Suprasellar Extension Research Articles

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 8mm, compression of frontal lobe parenchyma was present in all cases. Median sagittal tumor diameter was 21mm. 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.

Predictors of visual outcome with transsphenoidal excision of pituitary adenomas having suprasellar extension: A prospective series of 100 cases and brief review of the literature.

Objective:The aim was to assess the factors influencing the visual outcome following trans-sphenoidal excision of pituitary adenomas.Materials and Methods:One hundred consecutive patients of pituitary adenomas with suprasellar extension (SSE) were operated by trans-sphenoidal approach from July 2003 to December 2006. There were 52 male and 48 female patients with a mean age of 42.47 years. The visual impairment score, which was used to evaluate the visual outcome was produced by adding the scores for visual acuity and visual field defects of each patient (from the tables of the German Ophthalmological Society). The mean diameter, the SSE and the parasellar extension of the lesion were noted in the magnetic resonance imaging study. The average follow-up was 43.5 months.Results:The mean diameter of the tumor was 32.97 mm, and the mean SSE was 14.95 mm. The parasellar extension was present in 27 patients. The vision improved in 61 of the 71 patients (85.91%). The shorter the duration of visual symptoms and smaller the size of the lesion resulted in better visual outcome. The age and the preoperative visual impairment did not show any correlation with the visual outcome.Conclusions:Patients with visual symptoms of <1-year duration (P < 0.01) and adenomas of <36.5 mm diameter (P < 0.009) have better visual outcome.

Cerebral vasospasm after transsphenoidal surgery for pituitary adenoma: Case report and review of the literature

IntroductionCerebral vasospasm (CVS) is a rare complication of transsphenoidal surgery for pituitary adenomas. Case reportThe authors describe a symptomatic cerebral ischemia due to an internal carotid, left middle cerebral artery and anterior cerebral artery vasospasm in a patient undergoing transsphenoidal surgery for a pituitary macro adenoma. The patient was successfully treated by an endovascular balloon angioplasty. ConclusionThis issue should be taken into account, even when rare, in every patient who has surgery for a pituitary tumor, particularly when it concerns a large macro adenoma with suprasellar extension. Special precautions must be taken to avoid this rare complication.

Craniopharyngioma presenting with severe hyponatremia, hyponatremia-induced myopathy, and panhypopituitarism: a case report

BackgroundCraniopharyngiomas are rare intracranial tumors commonly presenting with neurological symptoms. Reports of severe hyponatremia as a presenting manifestation of a craniopharyngioma and hyponatremia-induced myopathy are rare. We report the case of a patient with craniopharyngioma presenting with severe hyponatremia, panhypopituitarism, and hyponatremia-induced myopathy.Case presentationA 52-year-old Sri Lankan man presented with anorexia, nausea, fatigue, generalized muscle weakness, and cramps for 1 week. The onset of his illness had been preceded by vomiting and diarrhea for 1 day which he attributed to food poisoning. On examination, he had an apathetic disposition with a generalized “sallow complexion.” He was not dehydrated. Apart from reduced muscle power (4/5) and hyporeflexia, the neurological examination was normal. His serum sodium was 102 mmol/l; potassium 4.1 mmol/l; chloride 63 mmol/l; plasma osmolality 272 mosm/KgH2O; urine osmolality 642 mosm/KgH2O; and urine sodium 79 mmol/l. His creatine phosphokinase was 12,400 U/l, lactate dehydrogenase 628 U/l, aspartate aminotransferase 360 U/l, and alanine aminotransferase 64 U/l. His hormone profile revealed panhypopituitarism. An electromyogram showed nonspecific abnormalities while a muscle biopsy did not show any pathology. Magnetic resonance imaging of his brain demonstrated a well-defined craniopharyngioma with suprasellar extension. His pituitary gland was compressed and the pituitary stalk was displaced by the tumor. He had marked improvement in muscle power and rapid reduction of serum creatine phosphokinase levels paralleling the correction of severe hyponatremia, even before the initiation of hormone replacement.ConclusionsThis case illustrates the rare presentation of severe hyponatremia and hyponatremia-induced myopathy in patients with craniopharyngioma, awareness of which would facilitate early appropriate investigations and treatment.

