Moyamoya disease associated with intracranial aneurysms

A case of moyamoya disease associated with multiple intracranial aneurysms is reported. The aneurysms were located at the bifurcation of the basilar artery and at the precommunicating segment of the right posterior-cerebral artery. They were found to be unruptured and clipped successfully through a right frontotemporal craniotomy. Outcome of the operation was satisfactory. We propose the operative indication for these cases on the basis of operative and radiological findings, and also discuss characteristic aspects of this disease.     

巨大垂体腺瘤的治疗策略

目的通过回顾性分析32例巨大垂体腺瘤病例的临床资料，探讨其治疗策略。方法男性17例，女性15例，平均年龄46．7岁，平均病程4．7年。其中垂体生长激素(GH)腺瘤1例，泌乳素(PRL)腺瘤6例，无功能腺瘤25例。采用经口鼻蝶窦入路手术24例，经额下人路手术1例，经蝶窦和经颅分期手术6例，药物治疗1例。结果手术显微镜下肿瘤全部切除20例，7欠全切除9例，部分切除2例；手术后放疗10例，药物治疗5例。术后死亡1例。随访6个月～4年，影像学检查显示肿瘤全部切除15例，次全切除14例，部分切除2例；肿瘤无复发。结论对巨大垂体腺瘤，应根据患者的临床表现、内分泌学检查和肿瘤的生长方式决定治疗方案；大多数巨大垂体腺瘤可以首选经蝶窦入路手术，并发症较少，死亡率低；对侵袭性生长的肿瘤，应采用手术、放疗和药物综合治疗。     

Treatment strategy for patients with unruptured intracranial aneurysms.

The increased sensitivity of neuroimaging techniques has enabled the more frequent diagnosis of unruptured aneurysms. Because the most devastating complication of an unruptured aneurysm is subarachnoid hemorrhage, it has been considered desirable to treat these aneurysms before they rupture. However, the optimal treatment strategy for patients with unruptured aneurysms remains controversial. The management decision requires knowledge of the natural history and an accurate assessment of the risks related to various treatment options. On December 10, 1998 the New England Journal of Medicine published an article by the International Study of Unruptured Intracranial Aneurysms Investigators. The study retrospectively assessed the natural history of unruptured aneurysms in 1449 patients; in addition, treatment-related mortality and morbidity were examined in a prospective cohort of 1172 patients. The major finding was that the rate of rupture for aneurysms smaller than 10 mm in diameter in patients with no history of subarachnoid hemorrhage was 0.05% per year, and that the mortality and morbidity associated with surgery greatly exceeded the risk of rupture in such patients. This report has generated tremendous controversy in the treatment of unruptured aneurysms and has influenced our decision making. The results of this study are critically analyzed and potential flaws presented. To develop recommendations for treatment, the literature was reviewed. The conclusion of this search is that there is insufficient evidence to recommend a standard of management. Therapeutic guidelines are provided, particularly for patients with small incidental aneurysms and no history of subarachnoid hemorrhage.     

A case of intracranial chordoma associated with pituitary adenoma.

We report a patient who had pituitary adenoma and parasellar chordoma coincidentally, with neither irradiation nor a history of head injury. Preoperative computed tomographic (CT) scan had shown an intrasellar mass that infiltrated into the right cavernous sinus. Histopathologically the intrasellar mass was diagnosed as pituitary adenoma and the other mass as chordoma. We present clinical, radiological and histopathological findings, and discuss previously reported cases with multiple intracranial tumours.     

Vascular abnormalities in the neck associated with intracranial aneurysms

In 102 cases of ruptured intracranial aneurysms, the cervical as well as the cranial areas were explored by angiography. Loops in the course of the cervical vessels were present in 28 patients; features of fibromuscular dysplasia were present in 31 patients; and in 50 patients, no abnormalities were observed. In 7 patients, both cervical anomalies were present. Loops were associated with single aneurysms (95%), located primarily on the anterior communicating artery (76%), predominantly in men (M/F = 1.6). Aneurysms with fibromuscular dysplasia affected women more than men (F/M = 7), were frequently multiple (58%), and were located mainly on the intracranial part of the internal carotid and vertebral arteries (51%). These data suggest new concepts of aneurysm formation from inherited diseases and should permit the detection of some aneurysms before rupture.     

