Gliomatosis Cerebri: A Unique Presentation with Accompanying Clinical Nuance.

Gliomatosis cerebri (GC) has classically been considered a rare malignancy with a poor prognosis and is developmentally unique from solid tumors. More recently, GC has become better understood as a phenotype along the spectrum of gliomas and, most importantly, not mutually exclusive from the more common presentation of a tumor mass. The following case report illustrates not only the implications of the ontogeny of gliomas in clinical practice but also the successes that can accompany the early recognition of such a disease. Here, we report the presentation of a solid temporal lobe glioma, which, on presentation, was disseminating along well-defined mesolimbic white matter tracts. Once properly diagnosed and managed, the patient remarkably proceeded to achieve an impressive outcome given the extent of her pathology.

Recurrent Caterpillar-like Arachnoid Cysts Following Initial Resection: A Unique Presentation of a Disorder Where the Limits of Modern Medicine Are Reached.

Of the many emergent neurological cases presenting to the emergency department (ED) and operating room (OR) for resection, arachnoid cysts are amongst one of the rarer pathologies. The proper resection of arachnoid cysts has substantially decreased the risk of recurrence. Current medicinal and surgical approaches have been refined over the years and prove to be successful for many patients. Where current practices begin to fail is during the treatment of complex and rare cases, such as the one presented in this manuscript. The commonly accepted surgical practices that were utilized to aid in the management of our patient, who initially presented with a simple arachnoid cyst, unexpectedly resulted in the further development of additional arachnoid cysts, a very rare occurrence, and a complication that should be discussed amongst all specialists in the hope of identifying more focused, novel, and less-invasive approaches to cyst removal and recurrence prevention.

Anti-coagulation Drug Warfarin Contributes to Severe Adverse Outcomes in Prolonged Unsupervised Use: A Double-edged Sword.

Anti-coagulation medications are widely used in clinical practice, especially in the United States as cardiac-related emergencies are on the rise. An overarching caveat in using anti-coagulation drugs as a long-term treatment regimen, such as warfarin, is that patients are closely monitored by their primary care provider. Routine monthly laboratory examinations are strictly required to assess the international normalized ratio (INR) which measures prothrombin time (PT), providing insight into how long it takes for blood to clot. A 58-year-old Caucasian male presented with non-traumatic acute quadriplegia with cord compression starting at the 3rd cervical (C3) level and continuing throughout the entire spinal cord. Radiological studies revealed severe cervical stenosis from C3 to C7 resulting from a posterior spinal mass, thoracic and lumbar spine studies revealed a compressive posterior acute epidural hematoma (EDH) compressing the thoracic spinal cord from T6 to T10 with signal change, as well as a L4-S1 posterior acute epidural hematoma compressive of the cauda equina. Upon detailed review of our patient's history it became evident that our patient had been on a prolonged and unsupervised warfarin (Coumadin) regimen for congestive heart failure without primary care follow-up or routine laboratory testing. Our patient was a prime surgical candidate for an immediate two-stage decompression, hematoma evacuation and instrumentated fusion surgery. Post-operatively, our patient regained leg strength to 2/5 and arm strength to 4+/5.

Trauma-induced Acute Epidural Hematoma: The Rising Sun in a Progressively Lethargic Man.

A young adult, 18 years of age, presented to the emergency department with severe traumatic brain injury (TBI) resulting from a bicycle versus vehicle head-on collision. The patient initially presented in a promising condition but quickly deteriorated into a state of unconsciousness with no meaningful responses to stimuli or coordinated voluntary movement. Stat computed tomography (CT) revealed a large, right-sided, acute epidural hematoma (EDH) with mass-effect and a severe midline shift indicative of immediate surgery. This case highlights the importance of closely monitoring traumatic brain injury patients regardless of initial presentation and neurological exam results, as the patient's condition may drastically and rapidly change without much warning. Additionally, it is key to utilize regular radiological studies on these patients, to detect any neurological changes as close to onset as possible. Lastly, it is imperative that neurosurgeons closely monitor the patients/ state of consciousness as a rapid decline serves as a key diagnostic indicator of the need for immediate surgery.

Concomittant fibrous dysplasia with aneurysmal bone cyst formation within the skull, humerus and rib.

Polyostotic fibrous dysplasia (FD) is a rare pathology characterized by the abnormal and gradual replacement of normal bone (calcium hydroxylapatite of osteoid matrix) with fibrous connective tissue. Aneurysmal bone cyst (ABC) is a tumor-like benign lesion with blood-filled cavities that can affect virtually any bone in the body. We report on a 20-year-old male presenting with an extremely rare pathology of FD with ABC formation of the skull, fourth rib and humerus. Our case report represents a novel literary addition to rare FD with ABC pathologies. Optimal diagnosis of this rare pathology can be achieved by a full body evaluation for clinical and radiographic FD with or without ABC, and optimal treatment for this rare pathology is the maximum surgical excision of the tumor and/or soft tissue.