Medulloblastoma with Suprasellar Extension and Atypical Presentation

: A 6-year-old boy was referred with symptoms of right partial ptosis, blurred vision and a two-week history of poor coordination and frequent falls. No symptoms of raised intracranial pressure were demonstrated. Imaging showed a large sella mass with extension into the posterior fossa and suprasellar region, suggestive of a craniopharyngioma. Histopathological examination of the post-surgical resection specimen revealed a small blue cell tumor consistent with a medulloblastoma. This case aims to highlight a posterior fossa tumor with an unusual spread and thus clinical presentation.

Recent Evolution of Endoscopic Endonasal Surgery for Treatment of Pituitary Adenomas.

For the treatment of pituitary tumors, microscopic transsphenoidal surgery has been considered the “gold standard” since the late 1960s. Over the last two decades, however, a worldwide shift towards endoscopic endonasal surgery is in progress for many reasons. These include a wide panoramic view, improved illumination, an ability to look around anatomical corners using angled tip and, in addition, application to the extended approaches for parasellar tumors. Both endoscopic and microscopic approaches appear equally effective for nonfunctioning adenomas without significant suprasellar or lateral extensions, whereas the endoscopic approach may improve outcomes associated with the extent of resection and postoperative complications for larger tumors. Despite many theoretical benefits in the endoscopic surgery, remission rates of functioning adenomas do not substantially differ between the approaches in experienced hands. The endoscopic approach is a valid alternative to the microscopic approach for adenomas. The benefits will be more appreciated in the extended surgery for parasellar tumors.

Magnetic Resonance-Guided Laser Ablation: A Viable Treatment Alternative for Recurrent Meningioma?

In this study, we evaluated the changes in resting-state networks (RSNs) under anesthesia in neurosurgical patients.RSNs were analyzed in 12 patients with pituitary adenoma presented by chiasma compression operated via standard transsphenoidal approach under propofol anesthesia before and after tumor resection. All the patients had suprasellar tumor extension with compression of the optic chiasma. We investigated second-level effects by contrasting dummy-encoded covariates representing the effects of the sessions (first vs. second) on RSNs. We corrected for multiple comparisons using a false discovery rate of 0.05 (2-sided).Connectivity between the right and left precentral gyri (motor network) decreased significantly from the first to the second session (P = 0.0002), as did the connectivity between the postcentral gyri (P = 0.009). The same was valid for connectivity between the visual cortices (P = 0.0002). The salience network showed a significant decrease in the connectivity of the anterior part of the cingulate gyrus and insular cortex (P = 0.0001). The default mode network showed a decrease in the connectivity between the posterior part of the cingulate gyrus, parietal, and frontal cortices (P = 0.0002). There was no significant correlation between the reduction in connectivity and dose or duration of anesthesia.Different RSNs could be identified under anesthesia and used for intraoperative brain mapping and remapping during tumor resection. However, RSNs showed a significant decrease in connectivity with the continuation of anesthesia.

Visual pathway impairment by pituitary adenomas: quantitative diagnostics by diffusion tensor imaging.

OBJECTIVE Despite ample experience in surgical treatment of pituitary adenomas, little is known about objective indices that may reveal risk of visual impairment caused by tumor growth that leads to compression of the anterior visual pathways. This study aimed to explore diffusion tensor imaging (DTI) as a means for objective assessment of injury to the anterior visual pathways caused by pituitary adenomas. METHODS Twenty-three patients with pituitary adenomas, scheduled for transsphenoidal tumor resection, and 20 healthy control subjects were included in the study. A minimum suprasellar tumor extension of Grade 2-4, according to the SIPAP (suprasellar, infrasellar, parasellar, anterior, and posterior) scale, was required for inclusion. Neuroophthalmological examinations, conventional MRI, and DTI were completed in all subjects and were repeated 6 months after surgery. Quantitative assessment of chiasmal lift, visual field defect (VFD), and DTI parameters from the optic tracts was performed. Linear correlations, group comparisons, and prediction models were done in controls and patients. RESULTS Both the degree of VFD and chiasmal lift were significantly correlated with the radial diffusivity (r = 0.55, p < 0.05 and r = 0.48, p < 0.05, respectively) and the fractional anisotropy (r = -0.58, p < 0.05 and r = -0.47, p < 0.05, respectively) but not with the axial diffusivity. The axial diffusivity differed significantly between controls and patients with VFD, both before and after surgery (p < 0.05); however, no difference was found between patients with and without VFD. Based on the axial diffusivity and fractional anisotropy, a prediction model classified all patients with VFD correctly (sensitivity 1.0), 9 of 12 patients without VFD correctly (sensitivity 0.75), and 17 of 20 controls as controls (specificity 0.85). CONCLUSIONS DTI could detect pathology and degree of injury in the anterior visual pathways that were compressed by pituitary adenomas. The correlation between radial diffusivity and visual impairment may reflect a gradual demyelination in the visual pathways caused by an increased tumor effect. The low level of axial diffusivity found in the patient group may represent early atrophy in the visual pathways, detectable on DTI but not by conventional methods. DTI may provide objective data, detect early signs of injury, and be an additional diagnostic tool for determining indication for surgery in cases of pituitary adenomas.

