Classical pituitary tumour apoplexy: clinical features, management and outcomes in a series of 24 patients

We retrospectively analysed the incidence, clinical presentation, endocrinological and radiological findings, medical and surgical management of pituitary apoplexy in our department (single-centre study), having a large experience in pituitary surgery. Among 1540 pituitary lesions, 24 patients presented with pituitary apoplexy. Their charts were retrospectively reviewed. The symptoms included headache (92%), nausea and vomiting (54%), visual deficit (50%), oculomotor paresis (54%) and/or an altered mental state (42%). Skull X-rays (n = 14) demonstrated an enlarged sella turcica in all cases; CT-scan and/or MRI always revealed a sellar and suprasellar expanding lesion. Panhypopituitarism was present on admission in 70% of the patients. Urgent therapeutic management included high-dose cortisone treatment in all but one patients and CSF drainage in three. Three patients were treated conservatively. Nine patients were operated on rapidly, within hours or a few days because of severe visual deficit and/or altered level of consciousness. Nineteen patients were operated by the trans-sphenoidal approach; one of them required a second operation by craniotomy. There were two deaths related to the illness and one to an ill-defined reason at 4 months. Among the other patients 95% made a good recovery. All but two patients required a substitutive treatment with adrenal (83%), thyroid (68%), gonadal (42%) and/or growth (16%) hormones. The preoperative visual deficits recovered in all but one patients (92%) whereas the oculomotor pareses improved in all but two patients (85%). In conclusion, pituitary tumour apoplexy is a rare event, complicating in our series 1.6% of 1540 pituitary adenomas. Even in severe cases, complete recovery is possible if the diagnosis is rapidly obtained and adequate management is initiated in time. Surgical results after trans-sphenoidal approach are in the majority of cases very satisfactory.     

Pituitary apoplexy as a consequence of lymphocytic adenohypophysitis in a pregnant woman: a case report

OBJECTIVE AND IMPORTANCE: A patient with pituitary apoplexy resulting from lymphocytic adenohypophysitis, which caused visual disturbance during pregnancy, is described. This is the first report of such case. CLINICAL PRESENTATION: A 23-year-old primigravida in her 25th week of gestation experienced headache and bitemporal hemianopsia of sudden onset. Magnetic resonance imaging (MRI) revealed a large pituitary mass with intratumoral hemorrhage. Although conservative treatment with intravenous glycerol improved the symptoms partially, the visual symptoms worsened again 6 weeks later. After delivering a girl by scheduled caesarean section her visual symptoms improved. Despite the symptomatic improvement, MRI showed the chiasmatic compression by the enlarged pituitary gland had not changed. Therefore, trans-sphenoidal surgery to decompress the chiasm was performed. Necrotic tissue was seen exuding behind the enlarged pituitary gland and adenohypophysitis with bleeding (apoplexy) was diagnosed histologically. After follow-up for 40 months, she was doing well without any visual or neurological deficits. CONCLUSION: Although relatively rare, pituitary apoplexy as a consequence of lymphocytic adenohypophysitis should be borne in mind when a pregnant woman presents with headache and visual disturbance of sudden onset.     

Late recurrent bleeding after surgical treatment for pituitary apoplexy.

Pituitary apoplexy is an uncommon syndrome characterised by sudden onset of headache, meningeal signs, visual disturbances, ophthalmoplegia and confusion. Documented recurrent apoplexy or treated apoplexy is even rarer with only few reports in the literature. Between 1994 and 2001, 18 patients were treated for pituitary apoplexy at Bayindir Medical Centre through transsphenoidal route. In all, topical bromocriptine was applied after tumour resection as described by Ozgen. We hereby present the cases of two patients with recurrent apoplexy 3 and 7 years after the initial surgical treatment for pituitary adenoma with apoplexy. The patients were treated non-surgically with success. Additional treatment in the form of radiosurgery was found necessary for the first patient. Surgical excision of the pituitary tumours with apoplexy reduces the risk of recurrent bleedings but eradication is not a rule. These patients need to be followed closely in the postoperative period for possible recurrence of bleeding.     

