Skull base hemangiopericytomas.

OBJECTIVE: To retrospectively evaluate the clinical outcome of six patients with skull base hemangiopericytomas (HPCs) and that of a cohort of 37 similar patients identified by a systematic review of the literature. METHODS: The series constitutes of three men and three women with newly diagnosed skull base HPC who underwent multimodal treatment including surgery, external beam radiotherapy (EBRT) and pre-operative embolization. Furthermore, a systematic review off the literature identified 37 reports of primarily intracranial skull base HPCs. RESULTS: Four patients had a gross total resection (GTR) and two patients had a near total resection. Five patients were referred for adjuvant EBRT with a survival ranging from 15 to 47 months. All patients had an excellent outcome and resumed their previous activities. Literature review identified 37 additional patients with skull base HPC. Altogether, tumors were unevenly distributed above and below tentorium. GTR was achieved in half the patients, and 72.1% were referred to EBRT. Out of 37 reported patients in the literature, survival longer than 1 year was described in only 24. Within the combined cohort including the present series, survival was 83.6 months. CONCLUSIONS: The present series shows that a radical resection of HPC can be achieved under the difficult anatomical conditions of skull base surgery. Pre-operative arterial embolization may be instrumental to maintain a clear visual field and prevent excessive blood loss. Finally, the results of the present cohort suggest that EBRT may be useful for local growth control, as an effective palliative measure for skull base HPCs.

IgG4-related pachymeningitis masquerading as foramen magnum meningioma: illustrative case.

BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated inflammatory condition with potential multiorgan involvement. Common manifestations include autoimmune pancreatitis and retroperitoneal fibrosis. Pathological analysis reveals lymphoplasmacytic infiltrate rich in IgG4-positive cells and characteristic storiform fibrosis. Early treatment with glucocorticoids may prevent progression to poorly responsive fibrotic disease. OBSERVATIONS: A 63-year-old female patient presented with reports of left-sided headaches, nausea, and photophobia in addition to recently diagnosed chronic rhinosinusitis (CRS). Neurological examination revealed dysarthria secondary to left hypoglossal nerve palsy. Computed tomography (CT) revealed a contrast-enhancing extraaxial mass at the left craniocervical junction, CRS with secondary hyperostotic reaction, and multiple hypodense lesions involving the occipital bone. Magnetic resonance imaging revealed a dural-based lesion involving the foramen magnum and invading the left hypoglossal canal. The patient underwent a far-lateral craniotomy. Histopathological analysis revealed severe lymphoplasmacytic inflammation, storiform fibrosis and rich plasma-cell population positive for IgG4. Serum IgG4 was markedly elevated. Total-body CT showed no systemic involvement. The patient was diagnosed with IgG4-RD and was prescribed prednisone, with normalization of her IgG4 levels after 1 month. LESSONS: IgG4-RD may mimic a variety of diseases, including skull-base meningiomas and CRS. Accurate diagnosis and expedited administration of steroids may prevent unnecessary interventions and progression to treatment-resistant fibrosis.

Sodium glucose cotransporter 2 inhibitors treatment in acromegalic patients with diabetes-a case series and literature review.

PURPOSE: Diabetes mellitus (DM) represents one of the most frequent comorbidities in patients with acromegaly. Sodium-glucose cotransporter 2 inhibitors (SGLT2i) represent an important class for diabetes management. However, limited data is reported regarding the use of this class in patients with acromegaly and diabetes. METHODS: Reporting data regarding patients with acromegaly and diabetes under treatment with SGLT2i. RESULTS: 29 acromegalic patients with diabetes were identified. Treatment with SGLT-2i was documented in nine patients, out of them 5 females and 4 males with a mean age (SD) of 61 ± 12 yr. The mean (SD) duration of treatment with SGLT2i was 27.5 ± 7.3 months. Mean HbA1c before and after SGLT-2i initiation was 8.1 ± 1.1 and 7.0 ± 0.9% respectively. Mean IGF-1 level (SD) before SGLT-2i initiation was 177 ± 68 ng/mL and the mean GH level (SD) was 0.7 ± 0.5 µg/L. All nine patients are still under treatment with SGLT2i and none of them had reported any adverse reaction related to SGLT2i. CONCLUSIONS: The present article provides us for the first time with new data regarding the use of SGLT2i among acromegalic patients with diabetes.

