Patients diagnosed with neurofibromatosis type 2 (NF2) are extremely likely to develop meningiomas, in addition to vestibular schwannomas. Meningiomas are a common primary brain tumor; many NF2 patients suffer from multiple meningiomas. In NF2, patients have mutations in the NF2 gene, specifically with loss of function in a tumor-suppressor protein that has a number of synonymous names, including: Merlin, Neurofibromin 2, and schwannomin. Merlin is a 70 kDa protein that has 10 different isoforms. The Hippo Tumor Suppressor pathway is regulated upstream by Merlin. This pathway is critical in regulating cell proliferation and apoptosis, characteristics that are important for tumor progression. Mutations of the NF2 gene are strongly associated with NF2 diagnosis, leading to benign proliferative conditions such as vestibular schwannomas and meningiomas. Unfortunately, even though these tumors are benign, they are associated with significant morbidity and the potential for early mortality. In this review, we aim to encompass meningiomas and vestibular schwannomas as they pertain to NF2 by assessing molecular genetics, common tumor types, and tumor pathogenesis.
Study Design:Focused literature review.Objective:The objective of this article was to help identify potential risk factors as well as strategies to help prevent surgical site infections (SSIs) in spine surgery.Methods:An article search was performed using PubMed, EMBASE, and the Cochrane database of systematic reviews using the terms “surgery” OR “surgical” AND “spine” OR “spinal” AND “infection”. Systematic review articles, meta-analyses, and clinical trials with more than 100 patients were reviewed.Results:Both patient and perioperative factors contribute to the development of SSIs. Patient factors such as smoking, obesity, diabetes, Methicillin-resistant Staphylococcus aureus (MRSA) colonization, and malnutrition are all modifiable risk factors that can lead to SSIs. Procedural steps, including preoperative MRSA screening and treatment for colonization, preoperative antibiotics, skin preparation, minimizing operative time, antibiotic or betadine irrigation, avoiding personnel turnover, and postoperative wound care have also been shown to decrease infection rates.Conclusion:There are several measures a spine practitioner may be able to take in the preoperative, intraoperative, and postoperative settings. Protocols to counsel patients regarding modification of preexisting risk factors and ensure adequate antimicrobial therapy in the perioperative period may be developed to reduce SSIs in spine surgery.
BACKGROUND AND IMPORTANCE: Aggressive pituitary adenomas (APAs) are pituitary tumors that are refractory to standard treatments and carry a poor prognosis. Current treatment guidelines are not standardized but combine surgical resection, radiation therapy, and chemotherapy. Temozolomide is the only chemotherapeutic agent with documented effectiveness and is recommended for APA in European Society of Endocrinology clinical guidelines. CLINICAL PRESENTATION: A 57-year-old man presented with visual deterioration and bitemporal hemianopsia. MRI of the brain demonstrated a sellar mass suspected to be pituitary macroadenoma with displacement of the stalk and optic nerve impingement. The patient underwent stereotactic endoscopic transsphenoidal resection of the mass. Postoperative MRI demonstrated gross total resection. Pathology revealed a sparsely granulated corticotroph adenoma with malignant transformation. Immunohistochemistry showed loss of expression of MLH1 and PMS2 in the tumor cells. Proton therapy was recommended given an elevated Ki67 index and p53 positivity. Before radiotherapy, there was no radiographic evidence of residual tumor. Temozolomide therapy was initiated after surveillance MRI showed recurrence at 16 months postoperatively. However, MRI demonstrated marked progression after 3 cycles. Next-generation sequencing using the MSK-IMPACT platform identified somatic mutations in MLH1 Y548lfs*9 and TP53 R337C. Immunotherapy with ipilimumab/nivolumab was initiated, and MRI demonstrated no residual tumor burden 34 months postoperatively. CONCLUSION: APA is a tumor with frequent recurrence and a short median expected length of survival. Here, we demonstrate the utility of immunotherapy in a single case report of APA, with complete resolution of recurrent APA and improved survival compared with life expectancy.
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