Regression of a nonfunctioning pituitary macroadenoma on the CDK4/6 inhibitor palbociclib: case report.

A 71-year-old female patient was referred in 2013 for evaluation of an asymptomatic nonsecreting pituitary adenoma. The adenoma, measuring 13 mm in height by 10 mm in width, was discovered incidentally on imaging in 2012. Biochemical testing demonstrated a nonfunctioning adenoma. Given the relatively small lesion size and the lack of symptoms, observation was preferred over surgical intervention. The patient was monitored with routine MRI, which until 2016 demonstrated minimal growth. In early 2016, the patient developed recurrence of metastatic breast cancer and was treated with palbociclib, a cyclin-dependent kinase (CDK) 4/6 inhibitor. This inhibitor acts on a pathway believed to be involved in pituitary adenoma tumorigenesis. One year after starting palbociclib, routine imaging demonstrated significant regression of her pituitary adenoma. The authors hypothesize that inhibition of the CDK4/6 pathway by palbociclib contributed to adenoma regression in this patient, and that palbociclib may represent a possible adjuvant therapy for the treatment of nonfunctioning pituitary adenomas.

Intramuscular myxoma of the cervical paraspinal musculature: case report and review of the literature.

CASE: A 57year old female patient presented with a painful right-sided neck mass that on MRI was shown to be adherent to the posterior aspect of the sternocleidomastoid muscle. The mass was surgically resected en bloc without complications. Histopathologic analysis revealed the mass to be a myxoma. REVIEW: To date, there have been several case reports of myxomas, although very few involving the head or neck. The majority of the literature available concurs that myxomas are benign neoplasms that exhibit characteristic qualities on MRI imaging. The definitive treatment by consensus is surgical excision. CONCLUSIONS: Although the incidence of head and neck myxomas is low, it is important to include in the differential of a neck mass with certain radiographic findings.

An endoscopic assisted retrosigmoid approach to the cerebello-pontine angle for resection of an epidermoid cyst.

Epidermoid cysts are rare lesions accounting for 1% of intracranial tumors with approximately 50% located within the cerebello-pontine angle (CPA). Resection is complicated by their close anatomical relation to critical neurovascular structures and their tendency to be densely adherent making complete removal a significant neurosurgical challenge. We present a 35-year-old woman with left sided tongue numbness and lower lip paresthesias with a CPA epidermoid. An endoscopic assisted retrosigmoid approach was utilized for resection. A 30-degree endoscope was used to assist in removal of unseen tumor in Meckel's cave, medial to the lower cranial nerves, and along the ventral pons. The video can be found here: http://youtu.be/bv0lMPbX7BY .

Endovascular Shunting for Communicating Hydrocephalus Using a Biologically Inspired Transdural Cerebrospinal Fluid Valved eShunt® Implant.

Cerebrospinal fluid (CSF) bathing the central nervous system is produced by brain and choroid plexus within the ventricles for re-absorption into the venous circulation through arachnoid granulations (AG). Communicating hydrocephalus results from disruption of the absorptive process, necessitating surgical catheter-based shunt placement to relieve excess pressure from CSF buildup. Adjustable valve designs and antibiotic impregnation have minimally impacted persistent failure rates and postoperative complications. To confront this challenge, we have developed an innovative endovascular shunt implant biologically inspired from AG function to restore the natural dynamics of CSF drainage while concurrently addressing the predominant factors contributing to conventional shunt malfunction.

Strangulation of the abducens nerve by an arachnoid band within an epidermoid cyst: illustrative case.

BACKGROUND: Intracranial epidermoid cysts (ECs) are rare benign lesions of ectodermal origin that can be found in the cerebellopontine angle (CPA). If large enough, they compress surrounding structures, causing cranial neuropathies, cerebellar dysfunction, and hydrocephalus. OBSERVATIONS: In this case report, the authors present a patient with headaches and diplopia secondary to a CPA EC. The cyst had wrapped itself around and was strangulating the abducens nerve. This choking of the nerve was believed to have been the cause of the patient's double vision. The arachnoid band was released with a myringotomy knife. LESSONS: Intracranial ECs typically cause symptoms from mass effect on surrounding structures; it is unusual for one to have choked off the nerve in the fashion presented in this case. Knowledge of the potential ways that ECs can affect nervous structures is critical for resecting them without injuring normal tissue.

