Pituitary stalk hemangioblastoma: the fourth case report and review of the literature

Supratentorial hemangioblastomas (HBL) have been rarely described in the literature. Herein we report the fourth case of pituitary stalk HBL diagnosed concurrently with cerebellar HBLs in a 51-year-old woman with von Hippel-Lindau disease. Complete resection of the lesion was achieved using left frontopterional craniotomy and no recurrence was observed after 8 years of follow-up. The clinical presentation, radiological features, pathological findings, and the management of this particular case are discussed in the light of the available literature. To our knowledge, this case is the only pituitary stalk HBL with total resection, and a long follow-up fully documented.     

Melanoma of the sellar region

Metastatic melanoma has a propensity for multiple intra cranial deposits. Rarely, metastatic melanoma to the pituitary gland has been reported, usually in conjunction with widespread systemic metastases. We describe a patient with metastatic melanoma to the pituitary gland as the first clinical presentation of widespread metastatic disease and review the relevant literature.     

Small cell lung cancer metastasis in the pituitary gland presenting with seizures and headache

We report an adult female with a small cell lung cancer (SCLC) pituitary metastasis who presented with seizures and headache. While headache is a common presentation of pituitary metastases, seizures have been very rarely reported. The patient was found on MRI to have a suprasellar tumor with pituitary infundibulum involvement. She underwent endoscopic transsphenoidal resection of the tumor which was shown to be metastatic SCLC. Subsequent imaging revealed widespread systemic metastases. The patient subsequently underwent chemotherapy.     

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.     

Pituitary abscess

Pituitary abscess is a rare disease, but one with potentially high mortality and morbidity. We present a 46-year-old man with progressive visual disturbance and general malaise for 1 year. Endocrine studies revealed hypopituitarism, and magnetic resonance imaging revealed a pituitary lesion with suprasellar extension. We attempted to excise the lesion using a transsphenoidal approach, but pus in the pituitary fossa was found at operation, and no tumour was identified. The culture yielded coagulase-negative Staphylococcus. Antibiotics were administered for 3 weeks, and the patient made a good postoperative recovery. He required life-long hormone replacement therapy. After one and a half years of follow-up, he was well and had no evidence of focal or systemic infection. We review the literature regarding pituitary abscess and discuss the appropriate treatment and possible pathological mechanism.     

A case of pituitary metastasis from breast cancer that presented as left visual disturbance

Tumors that metastasize to the pituitary gland are unusual, and are typically seen in elderly patients with diffuse malignant disease. The most common metastases to the pituitary are from primary breast and lung cancers. We report a 65-year-old woman with pituitary metastasis from breast cancer who presented with recent-onset left progressive deterioration of visual acuity and visual field. The clinical diagnosis was made after brain and sellar magnetic resonance imaging showed a large sellar mass compressing the optic chiasm and invading the pituitary stalk. An otorhinolaryngology and neurosurgery team removed the tumor via a transsphenoidal approach, and this procedure obtained symptomatic relief. Postoperatively, metastasis from breast invasive ductal adenocarcinoma was confirmed histologically. We report this unusual case with a review of the relevant literature.     

Germinoma with synchronous lesions in the pineal and suprasellar regions

Background Germinomas represent the most frequent tumour type in the pineal region and the synchronous involvement of the suprasellar region, frequently termed bifocal disease, is increasingly recognised. The incidence, pathogenesis, histomorphological, radiological, clinical features, and management strategies are discussed. Case report A 6-year-old girl presented with clinical features of hypothalamic–pituitary axis dysfunction. MRI showed two distinct lesions in the pineal and suprasellar regions. Tumour markers for germ cell tumours from blood and cerebrospinal fluid were within the normal range. The suprasellar lesion was biopsied and the histology revealed a germinoma. She received a radical course of radiotherapy covering the entire craniospinal axis (24 Gy/15#) followed by focal boost to the sites of primary disease (16 Gy/10#). Three months after the completion of treatment, the patient achieved a complete clinical and radiological response, which was maintained during the 13-month follow-up period. Conclusions The optimum management of primary intracranial pineal and suprasellar (bifocal) germinomas still remains controversial. The definition as either localised or disseminated disease has major implications on required treatment and its associated late morbidity. Prospective studies with large cooperative groups worldwide and consensus meetings may reduce uncertainties regarding available management options.     