Spontaneous haemorrhage into an empty sella turcica mimicking pituitary apoplexy

We present a case of spontaneous haemorrhage into an empty sella turcica with the features of subclinical pituitary apoplexy. A 66-year-old woman with a previously resected pituitary adenoma presented four months later with progressive headache and visual deterioration. Cranial MRI demonstrated hyperacute blood products in a recurrent pituitary adenoma. Operative findings were of subacute blood in an empty sella turcica. There was no operative or subsequent histological evidence of tumour recurrence. The intrasellar haemorrhage was evacuated via a trans-sphenoidal approach, resulting in a rapid improvement in visual function. Endocrine deficits required thyroxine, corticosteroid and desmopressin supplementation. Haemorrhage into an empty sella turcica has not been previously described and needs to be suspected as a clinical entity in patients presenting with the features of pituitary apoplexy. Awareness of this clinical condition will prevent preoperative misdiagnosis.     

Radiological Apoplexy and Its Correlation with Acute Clinical Presentation, Angiogenesis and Tumor Microvascular Density in Pituitary Adenomas

Objective

Pituitary apoplexy is life-threatening clinical syndrome caused by the rapid enlargement of a pituitary tumor due to hemorrhage and/or infarction. The pathogenesis of pituitary apoplexy is not completely understood. We analyzed the magnetic resonance imaging (MRI) of pituitary tumors and subsequently correlated the radiological findings with the clinical presentation. Additionally, immunohistochemistry was also performed to determine whether certain biomarkers are related to radiological apoplexy.

Methods

Thirty-four cases of pituitary adenoma were enrolled for retrospective analysis. In this study, the radiological apoplexy was defined as cases where hemorrhage, infarction or cysts were identified on MRI. Acute clinical presentation was defined as the presence of any of the following symptoms: severe sudden onset headache, decreased visual acuity and/or visual field deficit, and acute mental status changes. Angiogenesis was quantified by immunohistochemical expression of fetal liver kinase 1 (Flk-1), neuropilin (NRP) and vascular endothelial growth factor (VEGF) expression, while microvascular density (MVD) was assessed using Endoglin and CD31.

Results

Clinically, fourteen patients presented with acute symptoms and 20 for mild or none clinical symptoms. Radiologically, fifteen patients met the criteria for radiological apoplexy. Of the fifteen patients with radiologic apoplexy, 9 patients presented acute symptoms whereas of the 19 patient without radiologic apoplexy, 5 patients presented acute symptoms. Of the five biomarkers tracked, only VEGF was found to be positively correlated with both radiological and nonradiological apoplexy.

Conclusion

While pituitary apoplexy is currently defined in cases where clinical symptoms can be histologically confirmed, we contend that cases of radiologically identified pituitary hemorrhages that present with mild or no symptoms should be designated subacute or subclinical apoplexy. VEGF is believed to have a positive correlation with pituitary hemorrhage. Considering the high rate of symptomatic or asymptomatic pituitary tumor hemorrhage, additional studies are needed to detect predictors of the pituitary hemorrhage.     

Acute hemorrhage and ischemic necroses in hypophyseal tumors: hypophyseal apoplexy

Acute enlargement of pituitary adenomas due to haemorrhage or ischaemic necrosis in the tumour was described as "pituitary apoplexy" by Brougham et al. in 1950. Since then, more than 200 cases have been reported, but--especially in the German literature--the syndrome has caught only little attention. Therefore, in a series of 12 own patients, typical findings and clinical characteristics are demonstrated and the literature is discussed. 9 patients had a haemorrhage into the tumour, 3 an acute ischaemic necrosis. The guiding symptom was the acute onset with ophthalmoplegia (11 of 12 patients). Only in one case the adenoma was known before the apoplexy. Other symptoms were headache, blurred vision, drowsiness and, in severe cases, hemiparesis, coma, and hypothalamic disorder. Most important is the acute endocrinological substitution with hydrocortisone; this may be life-saving. Neuroophthalmological recovery depends on early operation: cases of oculomotor palsy require an operation within the first 2 weeks after the acute event. An emergency operation is required only by an acute amaurosis. In general there will be enough time for careful clinical endocrinological and radiological investigations.     