International Tuberculum Sellae Meningioma Study: Surgical Outcomes and Management Trends.

BACKGROUND AND OBJECTIVES: Tuberculum sellae meningiomas (TSMs) can be resected through transcranial (TCA) or expanded endonasal approach (EEA). The objective of this study was to report TSM management trends and outcomes in a large multicenter cohort. METHODS: This is a 40-site retrospective study using standard statistical methods. RESULTS: In 947 cases, TCA was used 66.4% and EEA 33.6%. The median maximum diameter was 2.5 cm for TCA and 2.1 cm for EEA ( P < .0001). The median follow-up was 26 months. Gross total resection (GTR) was achieved in 70.2% and did not differ between EEA and TCA ( P = .5395). Vision was the same or better in 87.5%. Vision improved in 73.0% of EEA patients with preoperative visual deficits compared with 57.1% of TCA patients ( P < .0001). On multivariate analysis, a TCA (odds ratio [OR] 1.78, P = .0258) was associated with vision worsening, while GTR was protective (OR 0.37, P < .0001). GTR decreased with increased diameter (OR: 0.80 per cm, P = .0036) and preoperative visual deficits (OR 0.56, P = .0075). Mortality was 0.5%. Complications occurred in 23.9%. New unilateral or bilateral blindness occurred in 3.3% and 0.4%, respectively. The cerebrospinal fluid leak rate was 17.3% for EEA and 2.2% for TCA (OR 9.1, P < .0001). The recurrence rate was 10.9% (n = 103). Longer follow-up (OR 1.01 per month, P < .0001), World Health Organization II/III (OR 2.20, P = .0262), and GTR (OR: 0.33, P < .0001) were associated with recurrence. The recurrence rate after GTR was lower after EEA compared with TCA (OR 0.33, P = .0027). CONCLUSION: EEA for appropriately selected TSM may lead to better visual outcomes and decreased recurrence rates after GTR, but cerebrospinal fluid leak rates are high, and longer follow-up is needed. Tumors were smaller in the EEA group, and follow-up was shorter, reflecting selection, and observation bias. Nevertheless, EEA may be superior to TCA for appropriately selected TSM.

International Tuberculum Sellae Meningioma Study: Preoperative Grading Scale to Predict Outcomes and Propensity-Matched Outcomes by Endonasal Versus Transcranial Approach.

BACKGROUND AND OBJECTIVES: Tuberculum sellae meningiomas are resected via an expanded endonasal (EEA) or transcranial approach (TCA). Which approach provides superior outcomes is debated. The Magill-McDermott (M-M) grading scale evaluating tumor size, optic canal invasion, and arterial involvement remains to be validated for outcome prediction. The objective of this study was to validate the M-M scale for predicting visual outcome, extent of resection (EOR), and recurrence, and to use propensity matching by M-M scale to determine whether visual outcome, EOR, or recurrence differ between EEA and TCA. METHODS: Forty-site retrospective study of 947 patients undergoing tuberculum sellae meningiomas resection. Standard statistical methods and propensity matching were used. RESULTS: The M-M scale predicted visual worsening (odds ratio [OR]/point: 1.22, 95% CI: 1.02-1.46, P = .0271) and gross total resection (GTR) (OR/point: 0.71, 95% CI: 0.62-0.81, P < .0001), but not recurrence ( P = .4695). The scale was simplified and validated in an independent cohort for predicting visual worsening (OR/point: 2.34, 95% CI: 1.33-4.14, P = .0032) and GTR (OR/point: 0.73, 95% CI: 0.57-0.93, P = .0127), but not recurrence ( P = .2572). In propensity-matched samples, there was no difference in visual worsening ( P = .8757) or recurrence ( P = .5678) between TCA and EEA, but GTR was more likely with TCA (OR: 1.49, 95% CI: 1.02-2.18, P = .0409). Matched patients with preoperative visual deficits who had an EEA were more likely to have visual improvement than those undergoing TCA (72.9% vs 58.4%, P = .0010) with equal rates of visual worsening (EEA 8.0% vs TCA 8.6%, P = .8018). CONCLUSION: The refined M-M scale predicts visual worsening and EOR preoperatively. Preoperative visual deficits are more likely to improve after EEA; however, individual tumor features must be considered during nuanced approach selection by experienced neurosurgeons.

