INNV-26. NCI-CONNECT PROGRAM PROVIDES RESOURCES TO IMPROVE CARE FOR PEOPLE WITH RARE CENTRAL NERVOUS SYSTEM (CNS) TUMORS

The National Cancer Institute Comprehensive Oncology Network Evaluating Rare CNS Tumors (NCI-CONNECT) program focuses on improving approaches to care and treatment for 12 rare CNS tumors, each with < 2,000 diagnoses per year. To reach this rare population, the primary objective was to develop a website to share educational resources that provide patients direct access to clinical care and trials. METHODS: The NCI-CONNECT website uses the Drupal platform within NCI’s framework and the content is free to syndicate. A multidisciplinary team developed tumor-specific content in English and Spanish, clinical trial information, and survivorship resources using evidence-based sources. The Central Brain Tumor Registry of the United States provided statistics on incidence and prevalence of rare tumors. Population reach was calculated using Adobe Experience Cloud website analytics. NCI-CONNECT referrals and study participation data were collected prospectively. RESULTS: The English website launched in September 2018 and visits have increased 2,384%. The Spanish website launched in March 2020 and visits have increased 1,137%. From April 2020 to March 2021, top website page views by English page views / Spanish page views / people living with this disease include oligodendroglioma (43,859 / 8,241 / 11,757), ependymoma (31,579 / 12,684 / 13,294), meningioma (30,261 / 19,507 / 2,692), medulloblastoma (28,487 / 9,999 / 3,840), diffuse midline gliomas (23,064 / 3,851 / 6,033), and pineal region tumors (19,939 / 9,973 / 1,297). Referral rates and participation have accelerated – 45% of patients visiting the Neuro-Oncology Clinic at NIH have a rare CNS tumor and 409 patients enrolled in an NCI-CONNECT study. CONCLUSION: Patient-focused websites can provide guidance to those affected by rare cancers outside of in-person health care visits. The NCI-CONNECT website is an educational and clinical resource for patients and families affected by rare CNS tumors and was created to raise awareness and improve patient outcomes.

Occipital transtentorial approach for pineal region lesions: Addressing the controversies in conventional teaching

Background: The occipital transtentorial (OT) approach is well-established approach for pineal region tumors and can be of choice for the lesions located around the suboccipital part of tentorium such as the quadrigeminal plate, posterior part of thalamus, tentorial surface of cerebellum, splenial region, posterior falx, and lesions around the tentorial incisura. However, it is not very much extensively used in the above-mentioned locations other than the pineal region. Methods: Thirty-one patients of pineal region lesions were operated by OT approach, the role of conventional preoperative evaluation of the anatomy of the venous sinuses, deep venous system, and tentorial angle was investigated. Results: A variety of lesions were operated using this approach achieving gross and near total resection in majority of the cases (76.6%), with acceptable postoperative mean modified Rankin scales (1.8). Conclusion: The OT is a preferable approach for pineal region lesions for patients of all ages and can be tailored for achieving high resectability rates irrespective of the status of the deep venous system and tentorial angle, with reasonable postoperative surgical outcome.

Endoscope-Assisted Microsurgery in Pediatric Cases With Pineal Region Tumors: A Study of 18 Cases Series

Background: An endoscope-assisted technique was recently introduced to microsurgery (MS) and may compensate for the disadvantages of MS for deep-seated lesions. This study was performed to identify the effectiveness and safety of endoscopic-assisted microsurgery (EAM) and share our experience of EAM for pediatric cases with pineal region tumors.Method: We retrospectively analyzed the clinical data of consecutive pediatric cases with pineal region tumors treated by EAM or MS from January 2016 to June 2020. These data included the patient population, clinical manifestations, preoperative examination findings, surgical approach, pathological results, and clinical outcomes. The clinical outcomes were analyzed in the EAM group and MS group with a focus on the gross total resection (GTR) rate, postoperative hydrocephalus remission rate, and Karnofsky performance score (KPS). Studies on the surgical management of children with pineal region tumors in the last decade were reviewed.Result: Eighteen children successfully underwent tumor resection via MS (n = 8) or EAM (n = 10). The children's mean age was 11.4 ± 4.7 years, and the male to female ratio was 7:2. Seventeen patients (94.4%) complicated preoperative hydrocephalus, and 16 (88.9%) presented headache with nausea and/or vomiting. The pathological examination revealed germ cell tumors in 11 (61.1%) patients, neuroepithelial tumors in 4 (22.2%) patients, and a pineoblastoma, arachnoid cyst, and atypical teratoid rhabdoid tumor in 1 (5.6%) patient each. GTR was more commonly achieved in the EAM than MS group (80.0 vs. 50.0%, respectively), and the postoperative hydrocephalus remission rate was higher in the EAM than MS group (87.5 vs. 50.0%, respectively). At a mean follow-up time of 23.6 ± 11.5 weeks, the mean improvement of the KPS 6 months postoperatively was greater in the EAM than MS group (24.0 ± 9.7 vs. 17.5 ± 7.1 points, respectively).Conclusion: EAM combines endoscopic and microsurgical techniques and can be safely and effectively performed to achieve GTR of pineal region tumors in pediatric patients. In children with pineal region tumors who have obstructive hydrocephalus, EAM could improves hydrocephalus remission rates by checking and clearing the midbrain aqueduct under visualization.