Comparison of MRI techniques for detecting microadenomas in Cushing's disease.

OBJECTIVE Many centers use conventional and dynamic contrast-enhanced MRI (DMRI) sequences in patients with Cushing's disease. The authors assessed the utility of the 3D volumetric interpolated breath-hold examination, a spoiled-gradient echo 3D T1 sequence (SGE) characterized by superior soft tissue contrast and improved resolution, compared with DMRI and conventional MRI (CMRI) for detecting microadenomas in patients with Cushing's disease. METHODS This study was a blinded assessment of pituitary MRI in patients with proven Cushing's disease. Fifty-seven patients who had undergone surgery for Cushing's disease (10 male, 47 female; age range 13-69 years), whose surgical findings were considered to represent a microadenoma, and who had been examined with all 3 imaging techniques were included. Thus, selection emphasized patients with prior negative or equivocal MRI on referral. The MRI annotations were anonymized and 4 separate imaging sets were independently read by 3 blinded, experienced clinicians: a neuroradiologist and 2 pituitary surgeons. RESULTS Forty-eight surgical specimens contained an adenoma (46 ACTH-staining adenomas, 1 prolactinoma, and 1 nonfunctioning microadenoma). DMRI detected 5 adenomas that were not evident on CMRI, SGE detected 8 adenomas not evident on CMRI, including 3 that were not evident on DMRI. One adenoma was detected on DMRI that was not detected on SGE. McNemar's test for efficacy between the different MRI sets for tumor detection showed that the addition of SGE to CMRI increased the number of tumors detected from 18 to 26 (p = 0.02) based on agreement of at least 2 of 3 readers. CONCLUSIONS SGE shows higher sensitivity than DMRI for detecting and localizing pituitary microadenomas, although rarely an adenoma is detected exclusively by DMRI. SGE should be part of the standard MRI protocol for patients with Cushing's disease.

Long-term results of endonasal endoscopic transsphenoidal resection of nonfunctioning pituitary macroadenomas.

BACKGROUND: Several studies report early results of endoscopic endonasal transsphenoidal surgery; however, none discuss long-term outcome measures such as tumor recurrence rates and the need for additional surgical procedures. OBJECTIVE: To discuss the long-term outcomes after endoscopic endonasal transsphenoidal surgery for nonfunctioning pituitary macroadenomas. METHODS: This is a retrospective study. Patients were included only if they had at least 5 years of clinical and imaging follow-up after surgery. RESULTS: Eighty patients met the study criteria. Grossly complete resection was achieved in 71% of patients. Knosp grade 0 to 2 tumors and tumor with volumes <10 cm were significantly more likely to have received a grossly complete resection. There were 7 recurrences (12%) in patients who had received grossly complete resections, with a mean time to recurrence of 53 months. Among the 23 patients who had subtotal resections, 11 (61%) progressed radiographically, and 3 (17%) had symptomatic progression. Knosp score, surgical and radiographic evidence of invasion, and preoperative visual deficits were predictive of recurrence in a univariate analysis, but Knosp grade was the only independent predictor in a multivariate analysis. Kaplan-Meier analysis projected a 10-year progression-free survival rate of 80% and 21% for patients with gross total resections and subtotal resections, respectively. CONCLUSION: At the long-term follow-up, 12% of patients had recurrent tumors after grossly complete resection. Recurrent or residual tumors were treated with either repeat surgery or Gamma Knife radiosurgery. Rates of complete resection, postoperative surgical and endocrinological complications, and additional surgical procedures are similar to previously published reports after microscopic transsphenoidal surgery.