Pituitary adenomas: presentations and outcomes in a South Asian country

OBJECTIVE: The purpose of this retrospective review of all operated cases of pituitary adenomas in the last decade, is to define the demographic patterns and characteristics of such tumors and to assess surgical outcomes with regards to safety and efficacy of trans-sphenoidal tumor removal in our institution. METHODS: Surgically treated pituitary adenomas presenting from 1995 till 2005 were reviewed for different variables. Results were expressed as mean, standard deviation and median for continuous and number with percentage for categorical data. Chi square test was applied to measure differences and significance was taken atp value < 0.05. RESULTS: One hundred and twenty-five patients were operated for pituitary adenoma. Sixty-three percent were male and mean age was 37 years. Sixty percent of the patients presented with headache and/or visual symptoms. Twelve percent presented with pituitary apoplexy and 28% presented with symptoms due to pituitary hyperfunction. Fifty-five percent of patients had functioning and 44% had nonfunctioning adenomas. Mean pre operative tumor diameter from 86 pre op MRI scans was 26.76 mm (3-78 mm). Eighty-four percent of patients underwent trans-sphenoidal tumor resection and three percent had craniotomy. Mean size of post op residual tumor as calculated from 76 available post operative scans was 5.3 mm (range 0-31 mm). 17.6% of the patients required hormone replacement beyond three months and 10% were re-operated. Overall mortality was 1.6%. CONCLUSION: In Pakistan, patients are more likely to present either with apoplexy or with a giant pituitary adenoma than patients reported from developed countries. Overall, our results have been satisfactory and comparable with the literature.     

Delayed pituitary apoplexy in patient with advanced prostate cancer treated with gonadotrophin-releasing hormone agonists

Gonadotrophin-releasing hormone agonists (GnRHAs) are used in many clinical conditions, particularly prostate cancer. There have been a few case reports of apoplexy from a previously undiagnosed pituitary tumour, occurring within hours to days of initiation of GnRHA therapy. We report a case of delayed onset pituitary apoplexy following GnRHA therapy. A 71-year-old man presented three weeks after onset of headache and vision loss. On examination, he was blind in the right eye with an intact nasal field of vision in the left eye. Two months before presentation, he had a subcutaneous GnRHA (Goserelin) implant for treatment of locally advanced prostate cancer (Gleeson 4+3). An MRI scan revealed a large sellar/suprasellar mass. His follicle stimulating hormone (FSH) and luteinizing hormone (LH) levels were grossly elevated. A trans-sphenoidal endoscopic decompression of the pituitary tumour was performed. His vision improved post-operatively and his FSH, LH, testosterone, prostate specific antigen (PSA) levels returned to normal levels. Histopathologic studies revealed a pituitary adenoma, which stained positive for FSH and LH. The prostate cancer management was changed to an anti-androgen agent and a GnRH antagonist. This case demonstrates that pituitary apoplexy can develop up to eight weeks after the initiation of treatment for prostate cancer with GnRHAs.     

Acute monocular disturbances mimicking optic neuritis in pituitary apoplexy

Three patients with acute monocular central scotoma and headache had the initial diagnosis of optic neuritis. Computed tomography showed large pituitary adenomas with necrosis and cyst formation consistent with the clinical symptoms of a previous pituitary apoplexy. After transsphenoidal adenomectomy the visual disturbances disappeared.     

Idiopathic granulomatous hypophysitis presenting as pituitary apoplexy

Idiopathic granulomatous hypophysitis (IGH) is an extremely rare chronic inflammatory lesion of the pituitary gland. This condition typically presents with chronic onset of headache and slow development of visual deficits. Pituitary apoplexy is a clinical syndrome characterized by sudden onset of headache, vision loss, opthalmoplegia, and signs of meningeal irritation. Although IGH has been previously described in the literature, its presentation as clinical pituitary apoplexy is novel. We report, to our knowledge, the first patient with IGH manifesting as clinical pituitary apoplexy. Physicians involved in the treatment of pituitary disease should be aware of this rare entity.     

Colloid cysts of the pituitary gland

Colloid cysts of the pituitary gland are very rare pathological lesions occurring in sellar region. Their pathogenesis is not clear. They are located between the anterior and posterior lobe of the pituitary. Colloid cysts of the pituitary gland are space occupying lesions and induce hypopituitarism, diabetes insipidus, visual disturbances, etc. They cause pituitary apoplexy too. Magnetic resonance imaging is the preferred neurodiagnostic method in evaluating these lesions. 503 transsphenoidal procedures were performed from January 1996 to February 2001. Hypothalamo-hypophyseal dysfunction was caused by colloid cyst in seven cases. The most frequent presenting symptoms were headaches, oligomenorrhea and loss of libido. Diabetes insipidus was found in two cases before surgery. Colloid cyst induced pituitary apoplexy in one patient. Two patients presented visual disturbances. All our patients were operated on. We performed six transsphenoidal procedures and one transcranial operation. Postoperatively, the endocrine function of the pituitary gland was evaluated. We concluded that transsphenoidal operation is a safe method for treating colloid cyst located in the sellar region.     