The New England Neurosurgical Society: growth and evolution over 70 years.

The New England Neurosurgical Society (NENS) was founded in 1951 under the leadership of its first President (Dr. William Beecher Scoville) and Secretary-Treasurer (Dr. Henry Thomas Ballantine). The purpose of creating the NENS was to unite local neurosurgeons in the New England area; it was one of the first regional neurosurgical societies in America. Although regional neurosurgical societies are important supplements to national organizations, they have often been overshadowed in the available literature. Now in its 70th year, the NENS continues to serve as a platform to represent the needs of New England neurosurgeons, foster connections and networks with colleagues, and provide research and educational opportunities for trainees. Additionally, regional societies enable discussion of issues uniquely relevant to the region, improve referral patterns, and allow for easier attendance with geographic proximity. In this paper, the authors describe the history of the NENS and provide a roadmap for its future. The first section portrays the founders who led the first meetings and establishment of the NENS. The second section describes the early years of the NENS and profiles key leaders. The third section discusses subsequent neurosurgeons who steered the NENS and partnerships with other societies. In the fourth section, the modern era of the NENS and its current activities are highlighted.

Gastrointestinal prophylaxis in neurocritical care.

The aim of this study is to review and summarize the relevant literature regarding pharmacologic and non-pharmacologic methods of prophylaxis against gastrointestinal (GI) stress ulceration, and upper gastrointestinal bleeding in critically ill patients. Stress ulcers are a known complication of a variety of critical illnesses. The literature regarding epidemiology and management of stress ulcers and complications thereof, is vast and mostly encompasses patients in medical and surgical intensive care units. This article aims to extrapolate meaningful data for use with a population of critically ill neurologic and neurosurgical patients in the neurological intensive care unit setting. Studies were identified from the Cochrane Central Register of controlled trials and NLM PubMed for English articles dealing with an adult population. We also scanned bibliographies of relevant studies. The results show that H(2)A, sucralfate, and PPI all reduce the incidence of UGIB in neurocritically ill patients, but H(2)A blockers may cause encephalopathy and interact with anticonvulsant drugs, and have been associated with higher rates of nosocomial pneumonias, but causation remains unproven and controversial. For these reasons, we advocate against routine use of H(2)A for GI prophylaxis in neurocritical patients. There is a paucity of high-level evidence studies that apply to the neurocritical care population. From this study, it is concluded that stress ulcer prophylaxis among critically ill neurologic and neurosurgical patients is important in preventing ulcer-related GI hemorrhage that contributes to both morbidity and mortality. Further, prospective trials are needed to elucidate which methods of prophylaxis are most appropriate and efficacious for specific illnesses in this population.

Retrosigmoid Craniectomy with a Layered Soft Tissue Dissection and Hydroxyapatite Reconstruction: Technical Note, Surgical Video, Regional Anatomy, and Outcomes.

Introduction There are many reported modifications to the retrosigmoid approach including variations in skin incisions, soft tissue dissection, bone removal/replacement, and closure. Objective The aim of this study was to report the technical nuances developed by two senior skull base surgeons for retrosigmoid craniectomy with reconstruction and provide anatomic dissections, surgical video, and outcomes. Methods The regional soft tissue and bony anatomy as well as the steps for our retrosigmoid craniectomy were recorded with photographs, anatomic dissections, and video. Records from 2017 to 2019 were reviewed to determine the incidence of complications after the authors began using the described approach. Results Dissections of the relevant soft tissue, vascular, and bony structures were performed. Key surgical steps are (1) a retroauricular C-shaped skin incision, (2) developing a skin and subgaleal tissue flap of equal thickness above the fascia over the temporalis and sub-occipital muscles, (3) creation of subperiosteal soft tissue planes over the top of the mastoid and along the superior nuchal line to expose the suboccipital region, (4) closure of the craniectomy defect with in-lay titanium mesh and overlay hydroxyapatite cranioplasty, and (5) reapproximation of the soft tissue edges during closure. Complications in 40 cases were pseudomeningocele requiring shunt ( n = 3, 7.5%), wound infection ( n = 1, 2.5%), and aseptic meningitis ( n = 1, 2.5%). There were no incisional cerebrospinal fluid leaks. Conclusion The relevant regional anatomy and a revised technique for retrosigmoid craniectomy with reconstruction have been presented with acceptable results. Readers can consider this technique when using the retrosigmoid approach for pathology in the cerebellopontine angle.