Preservation of hormonal function by identifying pituitary gland at endoscopic surgery

ObjectiveThe endonasal endoscopic approach has been established for perisellar tumor surgery with a higher resection rate and reduced complications. We analyzed the potential to identify the pituitary gland under endoscopic view, at surgery and see its relation to postoperative hormonal insufficiency in endonasal endoscopic procedures.MethodsBetween January 2011 and January 2014, 70 cases of pituitary adenomas with preoperative intact pituitary function underwent endoscopic endonasal transsphenoidal procedures for intrasellar pathologies. Endocrinologists and neurosurgeons followed these patients prospectively. Special attention was paid to intraoperative identification of gland tissue, surgical complications, degree of resection and postoperative hormonal insufficiency.ResultsThe pituitary gland was identified in 57 out of 70 procedures (81.4%). Eleven percent (8 of 70 patients) had persistent pituitary insufficiency. Two of these 8 patients belonged to the group with pituitary gland identification (2 out of 57); thus, when the pituitary gland was identified during the procedure postoperative hormonal insufficiency was seen in 3.5% of cases. Failure of pituitary gland identification presented with hormonal insufficiency of 46.2%. In analysis with Fisher’s exact test, there was a high significant correlation between the identification of the pituitary gland intraoperatively and normal pituitary function postoperatively (p < 0.005). On follow up radical tumor resection was seen in 88% (62 of 70 patients).ConclusionsThis study indicates that identification and preservation of pituitary gland tissue and function is possible in endoscopic transsphenoidal surgery. This preservation of gland tissue is a positive predictor of postoperative normal pituitary function.     

Cognitive function surrounding resection of nonfunctioning pituitary adenomas with suprasellar extension: A prospective matched-control study

ObjectivePatients suffering from pituitary adenomas may experience cognitive dysfunctions due to hormonal imbalance or suprasellar tumor extension displacing neural structures. Progressively enlarging or symptomatic nonfunctioning pituitary adenomas with suprasellar extension are frequently resected. The literature on neurocognitive performance surrounding resection of these lesions is sparse.MethodsA prospective matched-control study was conducted to investigate the impact of nonfunctioning pituitary adenomas with suprasellar extension on preoperative and postoperative cognitive performance. Controls were matched for age, sex, handedness, education, and profession. The neurocognitive test battery included perceptual speed, executive function, visual-spatial and verbal working memory, short- and long-term memory, verbal fluency, fluid intelligence, anxiety, and depression.ResultsTen patients and 10 healthy controls were matched. Median suprasellar tumor extension scored 8 mm, compression of frontal lobe parenchyma was present in all cases. Median sagittal tumor diameter was 21 mm. Preoperatively, patients scored worse in perceptual speed and short-term memory tasks. All patients underwent surgical resection either through a transnasal, transsphenoidal approach or a supraorbital frontolateral keyhole approach. The short-term memory deficit disappeared one week after surgery. Perceptual speed recovered within two months after surgical therapy. None of the patients experienced worsening of cognitive function. Routine postoperative imaging at six months did not reveal displacement of neural structures or surgery-related complications in any of the patients.ConclusionPatients suffering from nonfunctioning pituitary adenomas with suprasellar extension may experience preoperative impairments in some neurocognitive domains that resolve within two months after surgery. The risk for cognitive deterioration with surgery appears to be low.     

Utilizing a Novel Pituitary Retractor for Early Descent of the Diaphragma Sellae during Endoscopic Transsphenoidal Pituitary Surgery

ObjectiveEarly descent of the diaphragm sellae (DS) during endoscopic endonasal transsphenoidal surgery (EETS) for pituitary macroadenoma surgery is occasionally a troublesome event by blocking the surgical field. Here we introduce an alternative technique with the new pituitary retractor and present our clinical experiences. MethodsWe designed a simple and rigid pituitary retractor with the least space occupation in the nasal cavity to be compatible in EETS. The pituitary retractor was held by external holder system to support the herniated DS stably. We retrospectively reviewed a clinical 22 cases of pituitary macroadenomas underwent EETS using the pituitary retractor. ResultsThe pituitary retractor stably pushed up the herniated DS in all cases, and the surgeon proceeded the procedure with bimanual maneuver. The pituitary retractor was helpful to remove tumors around the medial cavernous sinus and behind the DS in 16 and seven cases, respectively. In four cases, the meticulous hemostasis was completed with the direct visualization by the DS elevation with this retractor. Gross total tumor resection was performed in 20/22 patients (91%). The impaired visual function and hypopituitarism were improved in 18/20 (90%) and 7/14 (50%) patients after surgery, respectively. There was no complication related with the pituitary retractor. ConclusionDuring EETS for pituitary macroadenomas, the novel pituitary retractor reported in this study is a very useful technique when the herniated DS block the surgical field and bimanual maneuver. This pituitary retractor can help to result in the excellent surgical outcomes with minimal morbidity.     