Lymphocytic hypophysitis: modern day management with limited role for surgery.

We set out to describe the modern day management of Lymphocytic hypophysitis (LH) with respect to its diagnosis, treatment and long-term endocrine and visual outcomes. We deliberately included patients with a clinical diagnosis of LH and compared them with those with a histological diagnosis following surgery. A multi-centre observational study was performed. Twenty-two patients were included and studied over a mean follow-up period of 8.6years (range 4-26years). Patients presented with headache (N=15; 68%), visual field disturbances (N=7; 32%) or a combination of these symptoms (N=5; 23%). The time lag between onset of symptoms and diagnosis was <1month, 1-6months or >6months in approximately a third of the patients each, respectively. In two-thirds of the patients (N=14) LH was diagnosed based on their clinical, biochemical and radiological findings. The surgical cohort (N=8) was statistically more likely to have presented with larger lesions, with suprasellar extension and greater visual field defects. During follow-up there was improvement in headaches and visual symptoms, but the rate of hypopituitarism as reflected by the need for on-going pituitary hormone replacement persisted. At the 1, 5 and 10year follow-up there was no significant difference between the medically and surgically managed cohorts in terms of ongoing symptomatology or need for pituitary hormone replacement. In the modern era, most patients with LH are diagnosed by non-surgical means and managed medically. There remains a significant time lag between the onset of symptoms and the eventual diagnosis of LH. Despite the considerable morbidity attached to LH, overall prognosis is good.

Transsphenoidal microsurgical treatment of infradiaphragmatic craniopharyngioma ⁎

Abstract Objective Typically, the transcranial approach has been used for the treatment of craniopharyngiomas with suprasellar extension, whereas the transsphenoidal approach has been used mostly for infradiaphragmatic craniopharyngioma. Total resection of craniopharyngioma can reduce the recurrence rate, especially in young children, but it may lead to severe complications. Therefore, any benefit of the degree of resection must be weighed against the risk of complications by the surgeons. The purpose of this study was to explore the therapeutic outcome after transsphenoidal microsurgical treatment of infradiaphragmatic craniopharyngioma and share our experiences. Methods Between January 2003 and June 2013, 30 patients with infradiaphragmatic craniopharyngioma underwent transsphenoidal microsurgical resection in our hospital. The neurological, visual, and endocrine functions, and extent of resection were analyzed retrospectively. Recurrence or growth of residual tumor tissue during follow-up was assessed using magnetic resonance imaging (MRI). Results Total resection was achieved in 25 patients (83.3%), subtotal resection was achieved in 4 patients (13.3%), and partial resection was achieved in 1 patient (3.4%). There were no perioperative deaths. Cerebrospinal fluid (CSF) leakage occurred in 6 patients, and among them, 2 required surgical repair of the sella. New-onset postoperative diabetes insipidus (DI) developed in 8 patients. Vision and visual fields were improved at different levels in 13 out of 16 patients who had sight impediments before treatment. Tumor recurrence and regrowth was observed in 2 patients; 1 patient underwent transsphenoidal reoperation, the condition of the other patient who had undergone several craniotomies grew worse over the 6-month follow-up period. Conclusion Transsphenoidal surgery is an ideal choice in treating infradiaphragmatic craniopharyngioma. The transsphenoidal approach, which preserves pituitary function and avoids damage to the hypothalamic structures and optic nerve, is associated with fewer complications than the transcranial approach and a low mortality rate.