First-in-human endovascular treatment of hydrocephalus with a miniature biomimetic transdural shunt.

Surgical ventriculoperitoneal shunting remains standard treatment for communicating hydrocephalus, despite persistently elevated infection and revision rates. A novel minimally invasive endovascular cerebrospinal fluid (CSF) shunt was developed to mimic the function of the arachnoid granulation which passively filters CSF from the central nervous system back into the intracranial venous sinus network. The endovascular shunt is deployed via a femoral transvenous approach across the dura mater into the cerebellopontine angle cistern. An octogenarian with intractable hydrocephalus following subarachnoid hemorrhage underwent successful endovascular shunting, resulting in swift intracranial pressure reduction from 38 to <20 cmH2O (<90 min) and resolution of ventriculomegaly. This first successful development of a percutaneous transluminal venous access to the central nervous system offers a new pathway for non-invasive treatment of hydrocephalus and the potential for intervention against neurological disorders.

Primary Spinal Cord Astrocytomas: Two-Center Clinical Experience of Low- and High-Grade Lesions.

OBJECTIVE: Primary spinal cord astrocytomas are rare, fatal, and poorly studied. METHODS: This study included a 2-center, retrospective analysis of primary spinal cord astrocytoma patients from 1997 to 2020. Patients with drop metastases or without at least one follow-up were excluded. RESULTS: Seven World Health Organization grade I, 6 grade II, 7 grade III, and 4 grade IV astrocytoma patients were included. Older patients had higher grades (median 20 years in grade I vs. 36.5 in grade IV). The median follow-up was 15 months. Thirteen patients were discharged to rehabilitation. Eight patients demonstrated radiographic progression. Adjuvant therapy was utilized more in higher grades (5 of 13 grades III vs. all 11 grades IIIIV). Six patients died (1 death in grades III vs. 5 in grades IIIIV). Ten patients had worsened symptoms at the last follow-up. The median progression-free survival in grade I, II, III, and IV tumors was 116, 36, 8, and 8.5 months, respectively. The median overall survival in grade I, II, III, and IV tumors was 142, 69, 19, and 12 months, respectively. Thrombotic complications occurred in 2 patients, one with isocitrate dehydrogenasewild type glioblastoma. CONCLUSIONS: Outcomes worsen with higher grades and lead to difficult postoperative periods. Clinicians should be vigilant for thromboembolic complications. Further research is needed to understand these rare tumors.

Complete endoscopic removal of colloid cyst using a nitinol basket retriever.

Neuroendoscopic treatment of colloid cysts is limited by the reach and flexibility of the instruments that can be passed through the working channels of the rigid neuroendoscope. The authors describe a case of a third ventricular colloid cyst where a large solid colloid fragment was recovered using a nitinol stone retrieval basket as a flexible wall-guided atraumatic salvage instrument. A flexible nitinol stone retrieval basket was successfully used through an endoscopic working channel to retrieve a large portion of the colloid cyst from the occipital horn of the lateral ventricle in a 70-year-old man who presented with progressive memory loss, urinary incontinence, and slowness of gait. A flexible nitinol stone retrieval basket can be safely and effectively maneuvered in the ventricular system, using the ventricular wall for deflection, and can be used to retrieve colloid cyst fragments as a salvage technique. Remaining free-floating large colloid cyst fragments in the ventricular system do not necessarily require a second craniotomy or bur hole for access but may be retrieved using a nitinol stone retrieval basket.

Hemangiopericytomas of the skull base.