Management Strategies and Outcomes for VHL-related Craniospinal Hemangioblastomas.

Hemangioblastomas are rare and benign tumors accounting for less than 2% of all central nervous system (CNS) tumors. The vast majority of hemangioblastomas occur sporadically, whereas a small number of cases, especially in younger patients, are associated with Von Hippel-Lindau (VHL) syndrome. It is thought that loss of tumor suppressor function of the VHL gene results in stabilization of hypoxia-inducible factor alpha with downstream activation of cellular proliferative and angiogenic genes that promote tumorigenesis. VHL-related hemangioblastomas predominantly occur in the cerebellum and spine. Lesions are often diagnosed on contrast-enhanced craniospinal MRIs, and the diagnosis of VHL occurs through assessment for germline VHL mutations. Surgical resection remains the primary treatment modality for symptomatic or worrisome lesions, with excellent local control rates and neurological outcomes. Stereotactic radiotherapy can be employed in patients who are deemed high risk for surgery, have multiple lesions, or have non-resectable lesions. Given the tendency for development of either new or multiple lesions, close radiographic surveillance is often recommended for asymptomatic lesions.

Improving C1-C2 Complex Fusion Rates: An Alternate Approach.

The surgical repair of atlantoaxial instabilities (AAI) presents complex and unique challenges, originating from abnormalities and/or trauma within the junction regions of the C1-C2 atlas-axis, to surgeons. When this region is destabilized, surgical fusion becomes of key importance in order to prevent spinal cord injury. Several techniques can be utilized to provide for the adequate fusion of the atlantoaxial construct. Nevertheless, many individuals have less than ideal rates of fusion, below 35%-40%, which also involves the C2 nerve root being sacrificed. This suboptimal and unavoidable iatrogenic complication results in the elevated probability of complications typically composed of vertebral artery injury. This review is a retrospective analysis of 87 patients from Cedars Sinai Medical Center in Los Angeles, California, who had the C1-C2 surgical fusion procedure performed within the time frame from 2001 to 2008, with a mean follow-up period of three years. These patients had presented with typical AAI symptoms of fatigability, limited mobility, and clumsiness. Diagnosis of C1-C2 instability was documented via radiographic studies, typically utilizing computed tomography (CT) scans or x-rays. All patients had bilateral C1 lateral masses and C2 pedicle screws. In addition, the C1-C2 joint was accessed by retracting the C2 nerve root superiorly and exposing the joint by utilizing a high-speed burr. The cavity that is developed within the joint is packed with local autologous bone from the cephalad resection of the C2 laminae. Fusion of the C1-C2 joint was achieved in all patients and a final follow-up was conducted approximately three years postoperative. Of the 87 patients, two presented with occipital headaches resulting from the C1 screws impinging on the C2 nerve root. The issue was rectified by removing instrumentation in both patients after documenting complete fusion via radiographic studies, with complete resolution of symptoms. No vertebral artery or spinal cord injuries were reported as a result of the minor complication. Overall, we aim to describe a safe and reliable alternative technique to fuse C1-C2 instability by focusing on intra-articular arthrodesis complementing instrumentation fixation. This methodology is advantageous from a biomechanical standpoint secondary to axial loading, as well as the large surface area available for arthrodesis. Additionally, this technique does not involve the resection of the C2 nerve root, resulting in low risk for vertebral artery or spinal cord injury.

Brief Report: Concurrent cervical giant perimedullary arteriovenous fistula, aneurysm on a feeding artery of fistula and unilateral congenital carotid aplasia.

Giant perimedullary arteriovenous fistulae (GPAVFs) are extremely rare, particularly cervical GPAVFs whose incidence has not been tabulated. The occurrence of aneurysm on an artery feeding a GPAVF has previously not been described. Internal carotid artery aplasia is also very rare (0.01%). The concurrence of these disorders has previously not been recorded. We report a case of a 5-year-old female with increasing headaches, who was found to have intraventricular hemorrhage and above anomalies. Coil embolization of GPAVF and the adjacent aneurysm was attempted. Treatment was complicated by stroke and death. Embryological and anatomical factors underlying these anomalies as well as, management options are discussed.

High incidence of morbidity following resection of metastatic pheochromocytoma in the spine.