Analysis of risk factors for visual acuity and visual field impairments in patients with pituitary adenoma

Objective To investigate the risk factors for visual acuity and visual field impairments, and the effect on their prognoses. Methods The clinical data of 637 patients with pituitary adenoma operated at the Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University from January 2008 to December 2013 were analyzed retrospectively. The patients were followed up at 3 years after procedure. Receiver operating characteristic (ROC) curve was used to obtain the optimal critical value of the risk factors for affecting visual acuity and visual field impairments and postoperative recovery. The round numbers were taken to group. Results In 637 patients, 436 (68.4%) experienced visual acuity and visual field impairments, 414 of them had decreased vision, and 292 had visual field defect. Receiver operating characteristic (ROC) curve showed that (1) the suprasellar extension height of tumors 10 mm group (all P 18.5 mm, the risk of causing permanent visual acuity and visual field impairments increased. There were significant differences in postoperative visual acuity improvement, visual acuity and visual field impairment score (VIS) grades between the suprasellar extension height of tumors ≤20 mm group and the and >20 mm group (P 0.05); (3) the patients with the duration of preoperative visual acuity and visual field impairments >13.5 months, the postoperative visual acuity and visual field impairments were more difficult to improve. There was significant difference between the recovery of visual acuity in patients with the duration of the disease >12 months after operation and the VIS grade poor than ≤12 months. Conclusions The greater the height of pituitary adenoma extending to the suprasaddle, the higher the probability of visual acuity and visual field impairments. The improvement of visual acuity and visual field after procedure is associated with the height of tumors extending to the suprasaddle and the course of visual acuity and visual field impairments. Key words: Pituitary neoplasms; Vision disorders; Risk factors

Surgical management of acromegaly: Long term functional outcome analysis and assessment of recurrent/residual disease.

Context:Functional growth hormone producing adenomas have long-term deleterious effects on the visual apparatus, the cardiovascular and musculoskeletal systems, and often predispose to malignancies. Since persistence of acromegaly affects outcome and quality of life, therapeutic interventions become mandatory.Aim:This study represents an analysis of long-term clinical and endocrinal outcome of 115 patients of acromegaly after surgical management.Setting and Design:Tertiary care retrospective study.Materials and Methods:One hundred and fifteen patients (male:female ratio: 1:1.09) with acromegalic features were studied. Apart from acromegalic features, their main clinical presentation also included headache, diminution of vision, field defects, ptosis, irregular menstruation, diabetes insipidus, diabetes mellitus and hypertension. Six of them presented with apoplexy. Their preoperative endocrinal evaluation included basal and suppressed growth hormone (GH), prolactin and thyroid levels. On the basis of axial and coronal CT scan or multiplanar MR imaging or both, the tumors were classified according to their suprasellar and parasellar extension (Hardy's grade). Transnasal trans-sphenoidal surgery (TSS) (n = 37) and sublabial, rhinoseptal TSS (n = 72) were the preferred approaches. Six patients with significant parasellar extensions underwent trans-cranial explorations. The patients were followed up at 6 and 12 weeks and then at 6 monthly intervals. Hormonal and CT/MR evaluation were also done. Attainment of random GH value less than 2.5 µg/L, and the nadir GH value after oral glucose tolerance test (OGTT) less than 1 µg/L were used as the criteria of cure.Findings:The patients were preoperatively in Hardy's tumor grade 0 (29), A (21), A+E (3), B (21), B+E (5), C (9), C+E (10), D (1) D+E (11), E (5), respectively. One hundred and one patients were available for follow-up (FU; median FU duration: 84 months; range: 6 to 132 months). Surgical cure was achieved in 73 patients following the first surgery; and, in 10 additional patients following a second intervention. No patient with a preoperative grade 0, A, B, C had a recurrence after attaining the initial remission. Recurrence after an initial cure occurred in 7 patients (overall remission rate following surgery: 75.24%). The preoperative grade of the latter patients was A+E:1, B+E:1, C+E:1, D:1, D+E:2, E:1, respectively. All these patients underwent subsequent radiotherapy (RT). The twelve patients with persistent symptoms and high GH levels following surgery underwent RT; six others with improved symptoms despite high GH levels were kept under strict observation. There was no surgical mortality.Conclusions:A high remission rate without significant morbidity could be achieved following surgical intervention in acromegalic patients. Following surgery, tumors with greater than or equal to 3 cm suprasellar height and without parasellar extension had no clinical recurrence at FU. A continuous monitoring is mandatory to pick up relapsed cases as well as those who develop delayed signs of hypopituitarism. A subset of patients who show clinical improvement following surgery but still have higher GH levels may be followed up without additional therapy unless clinical signs reappear or the serum GH levels progressively increase.