Characteristics of intracranial aneurysms associated with moyamoya disease

Summary A retrospective analysis of 111 patients with aneurysms associated with Moyamoya disease is presented. The subjects comprised of our 12 cases and 99 other well-documented cases. These 111 cases had 131 aneurysms. There were 48 males and 63 females. The average age was 40.3 years. The clinical manifestations were intracranial haemorrhage in 99 cases (89%), and ischaemic events in 9 cases (8%), but no mention was made of these in the last three cases (2%). The Hunt and Kosnik grades were grade 1 in 8%, grade 2 in 23%, grade 3 in 31%, grade 4 in 35%, and grade 5 in 3%. Of the 131 aneurysms, 73 (56%) were found distributed around the circle of Willis, 24 (18%) in the basal ganglia, 29 (22%) on collateral vessels, and 5 (4%) on other vessels. Forty-six percent of the cases were treated surgically, 51% conservatively, and 3% by endovascular procedures. The surgical procedures for the aneurysms were; neck clipping in 49%, aneurysmectomy in 18%, wrapping of the aneurysm in 11%, coating or cautery of the aneurysm in 7%, and revascularization only in 11%. The outcomes were Glasgow Outcome Scale 1 in 30%, 2 in 22%, 3 in 11%, 4 in 1%, and 5 in 25%. The main reasons for the unfavourable outcome were initial poor clinical grade and rebleeding. Follow-up angiography of 25 aneurysms demonstrated that all aneurysms in the basal ganglia or on the collateral vessels disappeared. We recommend surgical intervention for aneurysms associated with Moyamoya disease to prevent rupture or rebleeding, especially for aneurysms around the circle of Willis. However, direct surgery is not recommended for aneurysms found in the basal ganglia or on the collateral vessels.     

A case of systemic lupus erythematosus associated with multiple intracranial aneurysms

The author reports a case of systemic lupus erythematosus (SLE) with multiple intracranial aneurysms and subarachnoid hemorrhage. A 31-year-old woman was admitted to the department of internal medicine of Shizuoka General Hospital for the treatment of nephrotic syndrome due to systemic lupus erythematosus on 1984. She spend an uneventful life until April, 1985 when she suddenly complained of severe headache and nausea. The computed tomographic scan revealed subarachnoid hemorrhage and the cerebral angiography showed multiple intracranial aneurysms and stenotic lesion. A review of the literature was made to clarify the incidence, the pathological change, and the prognosis of systemic lupus erythematosus with subarachnoid hemorrhage. The incidence of subarachnoid hemorrhage in SLE was about two percent in the reported clinical cases. The mechanisms of the subarachnoid hemorrhage and the aneurysmal formation in SLE seemed to be due to angitic changes, which involved the vessels of the whole body systematically. The exact prognosis of SLE with subarachnoid hemorrhage is difficult to say, but it seems to be unfortunately poor due to multiplicity of the lesion and the difficulty of its treatment.     

Incidence of intracranial aneurysm associated with pituitary adenoma

The incidence of in tracranial aneurysm associated with pituitary adenoma is not definitely established although reported higher than in general population. This study was designed to find the existence of such association in a large series of pituitary adenoma cases.A retrospective study of 467 cases of pituitary adenoma (mean age: 41 ± 15 years) was done. All patients underwent cerebral angiography at least of anterior circulation, detailed hormonal study, and 155 cases had additional magnetic resonance (MR) angiography. Twenty-five cases (5.4%) of pituitary adenoma (mean age 52 years) had intracranial aneurysm, 97% on anterior circulation, and 12% had multiple aneurysms. Two cases presented with aneurysmal rupture and the rest were incidental. Aneurysm was more frequently seen with increasing age (p < 0.001) and the age distribution resembled that of aneurysm among general population. Although the combination was most frequent among nonfuctioning adenoma (8.8%), and least frequent among prolactinoma (2.4%), this association was again due to age factor. There was no association between hormone secretion, size and invasive nature of the tumor. The results showed no association between intracranial aneurysm and pituitary adenoma. Our speculation is that such occurrence is merely a chance factor and the risk is no greater than that among general population.     