A Case for Conservative Management: Characterizing the Natural History of Radiographically Diagnosed Rathke Cleft Cysts.

CONTEXT: Rathke cleft cysts (RCCs) are benign embryonic remnants of the Rathke's pouch found in 13% to 33% of the general population. When symptomatic, they manifest themselves by compressing adjacent structures, causing pressure effects such as headache, visual disturbance, or pituitary hormone deficits. Most RCCs are asymptomatic, and their management remains controversial. Surgical resection has generally been indicated to treat symptomatic RCCs but carries the risk of complications. OBJECTIVE: Our objective was to better characterize the outcomes for patients with presumed RCCs undergoing conservative management. DESIGN: This was a retrospective cohort study. SETTING: The setting was a pituitary program at a university medical center. PARTICIPANTS: The participants were 75 patients with radiographically diagnosed RCCs. METHODS: All brain magnetic resonance imaging (MRI) scans performed at the University of Virginia from 2006 through 2013 were searched for the words "Rathke cleft cyst," and pituitary clinic notes from 2007 to 2012 were reviewed for patients identified as probably having an RCC. Images for all patients were reviewed by the interpreting neuroradiologist, and those patients with at least 2 MRI scans were included. The dimensions of each cyst were assessed by the same neuroradiologist, and the volume of each cyst was analyzed as a function of the time from the first image obtained. RESULTS: A total of 75 patients (4-76 years old) met our inclusion criteria. The length of follow-up was 1 to 126 months (median 24 months). In 43 patients (57%) no detectable change in the size of their cysts was seen, in 21 patients (28%) cysts increased in size, and in 11 patients (15%) cysts decreased in size. The predicted mean cyst growth rate was not significantly different from 0. CONCLUSION: The increasingly prevalent use of brain imaging modalities such as MRI has resulted in an increase in the incidental discovery of pituitary lesions. Our study demonstrates that the majority of radiologically diagnosed RCCs remain unchanged or decrease in size over time. These results suggest that, in the absence of pressure symptoms, it is reasonable to manage patients with RCCs conservatively.

Delayed contrast extravasation MRI: a new paradigm in neuro-oncology.

BACKGROUND: Conventional magnetic resonance imaging (MRI) is unable to differentiate tumor/nontumor enhancing tissues. We have applied delayed-contrast MRI for calculating high resolution treatment response assessment maps (TRAMs) clearly differentiating tumor/nontumor tissues in brain tumor patients. METHODS: One hundred and fifty patients with primary/metastatic tumors were recruited and scanned by delayed-contrast MRI and perfusion MRI. Of those, 47 patients underwent resection during their participation in the study. Region of interest/threshold analysis was performed on the TRAMs and on relative cerebral blood volume maps, and correlation with histology was studied. Relative cerebral blood volume was also assessed by the study neuroradiologist. RESULTS: Histological validation confirmed that regions of contrast agent clearance in the TRAMs >1 h post contrast injection represent active tumor, while regions of contrast accumulation represent nontumor tissues with 100% sensitivity and 92% positive predictive value to active tumor. Significant correlation was found between tumor burden in the TRAMs and histology in a subgroup of lesions resected en bloc (r(2) = 0.90, P < .0001). Relative cerebral blood volume yielded sensitivity/positive predictive values of 51%/96% and there was no correlation with tumor burden. The feasibility of applying the TRAMs for differentiating progression from treatment effects, depicting tumor within hemorrhages, and detecting residual tumor postsurgery is demonstrated. CONCLUSIONS: The TRAMs present a novel model-independent approach providing efficient separation between tumor/nontumor tissues by adding a short MRI scan >1 h post contrast injection. The methodology uses robust acquisition sequences, providing high resolution and easy to interpret maps with minimal sensitivity to susceptibility artifacts. The presented results provide histological validation of the TRAMs and demonstrate their potential contribution to the management of brain tumor patients.

Retrospective analysis of a concurrent series of microscopic versus endoscopic transsphenoidal surgeries for Knosp Grades 0-2 nonfunctioning pituitary macroadenomas at a single institution.