Pheochromocytomas of the spine are uncommon and require careful preoperative planning. The authors retrospectively reviewed the charts of 5 patients with metastatic spinal pheochromocytoma who had undergone surgical treatment over the past 10 years at their medical center. They reviewed patient age, history of pheochromocytoma resection, extent and location of metastases, history of alpha blockage, surgical level, surgical procedure, postoperative complications, tumor recurrence, and survival. Metastases involved the cervical (1 patient), thoracic (3 patients), and lumbar (2 patients) levels. Preoperative treatment included primary pheochromocytoma resection, chemotherapy, alpha blockade, embolization, and radiation. Three patients had tumor recurrence, and 2 underwent 2-stage reoperations for tumor extension. Hemodynamic complications were common: 2 patients developed pulseless electrical activity arrest within 4 months after surgery, 1 patient had profound postoperative tachycardia with fever and an elevated creatine kinase level, and 1 patient experienced transient postoperative hypotension and paraplegia. One patient died of complications related to disseminated cerebral and spinal disease. With careful preoperative and surgical management, patients with symptomatic metastatic spinal pheochromocytoma can benefit from aggressive surgical treatment. Postoperative cardiovascular complications are common even months after surgery, and patients should be closely monitored long term.

Surgical management of primary tumors of the cervical spine: surgical considerations and avoidance of complications.

OBJECTIVE: Primary cervical spinal tumors are rare tumors of the spine and are associated with significant morbidity and mortality. Such tumors include multiple myeloma, chordomas, giant cell tumors, hemangiomas, osteosarcomas, chondrosarcomas, synovial sarcomas, aneurysmal bone cysts (ABC), hemangiomas, eosinophilic granulomas, osteoid osteomas, and osteoblastomas. We review the surgical decision-making process and identify critical key steps for surgical complication avoidance. We also present case illustrations demonstrating such pathological diagnoses and surgical treatments performed. METHODS: We retrospectively review the literature regarding the most common primary cervical spinal tumors that have undergone surgical resection with or without adjuvant treatment. RESULTS: En bloc resection of primary cervical tumors resulted in significantly increased progression-free survival and overall survival. From the limited data, adjuvant treatment with proton-beam therapy for chordomas has potential benefit. Neo-adjuvant chemotherapy for Ewing's sarcoma and osteogenic sarcoma has shown some promise, with en bloc resection demonstrating stronger benefit for osteogenic sarcoma. DISCUSSION: En bloc resection for primary spinal tumors has proven to be the standard of care in spinal oncology. Adjuvant and neo-adjuvant treatments such as chemotherapy and radiotherapy variants (conventional, proton beam, cyberknife) need to be studied further in most primary tumor types to become standard of care. Chordoma management is more widely studied with en bloc resection and adjuvant proton-beam therapy demonstrating improved progression-free survival and overall survival. Surgical management and adjuvant treatment strategies are case dependent, depending on tumor histology, patient neurological examination, prior surgeries at that level, and prior adjuvant treatment.

Palliative strategies for the management of primary and metastatic spinal tumors.

BACKGROUND: Technological advances during the last few decades have improved the success rate of surgery for the treatment of malignant spinal tumors. Nonetheless, many patients present with widespread tumor burden and minimal life expectancy, which excludes them from being surgical candidates. For these patients, palliative management is recommended. METHODS: The authors reviewed prospective and retrospective clinical studies as well as case series regarding palliative treatments for primary and metastatic spinal tumors. RESULTS: Analgesics, ranging from nonopioids to strong opioids, may be used depending on the degree of pain. Steroids may also improve pain relief, although they are associated with a number of adverse events. Vertebroplasty and kyphoplasty are conservative treatments with high rates of pain relief and vertebral body stabilization. Radiotherapy is the gold standard for palliative management, with approximately 60% of patients experiencing a decrease in tumor-related spinal pain and up to 35% experiencing complete relief. Stereotactic radiosurgery delivers high doses of radiation to patients to provide pain relief while also sparing delicate anatomical structures. CONCLUSION: Palliative management of spinal tumors is diverse. Analgesics may be used in conjunction with radiotherapy and/or kyphoplasty or vertebroplasty to offer pain relief.

Surgical management of primary and metastatic spinal tumors.

BACKGROUND: The axial skeleton is a common site for primary tumors and metastatic disease, with metastatic disease being much more common. Primary and metastatic spinal tumors have a diverse range of aggressiveness, ranging from benign lesions to highly infiltrative malignant tumors. METHODS: The authors reviewed the results of articles describing the treatment and outcomes of patients with metastatic disease or primary tumors of the spinal column. RESULTS: En bloc resection is the mainstay of treatment for malignant primary tumors of the spinal column. Intralesional resection is generally appropriate for benign primary tumors. Low-quality evidence supports the use of chemotherapy in select primary tumors; however, radiation therapy is often used for incompletely resected or unresectable lesions. Surgical considerations for the treatment of metastatic disease are more nuanced and require that the health care professional consider patient performance status and the pathology of the primary tumor. CONCLUSIONS: The treatment of metastatic and primary tumors of the spinal column requires a multidisciplinary approach in order to offer patients the best opportunity for long-term survival.