Bacterial intracranial aneurysms associated with infectious endocarditis--report of 3 cases

Three cases of bacterial intracranial aneurysms associated with infective endocarditis are reported. All of the patients were successfully treated by various combinations of cardiac surgery, neurosurgery and chemotherapy with antibiotics. Case 1: A 39-year-old female was admitted with the complaint of sudden onset of severe headache following persistent fever of several month's duration. CT scan revealed a left frontal old hematoma and angiography detected an aneurysm located in the frontal ascending branch of the left middle cerebral artery. After 2 weeks' antibiotic therapy, the second angiography showed the aneurysm to be decreased in size. The third angiography, performed 2 weeks after cardiac valve replacement for infective endocarditis, demonstrated complete resolution of the aneurysm. Case 2: A 19-year-old male entered hospital with high fever and chills. In addition to infective endocarditis, CT scan and angiography revealed an aneurysm arising from the distal portion of the left posterior cerebral artery. The patient was treated with high dose antibiotics and then his general condition improved. However, angiography examined 4 weeks after the initial study demonstrated the aneurysm to be apparently enlarged. Therefore, the aneurysm was excised before cardiac surgery. Repeated angiography after valve replacement showed no further aneurysm. Case 3: A 30-year-old female was admitted on the diagnosis of infective endocarditis and meningitis. CT scan showed abnormal density areas in the right frontal lobe and the left temporal lobe.(ABSTRACT TRUNCATED AT 250 WORDS)     

MIB1 immunopositivity is associated with rapid regrowth of pituitary adenomas

Summary Pituitary adenomas are generally regarded as benign tumours, but they may recur. We identified eight patients with pituitary adenomas that showed rapid regrowth within 2 years of initial surgery. We estimated the percentage of cells in each specimen that showed positive immunostaining for MIB1 (a novel anti-Ki-67) and compared the values to those of 40 adenomas that showed no regrowth. The mean MIB1 index for 40 adenomas that showed no evidence of regrowth was 0.19±0.06%. This was significantly (p<0.0001) lower than that for adenomas that showed rapid regrowth (1.27±0.31%), based on the initial resected specimens. Immediately after detection of rapid regrowth and in adenomas that were resistant to bromocriptine or irradiation, the MIB1 index was always greater than 1.0%. Most patients with rapidly regrowing adenomas were well controlled by radiation therapy.Our results suggest that a MIB1 index greater than 1.0% may be a useful predictor of rapid regrowth of pituitary adenomas and may be useful for planning of therapy.     

Giant intracranial aneurysms: morphology and clinical presentation

The purpose of this study was to correlate the morphology of giant intracranial aneurysms (GIA) with their clinical presentation. Eighty patients with GIA, 14 males and 66 females, were studied. Univariate and multivariate analyses were made to test the associations between morphological and clinical features. The main locations of the unruptured GIA included the carotid cavernous segment, and for the ruptured GIA, the most frequent were the carotid supraclinoid and middle cerebral arteries. There was a significant association among communicating arteries (CA) of “bad” quality and presence of thrombus and calcification (TC). The risk of rupture is 8 times higher in patients with CA of “bad” quality and 11 times higher in patients without TC. GIA are more frequent in the cavernous segment. There is a high rupture risk in the middle cerebral artery. CA of “bad” quality are associated with TC. The rupture risk is significantly higher in patients without TC.     

Giant pituitary adenomas: clinical characteristics and surgical results

Although most pituitary neoplasms are benign, some grow rapidly, spreading to extrasellar tissues. Definition of these 'giant' pituitary adenomas (PAs) is not clear. In order to clarify this question, we studied all pituitary macroadenomas operated in our hospital during the last 20 years, differentiating those with diameter > or = 3 cm (n = 43) and attempting to identify their typical features and prognosis. The reason for consultation was local in 58.3% of giant PAs, hormonal in 22.9% and tumour recurrence in 16.7%. Surgery was performed via a trans-sphenoidal approach in 92.5% of cases and succeeded in completely removing the tumour in only 27% of cases. In conclusion, we found that giant PAs are not a special type but the extreme case in the gradient of invasiveness of tumours of adenohypophyseal origin. They are characterized by a higher frequency of neuro-ophthalmological symptoms and hormonal deficits, and poorer response to surgical treatment.     