Pleomorphic xanthoastrocytoma at the pineal region: rare case report with successful surgical management

Background Pineal region tumors are commonly present in the pediatric age group. However, pleomorphic xanthoastrocytoma (PXA) is very rare at this region, and only few case reports have been reported till now in literature. Case presentation Here, we report a rare case of pineal region, juxta-thalamic, pleomorphic xanthoastrocytoma (PXA) in an 11-year-old male child. The child presented with severe headache after which MRI was done. It was suggestive of pineal region low-grade tumor. The patient was operated in Parkbench position with SCIT (supracerebellar approach) in a retractor-free manner. Gross total resection was done. However, the patient developed postoperative left-sided hemiparesis. It got improved in 1 month, and the patient became ambulatory. Histopathology came out as pleomorphic xanthoastrocytoma. Conclusion Surgical management should include careful resection near the internal capsule to avoid postoperative hemiparesis. Also, shunt should be delayed in the cystic cavity created by resection of tumor.

Interhemispheric transcallosal intervenous approach to a pineal region tumor

Pineal region tumors represent a formidable challenge to the neurosurgeon. Choosing the right approach is key to optimizing the extent of resection and minimizing surgical morbidity. In this video, the authors show an interhemispheric transcallosal approach to a pineal region tumor in a 15-year-old boy. The advantage of this corridor over posterior approaches is that it provides a nice view of the tumor plane with the venous complex, especially while dissecting tumor from the anterior aspect of the internal cerebral veins on their vertical path. Thus, this approach represents a safe and effective alternative for selected pineal tumors. The video can be found here: https://stream.cadmore.media/r10.3171/2021.4.FOCVID2120.

Resection of a pineal region papillary tumor using robotic exoscope: improved visualization and ergonomics for deep seeded tumor

Surgery for pineal region tumors is technically challenging due to their deep location and close proximity to critical deep venous structures, midbrain, and thalamus. A high-definition video exoscope was recently proposed as an alternative to the operating microscope. The authors illustrate a case of the midline supracerebellar infratentorial approach to resect a pineal region tumor using the Modus V exoscope and demonstrate the improved visualization of critical structures in this deep location. Additionally, the marked improvement in surgeon comfort suggests that this system may have significant advantages over traditional microscope-based surgery for tumors of the pineal region. The video can be found here: https://stream.cadmore.media/r10.3171/2021.4.FOCVID2127.

Pineal Region Hemangioblastoma Resection Through Paramedian Supracerebellar Approach: 2-Dimensional Operative Video

Pineal region tumors remain challenging lesions to safely resect because of their central location.1 Patients frequently present with symptoms associated with hydrocephalus and brainstem compression.2 Local anatomy, primarily the tentorium angle and venous anatomy, plays a central role in the selection of the approach.3 The paramedian supracerebellar approach pioneered by Yaşargil in 19844 allows to access the pineal region through a less steep angle while avoiding the central thickened arachnoid and midline cerebellar and vermian veins.3 Although the author strongly prefers the advantageous three-quarter concord position, this early case was performed in a sitting position, which requires a bubble test to rule out the presence of a persistent foramen ovale. The preoperative pineal differential diagnosis should be exhaustive, including blood and cerebrospinal fluid (CSF) tumor markers in suitable cases. Hemangioblastomas are seldom found or expected in the pineal area, and the surgeon must be alarmed by their typical “cherry nodule” appearance.2,5 Their recognition prior to resection is paramount in avoiding excessive blood loss from tumor entry. Similar to arteriovenous malformations, hemangioblastoma surgical tenets include en bloc resection and preservation of the main draining veins until the last steps of the resection. Von Hippel-Lindau (VHL) syndrome genetic workup is necessary is similar patients, as more than 25% of hemangioblastomas are associated with VHL tumor suppressor gene mutations in chromosome 3.2 The patient consented to the surgery and use of her photography. Image at 2:41 from Ueyama et al, Bridging veins on the tentorial surface of the cerebellum: a microsurgical anatomic study and operative considerations, Neurosurgery, 1998, 43(5),3 used with permission from the Congress of Neurological Surgeons.

Case Report: Metastatic Bronchopulmonary Carcinoid Tumor to the Pineal Region

Intracranial spread of a systemic malignancy is common in advanced staged cancers; however, metastasis specifically to the pineal gland is a relatively rare occurrence. A number of primary lesions have been reported to metastasize to the pineal gland, the most common of which is lung. However, metastasis of a bronchial neuroendocrine tumor to the pineal gland is a seldom-reported entity. Here, we present a 53-year-old female who presented with worsening headaches and drowsiness. MRI brain revealed a heterogeneously enhancing partially cystic mass in the pineal region. The patient had an extensive oncologic history consisting of remote stage IIA invasive breast ductal carcinoma as well as a more recently diagnosed atypical bronchopulmonary neuroendocrine tumor with lymph node metastases. She underwent microsurgical volumetric resection of the large pineal mass and a gross total removal of the tumor was achieved. Histopathology confirmed a metastatic tumor of neuroendocrine origin and the immunohistochemical profile was identical to the primary bronchopulmonary carcinoid tumor. Eight weeks after surgery, she underwent stereotactic radiosurgical treatment to the resection cavity. At 1-year follow-up, the patient remains clinically stable without any new focal neurological deficits and without any evidence of residual or recurrent disease on postoperative MRI. Metastatic neuroendocrine tumors should be considered in the differential diagnosis of pineal region tumors and aggressive surgical resection should be considered in selected patients. Gross total tumor resection may afford excellent local disease control. We discuss the relevant literature on neuroendocrine tumors and current treatment strategies for intracranial metastases of neuroendocrine origin.