OBJECT: Intracranial hemangiopericytomas are frequently located along the dural sinuses along the skull base and represent rare, aggressive CNS neoplasms that are difficult to distinguish from meningiomas based on both imaging and gross characteristics. The authors of this study describe 3 patients with these lesions and review the pertinent literature. METHODS: Two men and 1 woman, whose median age at the time of the initial presentation was 37 years (range 20-53 years), constitute this series. They underwent multimodal treatment consisting of resection, embolization, radiation therapy, and in 1 case chemotherapy. RESULTS: Two of the 3 patients treated were alive after a mean follow-up of 93 months (range 4-217 months). One patient died 217 months after the initial diagnosis. The longest tumor progression-free interval after the initial or secondary resection was 43 months (range 4-84 months). CONCLUSIONS: Hemangiopericytomas have been reclassified as mesenchymal non-meningothelial tumors. They have an inevitable tendency to recur locally and metastasize distally. The mainstay of therapy remains an aggressive attempt to achieve gross-total resection at the initial surgery. Postoperative adjuvant radiotherapy should be offered to all patients, regardless of the degree of resection achieved. Diligent long-term follow-up is paramount as local recurrences and distal metastases can develop sometimes years after the initial treatment.

Pneumosinus dilitans and meningioma: a case series and review of the literature.

Pneumosinus dilitans (PSD) is enlargement of the sinuses of the skull base and is frequently seen with meningiomas. Identifying PSD on imaging can assist with operative planning and preparation. Meningiomas associated with PSD are not more commonly high grade, and complete resection is frequently possible.

Endoscopic Endonasal Versus Transoral Odontoidectomy for Non-Neoplastic Craniovertebral Junction Disease: A Case Series.

BACKGROUND: Odontoidectomy is a challenging yet effective operation for decompression of non-neoplastic craniovertebral junction disease. Though both the endoscopic endonasal approach (EEA) and the transoral approach (TOA) have been discussed in the literature, there remain few direct comparisons between the techniques. OBJECTIVE: To evaluate the perioperative outcomes of EEA vs TOA odontoidectomy. METHODS: A retrospective review of all cases undergoing odontoidectomy by either the EEA or TOA was performed. Attention was paid to the need for prolonged nutritional support, prolonged respiratory support, and hospitalization times. RESULTS: During the study period between 2000 and 2018, 25 patients underwent odontoid process resection (18 TOA and 7 EEA). The most common indication for surgery was basilar invagination. Hospital length of stay, intensive care unit length of stay, and intubation days were all significantly shorter in the EEA group compared to the TOA group (P < .01, P = .01, P < .01, respectively). Prolonged nutritional support in the form of a gastrostomy tube was required in 5 patients and tracheostomy was required in 4 patients; all of these underwent odontoidectomy by the TOA. There was no statistical difference in neurological outcomes between the EEA and TOA groups (P = .17). CONCLUSION: Odontoidectomy can be performed safely through both the EEA and TOA. The results of this study suggest the EEA has shorter hospitalizations and a lower probability of requiring prolonged nutritional support. These advantages are likely the results of decreased oropharyngeal mucosa disruption as compared to the TOA.

Primary meningeal melanoma masquerading as neurofibromatosis type 2: illustrative case.

BACKGROUND: Primary meningeal melanocytic neoplasms are exceedingly rare tumors, representing only 0.06% to 0.1% of all primary brain tumors and ranging in spectrum from benign localized tumors to highly aggressive malignant lesions. The diagnosis of these tumors is often challenging from clinical, radiological, and pathologic standpoints. Equally challenging is the distinction between primary meningeal melanocytic neoplasm and metastatic melanoma. OBSERVATIONS: The authors reported the case of a 41-year-old man with imaging findings diagnostic of neurofibromatosis type 2: bilateral internal auditory canal lesions (most consistent with bilateral vestibular schwannomas), two dura-based lesions presumed to be meningiomas, multiple spinal lesions consistent with peripheral nerve sheath tumors, and one intramedullary spinal lesion consistent with an ependymoma. Biopsy of these lesions revealed melanocytic neoplasms with mild to moderate atypia and a mildly elevated proliferation index, which made the distinction between benign and malignant challenging. In addition, the disseminated nature of these tumors made it difficult to determinate whether they arose from the meninges or represented metastases from an occult primary melanoma. LESSONS: This case illustrated the challenges presented by the diagnosis of meningeal melanocytic neoplasms and highlighted the importance of integrating the clinical and radiographic findings with histologic appearance and molecular studies.

