scholarly journals The future: diagnostic and imaging advances in MEN1 therapeutic approaches and management strategies

2017 ◽  
Vol 24 (10) ◽  
pp. T209-T225 ◽  
Author(s):  
Jerena Manoharan ◽  
Max B Albers ◽  
Detlef K Bartsch
Keyword(s):  
Multiple Endocrine Neoplasia Type ◽  
Management Strategies ◽  
Diagnostic Tools ◽  
Future Perspectives ◽  
Therapeutic Approaches ◽  
Increase Life Expectancy ◽  
Life Threatening ◽  
Clinical Expert

Prospective randomized data are lacking, but current clinical expert guidelines recommend annual screening examinations, including laboratory assessments and various imaging modalities (e.g. CT, MRI, scintigraphy and EUS) for patients with multiple endocrine neoplasia type 1 (MEN1). Routine screening is proposed to detect and localize neuroendocrine manifestations as early as possible. The goal is timely intervention to improve quality of life and to increase life expectancy by preventing the development of life-threatening hormonal syndromes and/or metastatic disease. In recent years, some studies compared different and new imaging methods regarding their sensitivity and utility in MEN1 patients. This present article reviews the proposed diagnostic tools for MEN1 screening as well as potential future perspectives.

scholarly journals The future: surgical advances in MEN1 therapeutic approaches and management strategies

2017 ◽  
Vol 24 (10) ◽  
pp. T243-T260 ◽  
Author(s):  
S M Sadowski ◽  
G Cadiot ◽  
E Dansin ◽  
P Goudet ◽  
F Triponez
Keyword(s):  
Multiple Endocrine Neoplasia ◽  
Autosomal Dominant ◽  
Multiple Endocrine Neoplasia Type ◽  
Management Strategies ◽  
Hereditary Disease ◽  
Therapeutic Approaches ◽  
Autosomal Dominant Disorder ◽  
Appropriate Therapy

Multiple endocrine neoplasia type 1 (MEN1) is a hereditary autosomal dominant disorder associated with numerous neuroendocrine tumors (NETs). Recent advances in the management of MEN1 have led to a decrease in mortality due to excess hormones; however, they have also led to an increase in mortality from malignancy, particularly NETs. The main challenges are to localize these tumors, to select those that need therapy because of the risk of aggressive behavior and to select the appropriate therapy associated with minimal morbidity. This must be applied to a hereditary disease with a high risk of recurrence. The overall aim of management in MEN1 is to ensure that the patient remains disease- and symptom-free for as long as possible and maintains a good quality of life. Herein, we review the changes that occurred in the last 20 years in the surgical management of MEN1-associated functional and non-functional pancreatico-duodenal NETs and thymic and bronchial NETs.

scholarly journals Surgery Combined With Embolization in the Treatment of Plexiform Neurofibroma: A Case Report and Literature Review

2021 ◽  
Vol 6 (4) ◽  
pp. 01-04
Author(s):  
Reinaldo Filho
Keyword(s):  
Plexiform Neurofibroma ◽  
Arterial Embolization ◽  
Neurofibromatosis Type ◽  
Self Esteem ◽  
Life Threatening ◽  
Feeding Vessels ◽  
The Right ◽  
Polyvinyl Alcohol Particles

Neurofibromatosis type 1 (NF1) is an autosomal dominant multisystem genodermatosis resulting from a mutation on chromosome 17q11.2, characterized by diverse clinical expression with involvement of the skin, nervous system, bones, eyes and other systems. Neurofibromas are tumors located along nerve bundles; when they become large, they are called plexiform neurofibromas. Complications of neurofibromas are rare, but include malignant transformation and potentially life-threatening hemorrhages. A comprehensive perioperative management, including arterial embolization of feeding vessels, is required in order to perform a safe surgical procedure and to reduce potential surgical complications. We report a case of a large plexiform neurofibroma on the right flank, treated in a two-stage approach: first, embolization with polyvinyl alcohol particles of the inferior epigastric, lumbar and superior gluteal arteries on the right and, second, 24 hours after embolization, exeresis of the lesion, which weighed 2.5 kg. Two years after surgery, the patient was asymptomatic, without tumor recurrence and with an important improvement in self-esteem and quality of life.

scholarly journals Health-related quality of life in patients with Multiple Endocrine Neoplasia type 1