Metastatic hemangiopericytoma presenting as an epidural spinal cord lesion

OBJECTIVE: Hemangiopericytoma is an aggressive vascular tumour that rarely affects the central nervous system and is even more rarely spinal in presentation. The clinical features of a patient with a recurrent extraspinal hemangiopericytoma presenting with an epidural spinal cord compression by local invasion are described, including a review of the literature on metastatic hemangiopericytoma to the spine. METHODS: A case of a 53-year-old male, with a recurrent extraspinal hemangiopericytoma which metastasized to the thoracic spine five years after detection of the primary tumour is presented. A chart review was conducted where all pertinent history, physical, laboratory, and radiological data were collected. A Pub-Med search using the keyword "hemangiopericytoma" identified all reported cases documenting clinical features, treatment, recurrence and outcome with respect to metastatic hemangiopericytoma to the spine. RESULTS: Nine patients have been reported to have metastatic hemangiopericytoma to the spine. The median patient age was 47 years and there was a slight male preference. An unusual feature of the hemangiopericytoma is the prolonged period, up to 16 years, between the diagnosis of the primary hemangiopericytoma and the metastases to the spine. All patients were treated with a combination of radiation and surgery. CONCLUSIONS: Hemangiopericytomas show a slow clinical evolution with a strong propensity to relapse long after previous treatment and thus, once identified, prolonged follow-up for recurrence is indicated. A close follow-up of these patients is required because of frequent recurrences and delayed metastases even if the primary lesion was well-controlled. Although overall uncommon, hemangiopericytoma should be kept in mind in the differential diagnosis of vascular epidural spinal cord tumours.     

Differences in Clinical Characteristics and Surgical Outcomes of Patients with Ischemic and Hemorrhagic Pituitary Adenomas

ObjectiveIschemia and hemorrhage of pituitary adenomas (PA) caused important clinical syndrome. However, the differences on clinical characteristics and surgical outcomes between these two kinds apoplexy were less reported. MethodsA retrospective analysis was made of patients with pituitary apoplexy between January 2013 and June 2018. Baseline and clinical characteristics before surgery were reviewed. All patients underwent transsphenoidal surgery and were followed up at least 1 year. ResultsTotal 67 cases (5.8%) among 1147 pituitary tumor patients were enrolled, which consisted of 28 (~2.4%) ischemic PA and 39 (~3.4%) hemorrhagic PA. There were more male patients in the ischemic group compared with hemorrhagic group (78.6% vs 53.8%, p=0.043). However, the mean age, tumor size and functional tumor ratio were significant higher in the hemorrhagic group. Headache was more common in ischemic PA (82.1%) than that of hemorrhagic PA (51.3%, p=0.011). Magnetic resonance imaging findings found that mucosal thickening and enhancement of the sphenoid sinus was observed in 15 ischemic PA patients (n=27, 55.6%), but none in patients with hemorrhagic PA (n=38, p<0.0001). It was worth noting that the rate of pre-surgical hypopituitarism in ischemic PA patients were seemed higher than that in hemorrhagic PA patients, but not significant. The two groups got a total tumor resection rate at 94.1% and 92.9%, independently. No significant difference on the operative time, blood loss in operation and complications in perioperative period was observed in two groups. After operation, cranial nerve symptoms recovered to normal at 81.8% of ischemic PA patients and 82.6% of hemorrhagic PA patients. Importantly, the incidence of postoperative hypopituitarism partially decreased in both groups, among which the rate of hypothyroidism in ischemic PA patients significantly decreased from 46.4% to 18.5% (p=0.044). ConclusionPatients with ischemic PA presented different clinical characteristics to the hemorrhagic ones. Transsphenoidal surgery should be considered for the patients with neuro-ophthalmic deficits and might benefit for pituitary function recovery of the apoplectic adenoma patients, especially pituitary thyroid axis in ischemic PA patients.     

Multi-slice computed tomography-assisted endoscopic transsphenoidal surgery for pituitary macroadenoma: a comparison with conventional microscopic transsphenoidal surgery

Abstract

Objectives:

Intraoperative computed tomography (iCT) is a reliable method for the detection of residual tumour, but previous single-slice low-resolution computed tomography (CT) without coronal or sagittal reconstructions was not of adequate quality for clinical use. The present study evaluated the results of multi-slice iCT-assisted endoscopic transsphenoidal surgery for pituitary macroadenoma.