Pituitary apoplexy. A review

Pituitary apoplexy constitutes a syndrome that is characterized by the ischemic infarction or hemorrhage into a pituitary tumour. Clinically the patient develops sudden headache, meningismus, visual disturbances, even blindness, and occasionally decrease in level of consciousness. To diagnose it is basic to perform a cerebral MRI. Treatment consists in urgent sellar decompression by transsphenoidal surgery and substitute therapy with steroids.     

Precipitating factors in pituitary apoplexy

Pituitary apoplexy is a rare but life threatening condition caused by sudden haemorrhage or infarction of the pituitary gland. Potential precipitating factors in the occurrence of acute pituitary apoplexy in 30 consecutive patients were identified and compared with the clinical characteristics and outcome of patients with and without associated factors. Six patients had a previously known pituitary adenoma. All patients complained of severe headaches, associated with neuro-ophthalmological symptoms and signs in 83% and altered mental status in 30%. Potential risk factors were identified in nine patients (30%). When there was an associated factor, the clinical presentation was no different than in patients without such factors although altered mental status may be more frequent in patients with associated diseases. In these patients, the visual prognosis was worse and the diagnosis was more difficult to establish. Acute pituitary apoplexy is unpredictable and should be considered in any patient with abrupt neuro-ophthalmological deterioration associated with headache. Patients with pituitary apoplexy often have an associated disease that confounds recognition and treatment despite a typical presentation.     

经鼻蝶入路显微手术治疗垂体卒中的临床分析

目的总结55例垂体卒中患者的临床表现特征，分析经鼻蝶入路显微手术的疗效。方法回顾性分析经鼻蝶入路显微手术治疗的55例垂体卒中患者的临床和影像学资料。结果本组55例垂体腺瘤中，48例全切除，7例次全切，肿瘤全切率为87．3％。51例视力障碍、视野缺损的患者中46例术后恢复正常（90．2％），5例恢复欠佳；32例伴有内分泌症状的患者中29例明显缓解（90．6％），3例无明显变化。术后一过性尿崩症7例，脑脊液漏5例。无死亡病例5例。结论根据突发头痛及视路症状等相关症状、体征下并结合影像学检查一般可确诊垂体卒中；经鼻蝶入路显微手术和／或内窥镜手术可做为治疗垂体卒中的首选方法。     

Giant necrotic pituitary apoplexy

Apoplexy of the pituitary gland is a rare complication of pituitary adenomas, involving hemorrhage with or without necrosis within the tumor. This condition may be either asymptomatic or may present with severe headache, visual impairment, ophthalmoplegia, and pituitary failure. Transsphenoidal surgery is the treatment of choice, and early intervention is usually required to ensure reversal of visual impairment. Reports of pituitary apoplectic lesions exceeding 60.0 mm in diameter are very rare. A 39-year-old man with long-standing history of nasal congestion, decreased libido and infertility presented with a sudden onset of severe headache and diplopia. MRI of the head demonstrated a massive skull base lesion of 70.0 × 60.0 × 25.0 mm, compatible with a giant pituitary macroadenoma. The lesion failed to enhance after administration of a contrast agent, suggesting complete necrotic apoplexy. Urgent surgical decompression was performed, and the lesion was resected via a transnasal transsphenoidal approach. Pathological analysis revealed evidence of necrotic pituitary apoplexy. At the 2 month follow-up, the patient had near-complete to complete resolution of his visual impairment. To the authors’ knowledge, this report is unique as the patient demonstrated complete necrotic apoplexy and it underlines the diagnostic dilemma in such a case.     