2020 ◽  
Author(s):  
Rachel S van Leeuwaarde ◽  
Carolina R. C. Pieterman ◽  
Anne M. May ◽  
Olaf M. Dekkers ◽  
Anouk N.A. van der Horst-Schrivers ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Multiple Endocrine Neoplasia ◽  
Multiple Endocrine Neoplasia Type ◽  
Health Related Quality ◽  
Endocrine Neoplasia ◽  
Endocrine Neoplasia Type ◽  
Related Quality ◽  
Health Related

scholarly journals Multiple Endocrine Neoplasia Type 1: Latest Insights

2020 ◽  
Author(s):  
Maria Luisa Brandi ◽  
Sunita K Agarwal ◽  
Nancy D Perrier ◽  
Kate E Lines ◽  
Gerlof D Valk ◽  
...  
Keyword(s):  
Multiple Endocrine Neoplasia ◽  
Multiple Endocrine Neoplasia Type ◽  
Genetic Diagnosis ◽  
Diagnostic Tools ◽  
Surgical Approaches ◽  
Endocrine Tumors ◽  
Endocrine Neoplasia ◽  
Endocrine Neoplasia Type ◽  
Basic Biology

Abstract Multiple Endocrine Neoplasia Type 1 (MEN1), a rare tumor syndrome that is inherited in an autosomal dominant pattern, is continuing to raise great interest for endocrinology, gastroenterology, surgery, radiology, genetics and molecular biology specialists. There have been two major clinical practice guidance papers that were published in the past two decades, with the most recent publication 8 years ago. Since then, several new insights on the basic biology and clinical features of MEN1 have appeared in the literature and those data are discussed in this review. The genetic and molecular interactions of the MEN1 encoded protein menin with transcription factors and chromatin modifying proteins in cell signaling pathways mediated by TGF-β/BMP, few nuclear receptors, Wnt/β-catenin and Hedgehog (Hh), and preclinical studies in mouse models have facilitated the understanding of the pathogenesis of MEN1-associated tumors and potential pharmacological interventions. The advancements in genetic diagnosis have offered a chance to recognize MEN1 related conditions in germline MEN1 mutation negative patients. There is a rapidly accumulating knowledge about clinical presentation in children, adolescents and pregnancy that is translatable into the management of these very fragile patients. The discoveries about the genetic and molecular signatures of sporadic neuro-endocrine tumors support the development of clinical trials with novel targeted therapies, along with advancements in diagnostic tools and surgical approaches. Finally, quality of life studies in patients affected by MEN1 and related conditions represent an effort necessary to develop a pharmacoeconomic interpretation of the problem. Because advances are being made both broadly and in focused areas, this timely review presents and discusses those studies collectively.

scholarly journals Perspective on the Therapeutics of Anti-Snake Venom

Molecules ◽  
2019 ◽  
Vol 24 (18) ◽  
pp. 3276 ◽  
Author(s):  
Isabel Gómez-Betancur ◽  
Vedanjali Gogineni ◽  
Andrea Salazar-Ospina ◽  
Francisco León
Keyword(s):  
Ethnic Groups ◽  
Snake Venom ◽  
Tropical Disease ◽  
Neglected Tropical Disease ◽  
Future Perspectives ◽  
Therapeutic Approaches ◽  
Natural Sources ◽  
Snake Envenomation ◽  
The World ◽  
Life Threatening

Snakebite envenomation is a life-threatening disease that was recently re-included as a neglected tropical disease (NTD), affecting millions of people in tropical and subtropical areas of the world. Improvement in the therapeutic approaches to envenomation is required to palliate the morbidity and mortality effects of this NTD. The specific therapeutic treatment for this NTD uses snake antivenom immunoglobulins. Unfortunately, access to these vital drugs is limited, principally due to their cost. Different ethnic groups in the affected regions have achieved notable success in treatment for centuries using natural sources, especially plants, to mitigate the effects of snake envenomation. The ethnopharmacological approach is essential to identify the potential metabolites or derivatives needed to treat this important NTD. Here, the authors describe specific therapeutic snakebite envenomation treatments and conduct a review on different strategies to identify the potential agents that can mitigate the effects of the venoms. The study also covers an increased number of literature reports on the ability of natural sources, particularly plants, to treat snakebites, along with their mechanisms, drawbacks and future perspectives.

scholarly journals Recent Advances and Future Perspectives in the Development of Therapeutic Approaches for Neurodegenerative Diseases

2020 ◽  
Vol 10 (9) ◽  
pp. 633
Author(s):  
Melissa Bowerman
Keyword(s):  
Quality Of Life ◽  
Alzheimer’S Disease ◽  
Neurodegenerative Diseases ◽  
Huntington's Disease ◽  
Neuronal Cells ◽  
Future Perspectives ◽  
Therapeutic Approaches ◽  
Recent Advances ◽  
The Brain

Neurodegenerative diseases such as Alzheimer’s disease (AD), Parkinson’s disease (PD) and Huntington’s disease (HD), severely impact the function of neuronal cells in the brain and have devastating consequences on the quality of life of patients and their families [...]

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