Multiple adenomas of the human pituitary. A retrospective autopsy study with clinical implications

In a review of autopsy material from two centers, 20 pituitary glands were found containing multiple adenomas. In total, 44 adenomas were identified histologically; 16 glands contained double tumors and in four glands triple adenomas were found. Size was measured in 30 tumors, all of which were microadenomas. Thirty-four adenomas were located in the lateral wings and 10 lay in the median wedge. Forty-one tumors were chromophobic and three were basophilic. Immunocytochemical analysis of the 44 tumors demonstrated the presence of prolactin in 11, adrenocorticotropic hormone in three, growth hormone in one, and alpha-subunit as well as follicle-stimulating hormone and luteinizing hormone in one. Of the 20 patients studied, there were 11 men and nine women, with an average age of 69 years. All patients died from various nonendocrine causes. With the exception of one patient who appeared mildly acromegalic, no correlation was observed between pituitary morphology and clinical data. This study found a 10.4% frequency of adenomas in pituitaries studied randomly at autopsy. Multiple tumors were encountered in 0.9% of cases. Despite its low frequency, adenoma multiplicity may underlie surgical failure in cases in which one adenoma is removed and the other is left behind.     

垂体腺瘤经蝶显微手术的疗效分析

目的 探讨经蝶显微手术治疗垂体腺瘤的临床综合疗效、手术技巧、新技术应用等。方法 对于1997～2001年经蝶显微手术治疗的1462例垂体腺瘤患者的临床资料、影像学、内分泌及病理检查结果、肿瘤切除率、术后并发症以及随访结果进行回顾性总结和分析。结果 Hardy Ⅰ级的肿瘤全切率已达97．0％、Ⅱ级95．2％、Ⅲ级90．5％、Ⅳ级47．4％;术后患者临床症状及内分泌功能有显著改善,肿瘤复发率低(0．3％)。结论 随着神经显微操作技术的日益娴熟及新技术的应用,经蝶入路手术指征不断扩大,内窥镜和(或)神经导航辅助显微外科经鼻-蝶窦入路手术,是垂体腺瘤的首选治疗方法;肿瘤全切除的患者术后不必行常规放疗。     

多聚唾液酸神经细胞黏附分子在垂体腺瘤中的表达及其临床意义

目的 探讨垂体腺瘤中多聚唾液酸神经细胞黏附分子(PSA-NCAM)的表达及与侵袭性、组织学类型、Ki-67抗原之间的关系.方法 收集61例手术切除的垂体腺瘤标本及临床资料,3例正常垂体腺组织来自24 h内死亡的尸解标本,应用免疫组织化学法检测PSA-NCAM在不同类型腺瘤中的表达.结果 本组61例垂体瘤中有26例(52.6%)PSA-NcAM阳性表达,3例正常垂体组织皆为阴性.25例男性患者中11例(44.0%)PSA-NCAM阳性表达,36例女性患者中15例(41.7%)PSA-NCAM阳性表达,两者差异无统计学意义(P>0.05).PSA-NCAM阳性表达组的平均年龄(42.1±11.2)岁,阴性表达组为(44.7±12.4)岁,两者差异无统计学意义(P>0.05).PSA-NCAM阳性表达组的平均Ki-67抗原标记指数(Ki-67 LI)为(3.6±1.3)%,阴性表达组为(2.7±1.4)%,两者差异有统计学意义(P<0.05).PSA-NCAM阳性表达组的肿瘤平均宽度为(2.5±0.9)cm,阴性表达组的为(1.6±1.2)cm,差异有统计学意义(P<0.05).侵袭性垂体腺瘤的PSA-NCAM阳性表达率62.5%(20/32),非侵袭组20.7%(6/29),两者差异有统计学意义(P<0.01).结论 PSA-NCAM的表达与垂体腺瘤的大小、侵袭性、增殖指数密切相关,而与患者的性别、年龄等无明显相关.     

Ectopic pituitary adenomas with normal anterior pituitary glands

Two patients with ectopic pituitary adenomas and biopsy-proven normal anterior pituitaries are described. Both tumors were located in the sphenoid sinus. One tumor produced prolactin, and the other one was a plurihormonal adenoma that produced predominantly adrenocorticotropin and to a lesser extent thyroid stimulating hormone and alpha subunit. The patient with the plurihormonal tumor, who had Cushing's disease, was cured by surgery while the patient with the prolactinoma was treated by surgery and medical therapy. A review of these two cases and an additional nine cases from the literature of ectopic pituitary adenomas in patients with normal intrasellar anterior pituitaries indicate that these uncommon tumors are capable of secretory function and may be the only cause of excessive pituitary hormone production.