Endoscopic endonasal transsphenoidal surgery: predictors for disease control in a consecutive series of pituitary adenomas

Abstract Endoscopic endonasal transsphenoidal surgery for pituitary lesions has been predestined and evolved since its incipient description. However, tumour size and extrasellar extensions of pituitary adenomas remain a challenge for disease control (DC) after surgery. This study was conducted to evaluate the predictors that determine the early outcome in a consecutive series of pituitary adenomas operated using an endoscopic transsphenoidal approach. Sixty-five consecutive pituitary adenomas presenting over a 36-month period subjected to excision through an entirely endoscopic transsphenoidal approach were reviewed. DC, based on the extent of tumour resection and endocrinological remission, was evaluated according to the recent radiological and hormonal consensus criteria. Of 65 pituitary adenomas operated, 24 were endocrinally nonfunctioning and 41 were functioning adenomas. The follow-up duration ranged from 3 to 33 months. The overall DC was 67.7% as measured using total tumour resection and endocrinal cure. Cavernous sinus invasion, suprasellar extension and revision surgery negatively influenced DC of pituitary adenomas. Postoperative complications related to surgical resection of adenomas were seen in 12 cases (18.5%) − mainly, cerebrospinal fluid leakage, anterior pituitary insufficiency, and diabetes insipidus. This study reports standards for DC in a short follow-up series of purely endoscopic pituitary surgeries and identifies pituitary lesions associated with preoperative predictors that can influence postoperative outcome. These results authenticate the efficacy and safety of endoscopic endonasal transsphenoidal surgery in the treatment of pituitary adenomas, providing favourable DC for both functioning and nonfunctioning pituitary adenomas.

Combined surgical and medical treatment of giant prolactinoma: case report

Abstract The operative management of giant pituitary prolactinoma represents a significant challenge for neurosurgeons, due to the degree of local tumor infiltration into adjacent structures such as cavernous sinus. The degree of parasellar tumor extension can be classified according to the Knosp grading system’ while suprasellar extension is qualified in accordance with the modified Hardys classification system. This report describes the case of a male patient with a giant pituitary prolactinoma in which a partial tumor resection via a subfrontal approach was achieved. Typically, resection rates of less than 50% have been reported following surgery on giant pituitary adenomas. Prolactin levels were very high, consistent with invasive giant prolactinoma. Our patient was treated with Cabergoline which eventually normalized the prolactin level and significantly reduced the size of the residual tumor. This case serves to illustrate that in the presence of significant suprasellar and parasellar extension, multi-modal treatment strategies with surgery and dopamine agonist, is the gold standard in the management of locally aggressive pituitary prolactinomas.

Postoperative otorhinolaryngologic complications in transnasal endoscopic surgery to access the skull base

IntroductionThe large increase in the number of transnasal endoscopic skull base surgeries is a consequence of greater knowledge of the anatomic region, the development of specific materials and instruments, and especially the use of the nasoseptal flap as a barrier between the sinus tract (contaminated cavity) and the subarachnoid space (sterile area), reducing the high risk of contamination. ObjectiveTo assess the otorhinolaryngologic complications in patients undergoing endoscopic surgery of the skull base, in which a nasoseptal flap was used. MethodsThis was a retrospective study that included patients who underwent endoscopic skull base surgery with creation of a nasoseptal flap, assessing for the presence of the following post-surgical complications: cerebrospinal fluid leak, meningitis, mucocele formation, nasal synechia, septal perforation (prior to posterior septectomy), internal nasal valve failure, epistaxis, and olfactory alterations. ResultsThe study assessed 41 patients undergoing surgery. Of these, 35 had pituitary adenomas (macro- or micro-adenomas; sellar and suprasellar extension), three had meningiomas (two tuberculum sellae and one olfactory groove), two had craniopharyngiomas, and one had an intracranial abscess. The complications were cerebrospinal fluid leak (three patients; 7.3%), meningitis (three patients; 7.3%), nasal fossa synechia (eight patients; 19.5%), internal nasal valve failure (six patients; 14.6%), and complaints of worsening of the sense of smell (16 patients; 39%). The olfactory test showed anosmia or hyposmia in ten patients (24.3%). No patient had mucocele, epistaxis, or septal perforation. ConclusionThe use of the nasoseptal flap has revolutionized endoscopic skull base surgery, making the procedures more effective and with lower morbidity compared to the traditional route. However, although mainly transient nasal morbidities were observed, in some cases, permanent hyposmia and anosmia resulted. An improvement in this technique is therefore necessary to provide a better quality of life for the patient, reducing potential complications.

