Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla and the sympathetic/parasympathetic neural ganglia, respectively. The heterogeneity in its etiology makes PPGL diagnosis and treatment very complex. The aim of this article was to provide practical clinical guidelines for the diagnosis and treatment of PPGLs from a multidisciplinary perspective, with the involvement of the Spanish Societies of Endocrinology and Nutrition (SEEN), Medical Oncology (SEOM), Medical Radiology (SERAM), Nuclear Medicine and Molecular Imaging (SEMNIM), Otorhinolaryngology (SEORL), Pathology (SEAP), Radiation Oncology (SEOR), Surgery (AEC) and the Spanish National Cancer Research Center (CNIO). We will review the following topics: epidemiology; anatomy, pathology and molecular pathways; clinical presentation; hereditary predisposition syndromes and genetic counseling and testing; diagnostic procedures, including biochemical testing and imaging studies; treatment including catecholamine blockade, surgery, radiotherapy and radiometabolic therapy, systemic therapy, local ablative therapy and supportive care. Finally, we will provide follow-up recommendations.
Meningoencephalic herniation into the middle ear is a rare and potentially life-threatening condition that may require prompt surgical intervention. Preoperative diagnosis is based on a high index of suspicion. Sometimes, however, meningoencephalic herniation is discovered during surgery. High-resolution computed tomography and magnetic resonance imaging should be performed to confirm the diagnosis and to evaluate the extension of the herniated tissue. This article discusses the diagnostic approach, management strategy, and surgical technique used in 27 patients with meningoencephalic herniation. In an attempt to avoid infective complications, the authors used the middle cranial approach in patients with large herniations.
Collectively, this study unveiled a putative signaling axis of HIF-1α/miRNA-210/ISCU in a subset of HNPGLs that could have an impact on SDHB protein stability by a mechanism independent of SDH mutations, thus providing a foundation to better understand the functional interplay between HIF, miR-210, and mitochondria and its relevance in the pathogenesis of HNPGLs.
Subtotal petrosectomy in cochlear implantation permits obtaining a cavity isolated from the external environment, and when needed, it improves the access and visibility during the surgical procedure. Subtotal petrosectomy is a safe technique, with a low rate of complications.
The present cases demonstrate that cochlear implantation can be successful after a translabyrinthine approach for VS, regardless of the tumor size, the kind of patient (NF2, unilateral VS), and the type of implant. The results also are suggestive that cochlear implantation is more successful if done concurrent with surgery for tumor removal and before hearing is completely lost.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.