might be present. Meticulous endocrinologi cal evaluation may therefore reveal the real in cidence of disturbance of the hypothalamopituitary axis in moyamoya disease.

1985

9)

10)

11)

12)

13)

14)

15)

16)

References 17) 1)

2)

3) 4)

5)

6)

7)

8)

Darmody WR, Thomas LM, Gurdjian ES: Post-ir radiation vascular insufficiency syndrome. Neurology (Minneap) 17: 1190-1192, 1967 Debrun G, Sauvegrain J, Aicardi J, Goutieres F: Moyamoya, a nonspecific radiological syndrome. Neuroradiology 8: 241-244, 1975 du Boulay GH, Gemmal TEI, Trickey SE: True and false carotid retina. Br J Radiol 46: 205-213, 1971 Fukai H, Sato S, Ueki K: Evaluation of a group of cases presenting an abnormal vascular network at the base of the brain. No To Shinkei 18: 519-525, 1966 (in Japanese) Hattori M, Tokunaga J, Toya S, Shizawa T: An oc clusion of the circle of Willis after the removal of cerebellar astrocytoma. No To Hattatsu 5: 319-324, 1973 (in Japanese) Hilal SK, Solomon GE, Gold AP, Carter S: Primary cerebral arterial occlusive disease in children. Part 2: Neurocutaneous syndromes. Radiology 99: 87-93, 1971 Hiramoto C, Suyama K, Tsutsumi K: A case of recur rent optic glioma presenting with moyamoya vascularity. No Socchu 12: 171-176, 1990 (in Japanese) Hirata H, Matsukado Y, Mihara Y, Kochi M, Sonoda H, Fukumura A: Occlusion of the internal carotid artery after irradiation therapy for the chiasmal lesion. Acta Neurochir (Wien) 74: 141-147,

18)

19)

20)

21)

22)

23)

24)

25)

Ishibashi Y, Okada H, Mineura K, Kodama N: A case of radiation necrosis with vascular changes on main cerebral arteries. No Shinkei Geka 10: 337-341, 1982 (in Japanese) Ishikawa M, Handa H, Mori K, Matsuda I: Moyamoya” vessels on the tumor in the sellar“ region. Nippon Geka Hokan 48: 639-644, 1979 (in Japanese) Kaku M, Nakayama T, Matsukado Y: A case of me ningioma accompanied with abnormal vascular net work at the base of the brain. Shujutsu 27: 1159 1162, 1973 (in Japanese) Klatte EC, Franken EA, Smith JA: The radiographic spectrum in neurofibromatosis. Semin Roentgenol 11: 17-33, 1976 Klaus VE, Nekula J, Minarikova E, Farkova H: Neurofibromatosis v. Recklinghausen mit intra kranialer Stenose der A. Carotis interna bei orbito sphenoidaler Knochendysplasie. Fortschr Roent genstr 129: 250-252, 1978 Kudo T: Spontaneous occlusion of circle of Willis. A disease apparently confined to Japanese. Neurology (Minneap) 18: 485-496, 1968 Lamas E, Laboto RD, Cabello A, Abad JM: Multi ple intracranial arterial occlusions (moyamoya disease) in patients with neurofibromatosis. Acta Neurochir (Wien) 45: 133-145, 1978 Lee KF, Hodes PJ: Intracranial ischemic lesions. Radiol Clin North Am 5: 363-393, 1973 Levisohn PM, Mikhael MA, Rothman SM: Cerebrovascular changes in neurofibromatosis. Dev Med Child Neurol 20: 789-793, 1978 Miyamoto S, Kikuchi H, Karasawa J, Nagata I: Visual disturbances in moyamoya disease. No To Shinkei 38: 765-772, 1986 (in Japanese) Mizukawa N, Iwatsuki K: A case of cerebral rete mirabile combined with brain tumor. Rinsho Shinkeigaku 13: 609-616, 1973 (in Japanese) Montanera W, Chui M, Hudson A: Meningioma and occlusive vasculopathy: Coexisting complications of past extracranial radiation. Surg Neurol 24: 35-39, 1985 Mori K, Takeuchi J, Ishikawa M, Handa H, Toyama M, Yamaki T: Occlusive arteriopathy and brain tumor. J Neurosurg 49: 22-35, 1978 Nishimoto A, Takeuchi S: Abnormal cerebrovas cular network related to the internal carotid ar teries. J Neurosurg 29: 255-260, 1968 Okimura Y, Sunada S, Oka N, Yamaura A: Neurofibromatosis with occlusion of internal carotid artery and malignant astrocytoma. Case report. Neurol Med Chir (Tokyo) 27: 1208-1213, 1987 (in Japanese) Okuno T, Prensky AL, Gado M: The moyamoya syn drome associated with irradiation of an optic glioma in children: Report of two cases and review of the literature. Pediatr Neurol 1: 311-316, 1985 Painter MJ, Chutorian AM, Hilal SK: Cerebro

26)

27)

28)

29) 30) 31)

vasculopathy following irradiation in childhood. Neurology (Minneap) 25: 189-194, 1975 Rajakulasingam K, Cerullo LJ, Raimondi A: Childhood moyamoya syndrome. Postradiation pathogenesis. Childs Brain 5: 467-475, 1979 Rosengren K: Moya-moya vessels. Collateral arteries of the basal ganglia. Malignant occlusion of the anterior cerebral arteries. Acta Radiol [Diagn] (Stockh) 15: 145-151, 1974 Servo A, Puranen M: Moyamoya syndrome as a com plication of radiation therapy. J Neurosurg 48: 1026 1029, 1978 Suzuki J: Moyamoya Disease. Tokyo, Igaku-Shoin, 1983, pp 132-136 (in Japanese) Suzuki J, Takaku A: Cerebrovascular moyamoya disease. Arch Neurol (Chicago) 20: 288-299, 1969 Toyama M, Yamaki T, Naruse S: Moyamoya follow ing irradiation for glioma in the basal ganglia. Shoni No Noshinkei 2: 215-220, 1977 (in Japanese)

32) Yamaguchi T, Tashiro M, Minematsu K, Kitamura K: The summary of clinical study of spontaneous oc clusions of circle of Willis, in: The Report of Ad'hoc Committee of Japanese Health and Welfare Ministry. 1980, pp 13-22 (in Japanese) 33)

34)

Yoshida T, Sakai T, Ikeda N, Abe H, Ichikawa A: Impairment of visual function in moyamoya disease. Shinkei Ganka 3: 163-168, 1986 (in Japanese) Zawadzki MB, Anderson M, De Armond S, Conley FK, Jahnke RW: Radiation induced large in tracranial vessel occlusive vasculopathy. AJR 134: 51-55, 1980

Address of

reprint

Medicine, 734,

requests

Neurosurgery, Japan.

1-2-3

to: K. Arita, Hiroshima Kasumi,

M.D.,

University Minami-ku,

Department School Hiroshima

of

Moyamoya disease associated with pituitary adenoma--report of two cases.

Moyamoya disease associated with prolactin (PRL)-producing pituitary adenomas occurred in two females with elevated blood PRL levels (285 and 120 ng/m...
512KB Sizes 0 Downloads 0 Views