Methods:

This retrospective study included 30 consecutive patients with newly diagnosed or recurrent pituitary macroadenoma with supradiaphragmatic extension who underwent endoscopic transsphenoidal surgery using iCT (eTSS+iCT group), and control 30 consecutive patients who underwent conventional endoscope-assisted transsphenoidal surgery (cTSS group). The tumour volume was calculated by multiplying the tumour area by the slice thickness. Visual acuity and visual field were estimated by the visual impairment score (VIS).

Results:

The resection extent, (preoperative tumour volume – postoperative residual tumour volume)/preoperative tumour volume, was 98.9% (median) in the eTSS+iCT group and 91.7% in the cTSS group, and had significant difference between the groups (P?=?0.04). Greater than 95 and >90% removal rates were significantly higher in the eTSS+iCT group than in the cTSS group (P?=?0.02 and P?=?0.001, respectively). However, improvement in VIS showed no significant difference between the groups. The rate of complications also showed no significant difference.

Discussion:

Multi-slice iCT-assisted endoscopic transsphenoidal surgery may improve the resection extent of pituitary macroadenoma. Multi-slice iCT may have advantages over intraoperative magnetic resonance imaging in less expensive, short acquisition time, and that special protection against magnetic fields is not needed.     

Sella turcica metastasis from follicular carcinoma of thyroid

Abstract

A case of metastasis to the sella turcica from a follicular adenocarcinoma of the thyroid gland is presented. Metastasis to this site is rare and review of the literature reveals only 12 cases of metastatic thyroid carcinoma involving the sella turcica and pituitary gland. The optimal treatment strategy is still to be determined. A 43-year-old woman presented with headache, nausea, visual impairment and galactorrhea. An MRI scan of the cranium revealed an enhancing destructive sellar lesion. The patient underwent transsphenoidal removal of the lesion to alleviate visual loss. The histological features of the sellar tumor were identical to those of a follicular adenocarcinoma partially removed from the thyroid gland 22 months earlier. Total thyroidectomy followed by three courses of iodine-131 ablation enhanced with synthetic thyrotropin and thyroid hormone suppression therapy was instituted. The post-operative course was satisfactory with improved vision and ceased galactorrhea. This case was successfully treated with a combination of surgical removal, iodine-131 ablation and hormone suppression therapy, which resulted in disease control duration of four years. Sella turcica metastases of thyroid carcinoma are exceedingly rare and currently there are no established therapeutic guidelines.     

Bony metastases of anaplastic oligodendroglioma respond to temozolomide

BACKGROUND: Fewer than 30 cases of oligodendroglioma or anaplastic oligodendroglioma metastatic to bone are reported in the literature. Prolonged survival even with therapy is uncommon. METHODS: We report a case of anaplastic oligodendroglioma metastatic to bone with a dramatic and durable response to temozolomide therapy. A retrospective case review, molecular analysis, and literature search were performed. RESULTS: The patient presented with a right frontal mass in 1990. Progression led to resection of the lesion in 1995. Histology revealed an anaplastic oligodendroglioma and the tumour was found to have allelic loss of heterozygosity (LOH) of chromosome 1p (1p-). He received standard radiation therapy. In 2000 he developed hip and pelvic pain. A bone scan showed multiple skeletal lesions. Magnetic resonance imaging of the brain showed stability of intracranial disease. Resection of one lesion found metastatic anaplastic oligodendroglioma with identical morphology to the patient's original tumour, including glial fibrillary acidic protein expression. The patient was started on standard temozolomide chemotherapy and celecoxib with prompt pain relief, and rapid normalization of serum alkaline phosphatase. He received a total of 12 cycles of combined therapy with no toxicity and no evidence of progression until increasing pain marked disease recurrence. The patient underwent palliative chemo- and radiation therapy but eventually succumbed. DISCUSSION: Loss of heterozygosity 1p- is associated with prolonged survival in anaplastic oligodendroglioma and may increase the cumulative risk for development of systemic metastases. We speculate that metastases from oligodendroglioma harbouring loss of heterozygosity at chromosome 1p- retain the chemosensitivity of the initial lesion.     

Spontaneous subarachnoid haemorrhage from rupture of an anterior communicating artery aneurysm in a patient with pituitary macroadenoma

The presence of a cerebral aneurysm in patients with pituitary adenoma is a rare event. Diagnostic suspicion may stem from magnetic resonance imaging, which should lead to complementary investigation. As for treatment, even in conditions in which there has been no previous bleeding, the simultaneous approach should be considered, prioritising the aneurysm most of the time.The present report describes the case of a patient with a history of pituitary macroadenoma, who had undergone a partial transsphenoidal resection ten years earlier. Admission to our service occurred after a sudden headache followed by mental confusion. A cranial computed tomography showed subarachnoid haemorrhage and expansive suprasellar lesion. Cerebral angiography showed a saccular aneurysm of the anterior communicating complex. The patient underwent a surgical procedure for microsurgical clipping of the aneurysm and partial resection of the pituitary tumour. We have also included a brief review of the literature on this subject.     