The Supraorbital Keyhole Approach to the Suprasellar and Supra-Intrasellar Rathke Cleft Cysts Under Pure Endoscopic Visualization

To evaluate the feasibility of the supraorbital keyhole approach for suprasellar or intrasuprasellar Rathke cleft cysts (RCCs) under pure endoscopic visualization. Thirteen surgically treated patients with symptomatic RCCs were analyzed retrospectively. Preoperative manifestations included headache (77%), visual impairment (38%), polyuria (31%), and pituitary dysfunction (15%). The maximal cyst diameter averaged 29.3 mm (23-46 mm). Eight patients (62%) had a purely suprasellar lesion, and 5 (38%) had a sellar lesion with suprasellar extension. All patients underwent surgery via supraorbital keyhole approach under pure endoscopic view. The mean duration of follow-up was 21.4 months (9-45 months). The cyst content was removed completely in all cases, and the cyst wall was resected partially without transgressing surrounding structures. Preoperative headaches, visual dysfunction, diabetes insipidus, and hypopituitarism resolved in 80%, 60%, 50%, and 50% of patients, respectively. Postoperative complications included transient polyuria in 2 cases and temporary subcutaneous fluid collection in one. No radiological recurrence was observed during the follow-up. The supraorbital approach under pure endoscopic visualization provides a valuable optional minimally invasive access for suprasellar and intrasuprasellar RCCs. This approach combines the advantage of minimal invasiveness and improved visualization while avoiding the risk of cerebrospinal fluid leaks, rhinonasal complications, and gland injury. It is particularly suitable for those cysts with the normal pituitary gland occupying the whole or most part of sellar floor.

Epidemiology and radiological geometric assessment of pituitary macroadenomas: population-based study.

Pituitary adenomas are relatively common tumours with diverse clinical features. Epidemiological data are important to help quantify health burden. To provide in-depth epidemiological data on macroadenomas and radiologically characterize macroadenomas. Population-based retrospective analysis, Prevalence as at 2014; Incidence based on data from 2000 to 2014, Retrospective analysis of baseline MRI. The Maltese islands. 173/136 patients with macroadenomas for prevalence/incidence estimates respectively, 122 baseline MRI for radiological characterization. Prevalence rates, Standardized Incidence rates (SIR), MRI findings. The prevalence for macroadenomas was 40·67/100 000 people and the SIR was 1·90/100 000/year. Giant pituitary adenomas (>40 mm) constituted 4·8% of the whole cohort of PAs and the SIR was 0·18/100 000/year. Giant prolactinomas constituted 4·7% of all the prolactinomas and the SIR was 0·07/100 000/year, while giant NFPA constituted 6·0% of all NFPA and the SIR was 0·12/100 000/year. There was a statistically significant difference in the degree of suprasellar extension (P < 0·001) and infrasellar extension (P = 0·028) between the different macroadenoma subtypes and in the vertical extension indices (median vertical extension index NFPA 3·0 mm; PRLoma -7·7 mm; GH-secreting PA -1·7 mm; P < 0·001). Pituitary macroadenomas with cavernous sinus invasion were statistically significantly larger than those without cavernous sinus invasion (P < 0·001). NFPA had predominantly a superior extension into the cavernous sinus (63·6%) compared to the functional PAs which had predominantly an inferior extension into the cavernous sinus (59·1%) (P = 0·032). The various macroadenoma subtypes' epidemiological data are presented and differences between growth patterns among the various subtypes are highlighted.

