A case series of extraneural metastatic glioblastoma at Memorial Sloan Kettering Cancer Center.

BACKGROUND: Extraneural metastasis of glioma is a rare event, often occurring in patients with advanced disease. Genomic alterations associated with extraneural glioma metastasis remain incompletely understood. METHODS: Ten patients at Memorial Sloan Kettering Cancer Center diagnosed with extraneural metastases of glioblastoma (9 patients) and gliosarcoma (1 patient) from 2003 to 2018 were included in our analysis. Patient characteristics, clinical course, and genomic alterations were evaluated. RESULTS: Patient age at diagnosis ranged from 14 to 73, with 7 men and 3 women in this group. The median overall survival from initial diagnosis and from diagnosis of extraneural metastasis was 19.6 months (range 11.2 to 57.5 months) and 5 months (range 1 to 16.1 months), respectively. The most common site of extraneural metastasis was bone, with other sites being lymph nodes, dura, liver, lung, and soft tissues. All patients received surgical resection and radiation, and 9 patients received temozolomide, with subsequent chemotherapy appropriate for individual cases. 1 patient had an Ommaya and then ventriculoperitoneal shunt placed, and 1 patient underwent craniectomy for cerebral edema associated with a brain abscess at the initial site of resection. Genomic analysis of primary tumors and metastatic sites revealed shared and private mutations with a preponderance of tumor suppressor gene alterations, illustrating clonal evolution in extraneural metastases. CONCLUSIONS: Several risk factors emerged for extraneural metastasis of glioblastoma and gliosarcoma, including sarcomatous dedifferentiation, disruption of normal anatomic barriers during surgical resection, and tumor suppressor gene alterations. Next steps with this work include validation of these genomic markers of glioblastoma metastases in larger patient populations and the development of preclinical models. This work will lead to a better understanding of the molecular mechanisms of metastasis to develop targeted treatments for these patients.

Neurologic Complications of Sickle Cell Disease.

PURPOSE OF REVIEW: Sickle cell anemia is a multiorgan disease with acute and chronic complications. Involvement of the central nervous system (CNS) is associated with increased mortality and morbidity. This review highlights the broad spectrum of neurological complications seen in patients with sickle cell disease. RECENT FINDINGS: Increasing recognition of neurological complications has led to improved diagnostic and treatment options throughout the years. Neurologic complications in sickle cell disease include silent cerebral ischemia, ischemic/hemorrhagic stroke, moyamoya syndrome, posterior reversible encephalopathy syndrome, cerebral fat embolism, and cerebral venous sinus thrombosis. Treatment varies depending on the neurological complication. Sickle cell disease is the most common hereditary anemia with increasing global disease burden. Early recognition and treatment is imperative.

Neurological and Neurosurgical Emergencies in Patients with Hematological Disorders.

PURPOSE OF REVIEW: Hematologic diseases are blood disorders which can affect different organs, including the central and peripheral nervous systems. Some of them are associated with increased risk of permanent disability and death. This review highlights a selected group of primary and acquired hematologic disorders that can present as neurologic or neurosurgical emergencies. RECENT FINDINGS: There is an increasing recognition of the broad neurologic presentations of hematologic disorders. Diagnostic criteria continue to be revised as we learn more about these diseases. Treatment options are varied depending on the hematologic syndrome. Clinical judgment is important on a case by case basis given the complexity of these patients. Early recognition of neurologic manifestations of hematologic disorders is important as emergent treatment may be warranted. Clinical signs, appropriate laboratory testing and progression of disease must be taken into consideration to make a timely and definitive diagnosis which will aid in guiding treatment.

Assessing the complications and effectiveness of open carpal tunnel release in a tertiary care centre in a developing country.

INTRODUCTION: Open surgical release for carpal tunnel syndrome is not devoid of complications and its quantitative assessment with the Boston questionnaire in a developing country had not been conducted, where, lack of facilities and surgical technique can influence the outcome. PRESENTATION OF CASE: This was a prospective study in which all cases of carpal tunnel syndrome undergoing open release between June 2007 and June 2012 and who returned for follow up were included. Each patient was requested to fill out the Boston questionnaire twice both pre and post op at 3 months. All complications were recorded as well as bio-data of patients and co morbidities. Follow up was at 2 weeks and at 3 months. Those reporting complications at 3 months were further followed up until 6 months. 373 patients were included in the study. Twenty four patients developed complications. Of these, 12 experienced pain resulting from reflex sympathetic dystrophy. Three patients developed wound dehiscence, 2 cases acquired infections, 4 patients developed immediate post-operative haemorrhage and in 3 patients there was late recurrence of median nerve compression. The symptom severity score pre-operatively was 3.30 (±0.60) and it improved to 1.65 (±0.75) post-operatively indicating a significant change (p<0.0001). The preoperative functional status score was 2.58 (±0.75) and post-op it became 1.60 (±0.80) again implying a good improvement with an effect size of 1.3. DISCUSSION: All of the complications produced were well managed. The complication incidence was low. The open release procedure produced good improvement in hand function and in decreasing the symptom severity. CONCLUSION: Conducting open release for carpal tunnel syndrome in a tertiary referral centre in a developing country offers a good outcome.