经鼻蝶入路显微镜下切除垂体瘤118例临床分析

目的探讨经鼻蝶显微镜下入路切除垂体瘤的临床效果。方法回顾性分析118例行经鼻蝶显微镜下垂体瘤切除手术患者术后及各类并发症的处理。结果肿瘤全切93例,次全切20例,部分切除5例,术后临床效果满意。结论经蝶窦入路显微切除垂体瘤手术,肿瘤全切除率高,症状改善明显,并发症较少。     

动态MRI检查在Cushing病的临床应用价值

目的 为分析动态：MRI检查对普通MRI检查未发现垂体瘤的Cushing病(库欣病)的诊断价值。方法 回顾性分析7例普通：MRI检查未发现垂体微腺瘤的Cushing病人进行动态：MRI检查的结果，以及比较术中所见。7例均为女性，平均年龄33．5岁，平均病程2．7年，有典型的Cushing综合征的表现。结果动态MRI检查7例中，4例显示垂体微腺瘤，并经手术证实；3例未显示垂体腺瘤，但手术证实2例为垂体微腺瘤，1例为垂体细胞增生。随诊1—6个月，皮质醇降至正常6例。结论 对于临床症状和内分泌学检查符合Cushing病，而普通MRI检查未发现垂体腺瘤的病例，采用动态MRI检查能提高垂体ACTH微腺瘤的发现率。     

Giant pituitary macroadenoma at the age of 4 months: case report and review of the literature

Case report Adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma is extremely rare. Only five cases are reported in the literature to date. We report the youngest patient of Cushings disease due to ACTH-secreting pituitary macroadenoma at the age of 4 months. The patient presented with cushingoid facial features, hypertension and optic atrophy. Serum cortisol level was 97 g/dl, with increased secretion of urinary free cortisol; plasma ACTH level was 353 pg/ml. Magnetic resonance imaging of the brain showed a large contrast-enhancing solid mass sitting in the sellar region, with suprasellar and lateral extension. Surgical resection was done, and immunohistochemistry confirmed the diagnosis of ACTH-secreting pituitary adenoma. Postoperatively, the patient died of disseminated intravascular coagulopathy.Conclusion Cushings disease due to ACTH-secreting pituitary macroadenoma is a possible diagnosis in early infancy. This report, along with five previously reported cases in the literature, revealed a poor surgical outcome. This surgical morbidity is possibly attributed to difficult peroperative hemostasis due to vasculopathy in Cushings disease, which leads to excessive blood loss, adverse hemodynamic changes, myocardial dysfunction and disseminated intravascular coagulopathy.     

Predicting Arachnoid Membrane Descent in the Chiasmatic Cistern in the Treatment of Pituitary Macroadenoma

ObjectivePreoperative prediction of the arachnoid membrane descent in pituitary surgery is useful for achieving gross total removal and avoiding cerebrospinal fluid leakage resulting from tearing of the arachnoid membrane in the chiasmatic cistern. In this study, we analyzed the patterns of arachnoid membrane descent during or after pituitary tumor surgery and identified the factors related to this descent. MethodsAnalysis was restricted to pituitary macroadenomas not extending into the third ventricle or over the internal carotid artery. To minimize confounding factors, patients who underwent revision surgery, those who had a torn arachnoid during operation or small medial diaphragma sellae (DS) opening, and subtotal resections were excluded. We enrolled 41 consecutive patients in this retrospective analysis. The degree of arachnoid descent was categorized using intraoperative videos. Preoperative magnetic resonance findings, including tumor height, suprasellar extension, and variables including DS area and medial opening size, tumor composition, and displacement of the pituitary stalk and gland were evaluated to determine their correlations with arachnoid membrane descent. ResultsArachnoid membrane descent was significantly correlated with DS area and medial opening size. Based on T2-weighted images (T2WI) magnetic resonance (MR) images, tumor composition was significantly associated with arachnoid membrane descent. Other factors were not significantly correlated with arachnoid membrane descent. ConclusionT2WI of tumor composition and preoperative MR imaging of DS area and medial opening provided valuable information regarding arachnoid membrane descent. These parameters may serve as fundamental measures to facilitate complete resection of pituitary macroadenomas.