Clinical and histopathological principles for the diagnosis of a recurrent paraganglioma of the jugular foramen initially diagnosed as a middle ear adenoma: illustrative case.

BACKGROUND: Paragangliomas (PGLs) are rare neoplasms that may be associated with hereditary PGL syndromes and variable risk of metastasis. Middle ear adenomas are extremely rare tumors with no known hereditary predisposition and extremely low risk of metastasis. Although often easily differentiated, they may share clinical and pathological features that misdirect and confuse the diagnosis. OBSERVATIONS: The authors discussed a 35-year-old woman with left-sided hearing loss and bleeding from the external ear canal who presented to an outside hospital. She underwent resection of a middle ear and mastoid mass, initially diagnosed as a middle ear adenoma with neuroendocrine features, with later mastoidectomy and ligation of the sigmoid sinus with microsurgical excision of persistent tumor in the jugular foramen and temporal bone. Histopathologically, her tumor was vascular, composed of benign-appearing epithelioid cells with "salt and pepper" neuroendocrine chromatin arranged in vague nests. Lesional cells were GATA3-immunopositive, glucagon-negative, and succinate dehydrogenase-immunonegative, consistent with PGL rather than middle ear adenoma, and required further workup for hereditary PGL syndromes. LESSONS: This case demonstrates potential challenges in differentiating a PGL from a middle ear adenoma. The authors offer clinical, histopathological, and imaging principles to aid in diagnosis and workup.

Pneumocephalus: case illustrations and review.

BACKGROUND: Pneumocephalus is commonly encountered after neurosurgical procedures but can also be caused by craniofacial trauma and tumors of the skull base and rarely, can occur spontaneously. Contributing factors for the development of pneumocephalus include head position, duration of surgery, nitrous oxide (N(2)O) anesthesia, hydrocephalus, intraoperative osmotherapy, hyperventilation, spinal anesthesia, barotauma, continuous CSF drainage via lumbar drain, epidural anesthesia, infections, and neoplasms. Clinical presentation includes headaches, nausea and vomiting, seizures, dizziness, and depressed neurological status. In this article, we review the incidence, mechanisms, precipitating factors, diagnosis, and management of pneumocephalus. Search of Medline, databases, and manual review of article bibliographies. Considering four case illustrations that typify pneumocephalus in clinical practice, we discuss the common etiologies, and confirm the diagnosis with neuroimaging and management strategies. Avoidance of contributing factors, high index of suspicion, and confirmation with neuroimaging are important in attenuating mortality and morbidity. A significant amount of pneumocephalus can simulate a space-occupying lesion. Supplemental oxygen increases the rate of absorption of pneumocephalus.

Colloid Cysts: Evolution of Surgical Approach Preference and Management of Recurrent Cysts.

BACKGROUND: Optimal management of third ventricular colloid cysts remains debated. While microsurgery offers greater resection rates and lower recurrences, endoscopy offers a perceived less invasive option. OBJECTIVE: To describe the evolution of our practice to favor microsurgery and determine the optimal management of recurrent colloid cysts. METHODS: Any patient having undergone surgery for a colloid cyst by the senior author was identified and included in the study cohort. Clinical, radiographic, and operative records were reviewed, with attention paid to those patients requiring recurrent surgery. RESULTS: Thirty-three patients were treated for intracranial colloid cysts between 1995 and 2017. Two patients had initial surgical treatment at an outside institution prior to presentation at our institution. Microsurgery was used in 15/31 initial cases, endoscopy in 13 cases, and ventriculoperitoneal shunting in 3 cases. Between 1995 and 2005, 89% of colloid cysts (8/9 cases) were resected endoscopically, whereas 74% of colloid cysts (14/19 cases) were resected by microsurgery after 2005. Of the 13 patients treated endoscopically, 6 (46%) required surgery for recurrent cysts. Incomplete cyst wall resection at the initial operation increased the recurrence rate to 55%. There were no recurrences in the microsurgery cohort. CONCLUSION: Surgical resection of recurrent colloid cysts should focus on complete removal of the cyst wall to minimize the chance of recurrence. Microsurgery has been shown to provide the highest success rates for cyst wall resection and lowest rates of recurrence and is therefore recommended for patients undergoing surgery for primary and recurrent colloid cysts.