A Case of Recurrent Acromegaly: Medical Management of the Disease

Introduction: Acromegaly is a rare, chronic, insidious disease characterized by overproduction of growth hormone (GH) and its tissue mediator insulin-like growth factor 1 (IGF-1) typically from a pituitary adenoma. Recurrent acromegaly is established by GH hypersecretion after initial biochemical cure, and is even rarer than the disease itself, reported in only 2 to 3% of the cases. Case Report: We present a case of a 44 year-old Hispanic male with a past medical history of acromegaly diagnosed 20 years ago initially treated with transsphenoidal microsurgery and open brain surgery two years after the first procedure. He was lost to follow up and presented to our outpatient clinic 18 years later complaining of frequent headaches with nose and hands enlargement. Physical examination revealed an overweight male patient with a height of 75 inches, enlarged nose, lips, splayed teeth, coarse facial features, macroglossia, and enlargement of both hands with hyperhidrosis of his palms. Laboratory work up confirmed the diagnosis of recurrent acromegaly with elevated fasting IGF-1 level. MRI of the brain showed a 4.3 x 3.8cm pituitary macroadenoma with extensive suprasellar extension and optic chiasm compression. Medical treatment with Octreotide Long-Acting Release (LAR) was started immediately and subsequent repeat IGF-1 levels have shown progressive improvement. Discussion: This patient’s case of recurrent acromegaly shows the importance of follow up after any surgical or medical intervention in acromegaly patients. Recurrence, though infrequent, is possible and these patients have been identified as a subset of patients that can be difficult to manage. Therefore close monitoring with IGF-1 and GH levels is crucial to diagnose and treat recurrence of the disease and its comorbidities. Our goal was to achieve a biochemical cure, however given the fact that the patient refused any surgical intervention, makes the case even harder to manage. Somatostatin analogues have proven to be effective in cases like ours, yet its efficacy varies among studies.

Pituitary hyperplasia mimicking macroadenoma secondary to primary hypothyroidism

Objective - We aim to emphasize the importance of extensive endocrine workup in cases of pituitary masses. Case report - We report on a case of pituitary thyrotrophic hyperplasia in a 12-year-old girl who was thought to have a pituitary macroadenoma with suprasellar extension. The main complaint was headache, while other symptoms of hypothyroidism were present, but weren't recognised. Hormonal testing revealed low total thyroxine (<12.8 nmol/l) and high TSH (310.5 mIU/l) levels, and hyperprolactinemia (prolactin level at 1680 mIU/l). Based on the clinical history, laboratory data, and MRI, a diagnosis of pituitary hyperplasia secondary to primary hypothyroidism, consequent to chronic autoimmune thyroiditis, was made. Therapy with levothyroxine was initiated at 50 I¼g/day and gradually increased to 75 I¼g daily. After three months of thyroxine replacement, she was clinically and biochemically euthyroid. A follow-up MRI, 4 months after thyroxine replacement was initiated, showed complete resolution of the mass, and normal pituitary gland. Conclusion - Primary hypothyroidism should be considered in the differential diagnosis of pituitary masses. Multidisciplinary assessment in these cases will help to avoid delays in diagnosis and prevent unnecessary surgery.

Snare technique for the remodeling of the redundant arachnoid pouch to prevent cerebrospinal fluid rhinorrhea and hematoma collection during transsphenoidal surgery for suprasellar-extended pituitary tumors.

OBJECTIVE Transsphenoidal surgery (TSS) is considered a most effective treatment for pituitary tumors with huge suprasellar extension. However, the chance of developing CSF leakage is relatively high, because tearing of the arachnoid membrane is common and there could be multiple tear points during the dissection of suprasellar tumors from the overlying arachnoid membrane. If there are multiple leaking points in the arachnoid membrane packing methods such as using fat or multilayered fascia graft may not be sufficient to seal off the leaking points. Moreover, the packing material may not provide sufficient tamponade to stop bleeding, and thus generates postoperative hematoma formation in the tumor resection cavity. To prevent these complications, the authors have developed a new technique for remodeling the redundant arachnoid pouch (the so-called snare technique) to reconstruct the diaphragm, seal off the CSF leak points completely, and reduce the dead space in the tumor resection cavity. METHODS In 9 patients with huge macroadenomas (> 2.5 cm in diameter) with suprasellar extension, the snare technique was used to remodel the arachnoid pouch after tumor removal via standard TSS between July 2009 and August 2014. Complications were investigated, including postoperative CSF rhinorrhea, postoperative hematoma collection, and visual compromise. RESULTS During the resection of the tumor, CSF leakage was encountered in 8 cases, all of which were sealed off using the snare technique. In 1 case without intraoperative CSF leakage, the snare technique was also applied after intentional puncturing of the arachnoid membrane to reduce the volume and tension of the arachnoid pouch. None of the 9 patients experienced postoperative CSF rhinorrhea. Lumbar CSF drainage was not required in any case. Magnetic resonance imaging studies performed 24 hours after surgery revealed a remarkable reduction in the height of the diaphragm in all cases. Visual deficits improved in all patients immediately after surgery. CONCLUSIONS Remodeling of the arachnoid pouch using the snare technique is simple and effective for completely sealing off the CSF leak point and preventing hematoma collection in the tumor resection cavity after TSS for huge pituitary tumors with suprasellar extension.