OBJECT: The object of this study was to compare surgical outcomes and complications in a contemporaneous series of patients undergoing either microscopic or endoscopic transsphenoidal surgery for nonfunctioning pituitary macroadenomas without imaging evidence of cavernous sinus invasion. METHODS: This is a retrospective analysis of a prospectively collected database from a single institution. Data were collected from patients whose surgery had occurred in the period from June 2010 to January 2013. Patients who underwent microscopic or endoscopic surgery for Knosp Grade 0, 1, or 2 nonfunctioning pituitary macroadenomas were included in the study. Patients who had clinically secreting or Knosp Grade 3 or 4 tumors and patients who were undergoing revision surgery were excluded from analysis. Eligible patient records were analyzed for outcomes and complications. Statistical analyses were performed on tumor volume, intraoperative factors, postoperative complications, and degree of resection on 1-year postoperative MRI. The results were used to compare the outcomes after microscopic and endoscopic approaches. RESULTS: Forty-three patients underwent microscopic transsphenoidal surgery, and 56 underwent endoscopic transsphenoidal surgery. There were no statistical differences in the intraoperative extent of resection or endocrinological complications. There were significantly more intraoperative CSF leaks in the endoscopic group (58% vs 16%); however, there was no difference in the incidence of postoperative CSF rhinorrhea (12% microscopic vs 7% endoscopic). Length of hospitalization was significantly shorter in patients undergoing an endoscopic approach (3.0 days vs 2.4 days). Two-month follow-up imaging was available in 95% of patients, and 75% of patients had 1-year follow-up imaging. At 2 months postprocedure, there was no evidence of residual tumor in 79% (31 of 39) and 85% (47 of 55) of patients in the microscopic and endoscopic groups, respectively. At 1 year postprocedure, 83% (25 of 30) of patients in the microscopic group had no evidence of residual tumor and 82% (36 of 44) of those in the endoscopic group had no evidence of residual tumor. CONCLUSIONS: The microscopic and endoscopic techniques provide similar outcomes in the surgical treatment of Knosp Grades 0-2 nonfunctioning pituitary macroadenomas.

Delayed contrast extravasation MRI for depicting tumor and non-tumoral tissues in primary and metastatic brain tumors.

The current standard of care for newly diagnosed glioblastoma multiforme (GBM) is resection followed by radiotherapy with concomitant and adjuvant temozolomide. Recent studies suggest that nearly half of the patients with early radiological deterioration post treatment do not suffer from tumor recurrence but from pseudoprogression. Similarly, a significant number of patients with brain metastases suffer from radiation necrosis following radiation treatments. Conventional MRI is currently unable to differentiate tumor progression from treatment-induced effects. The ability to clearly differentiate tumor from non-tumoral tissues is crucial for appropriate patient management. Ten patients with primary brain tumors and 10 patients with brain metastases were scanned by delayed contrast extravasation MRI prior to surgery. Enhancement subtraction maps calculated from high resolution MR images acquired up to 75 min after contrast administration were used for obtaining stereotactic biopsies. Histological assessment was then compared with the pre-surgical calculated maps. In addition, the application of our maps for prediction of progression was studied in a small cohort of 13 newly diagnosed GBM patients undergoing standard chemoradiation and followed up to 19.7 months post therapy. The maps showed two primary enhancement populations: the slow population where contrast clearance from the tissue was slower than contrast accumulation and the fast population where clearance was faster than accumulation. Comparison with histology confirmed the fast population to consist of morphologically active tumor and the slow population to consist of non-tumoral tissues. Our maps demonstrated significant correlation with perfusion-weighted MR data acquired simultaneously, although contradicting examples were shown. Preliminary results suggest that early changes in the fast volumes may serve as a predictor for time to progression. These preliminary results suggest that our high resolution MRI-based delayed enhancement subtraction maps may be applied for clear depiction of tumor and non-tumoral tissues in patients with primary brain tumors and patients with brain metastases.