急性垂体腺瘤卒中的临床研究

目的　对急性垂体卒中的临床表现、诱发因素、辅助检查、治疗和结果进行分析讨论。方法　回顾性分析1996年1月至2001年12月经手术治疗的35例急性垂体卒中病人。男17例,女18例。年龄19～69岁,平均46.5岁。所有患者术前、术后均行内分泌激素及头颅CT和/(或)MRI检查。结果　头痛是急性垂体卒中最常见的临床症状,其次是视觉障碍,再次为眼肌麻痹。急性垂体卒中的可能诱发因素为高血压、糖尿病。CT和MRI检查对急性垂体卒中确诊率分别为26%和90%。经蝶手术22例,开颅手术13例,无手术死亡。无功能性垂体腺瘤最多见,占65.7%。在急性垂体卒中后7天内手术,术后视力、视野改善率较高(P<0.05)。术后随访时间平均为3.2年,均恢复良好,肿瘤复发2例。术后4例放疗。术后长期需要甲状腺激素、皮质类固醇激素、睾酮和抗利尿激素替代治疗者分别为51%、56%、34%(男)、和3%。结论　突发头痛是急性垂体卒中最常见的症状。高血压和糖尿病是急性垂体卒中的重要诱发因素,无功能腺瘤在急性垂体卒中最多见。MRI是急性垂体卒中首选的检查方法。经蝶入路手术减压是治疗垂体卒中安全有效的方法。在垂体卒中后7d内手术视力、视野改善更明显。术后放疗仅适用于肿瘤残留或复发者。     

Pituitary apoplexy, therapeutic assessment.

The management of pituitary apoplexy with reference to both diagnosis and operative sequelae remains a major challenge. Acute onset of retro-orbital headache in association with visual loss and ophthalmoplegia are the cardinal symptoms; however, obtundation and signs of subarachnoid hemorrhage also may be present. Good quality plain skull radiographs and complete angiography prove sufficient for preoperative radiographic studies. Preoperative endocrine preparation focuses on supplemental glucocorticoids since these patients must be presumed deficient in cortisol reserve. Residual visual deficit appears to be more a function of the extent of damage at the time of ictus rather than rapidity of decompression. Our experience indicates that transsphenoidal decompression in appropriate cases offers an ideal opportunity to minimize mortality and morbidity. The acute onset of severe retro-orbital headache in association with stupor and ocular palsies would alert most physicians to the potential diagnosis of spontaneous subarachnoid hemorrhage. The association of complex ophthalmoplegias and visual defects in this constellation of symptoms should, in addition, alert one to the possibility of an acute intrasellar or parassellar expansile process. During the past two years, we have had the opportunity to care for 8 such patients with confirmed diagnoses of acute hemorrhagic infarction of the pituitary enabling us to formulate diagnostic and therapeutic schemata with reference to management of this problem.     

Pituitary Apoplexy in a Teenager—Case Report

BackgroundPituitary apoplexy is a rare clinical emergency which results from hemorrhage or infarction in the pituitary gland.PatientWe present a 14-year-old girl with pituitary apoplexy and review the literature.ResultsOur patient experienced blurred vision, nausea, and headache. Her best-corrected visual acuity was 20/200 and 20/20. Confrontation test visual field testing revealed bitemporal hemianopsia. Brain imaging demonstrated a suprasellar mass. The microscopic endonasal transsphenoidal approach only found 5-10 mL brownish fluid-like material. Pathology confirmed no malignancy. Pituitary apoplexy was diagnosed. Her nausea and headache gradually improved. Six months after operation, her best-corrected visual acuity had improved to 20/30 and 20/20.ConclusionsAlthough pituitary apoplexy is rare in pediatric patients, prompt evaluation including detailed ophthalmic examination, biochemical evaluation, endocrine workup, and image study are very important.     

垂体卒中的临床与磁共振特征

目的:探讨垂体卒中的临床和MRI特征。方法:分析13例垂体卒中患者的临床和头MRI资料。结果:76.9%的患者卒中前无垂体瘤表现,84.6%的患者发病前无明显诱因。典型临床表现为头痛、呕吐及视力障碍等。MRI检查发现,瘤内出血8例,表现为T1WI等信号合并高信号,T2WI像为高信号;肿瘤内坏死3例,表现为T1WI低信号,T2WI高信号;出血合并坏死2例,表现为T1WI高低混杂信号,T2WI高信号。结论:垂体卒中临床表现多样,易漏诊误诊。MRI检查结合临床,对诊断和选择治疗方案有重要价值。     

Pituitary apoplexy in a child presenting with massive subarachnoid and intraventricular hemorrhage.

A case of pituitary apoplexy in a child with massive subarachnoid and intraventricular hemorrhage is reported. The patient presented with a sudden onset of headache, diminution of visual acuity and a bitemporal visual field defect. Computerized tomography revealed a massive subarachnoid hemorrhage with extension of blood into the third ventricle. Angiography did not show an aneurysm. The patient was operated on and altered blood within a necrotic pituitary adenoma was decompressed. His vision and field defect improved along with resolution of headache in the postoperative period.