Neuroendocrine tumour of the ampulla of Vater: a rare neoplasm at an atypical site

2020 ◽  
pp. 1-10
Author(s):  
Abrar Zahid ◽  
Danish Ali ◽  
Muhammad Zubair ◽  
Irfan Ahmed ◽  
Tauseef Fatima ◽  
...  
Keyword(s):  
Weight Loss ◽  
Immunohistochemical Staining ◽  
Poor Prognosis ◽  
Carcinoid Syndrome ◽  
Neuroendocrine Tumour ◽  
Carcinoid Tumour ◽  
Treatment Options ◽  
Ampulla Of Vater ◽  
Neuroendocrine Neoplasms ◽  
Late Event

Abstract The periampullary neuroendocrine tumour is an infrequently occurring tumour. Its prevalence among gastrointestinal neuroendocrine neoplasms is less than 0.3%, and less than 2% out of periampullary tumours. These neoplasms have relatively poor prognosis. Jaundice and pain in the abdomen are the early and most commonly occurring symptoms with weight loss being a late event. The carcinoid syndrome presents infrequently in periampullary neuroendocrine tumour and happens only if hepatic metastasis occurs. In this scenario, histopathology plays a paramount role in the diagnosis. Specific immunohistochemical staining is used for diagnosis while the treatment options are local excision, endoscopic excision and pancreaticoduodenectomy. Here is a case report of a 42-year-old patient who presented with complaint of obstructive jaundice for one month. Periampullary carcinoid tumour was diagnosed on biopsy, and she underwent Pancreaticoduodenectomy as treatment. Continuou...  

scholarly journals P55 Carcinoid syndrome on etanercept and subsequent treatment with secukinumab in a patient with psoriatic arthritis

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Ali S. F Sheikh ◽  
Sagar G Srivastva ◽  
Fiona Wood
Keyword(s):  
Psoriatic Arthritis ◽  
Carcinoid Syndrome ◽  
Neuroendocrine Tumour ◽  
Early Recognition ◽  
Carcinoid Tumour ◽  
Safety Data ◽  
Biologic Agents ◽  
Tnf Alpha ◽  
First Line ◽  
Alpha Blockers

Abstract Background Psoriatic arthritis requires early recognition and treatment for prevention of disease progression. Conventional disease modifying drugs are first-line agents followed by biologic DMARDs for patients with active disease. TNF-alpha blockers are first line biologic agents in the UK. Th17 inhibitors are used since the elucidation of Th17 pathway. Safety and efficacy profiles of biologic agents inhibiting the Th17 pathway, including secukinumab (IL-17A) and ustekinumab (IL-12/23p40) have been studied. Methods We report a case of carcinoid syndrome in a lady on etanercept for psoriatic arthritis, carcinoid as a potential TNF alpha side effect. We also report safety of Th17 (secukinumab) inhibitors in the patient to date (>1 year). Results A female with a history of acne rosacea, was diagnosed with psoriatic arthritis in 2000 age 32. Initial sulphasalazine failed, then received methotrexate until 2011, when her arthritis flared. She was commenced on etanercept which proved effective. After 4 years of etanercept and methotrexate, her liver profile became deranged. Investigations off treatment included ultrasound abdomen demonstrating a liver mass, which resembled focal nodular hyperplasia on magnetic resonance imaging. Further screening revealed high urinary 5HIAA (527 umol/24h) and raised chromogranin A & B levels (1574 pmol/L and 373 pmol/L respectively). She had no symptoms suggestive of carcinoid, although facial flushing could have been camouflaged by her rosacea. Octreotide scan was positive, CT enterogram showed a distal ileal neuroendocrine tumour with adjacent lymphadenopathy. She underwent right hemihepatectomy and hemicolectomy. Her liver profile deteriorated again on methotrexate, leflunomide was ineffective. In November 2017 she was started on ustekinumab, which was ineffective and was withdrawn after 8 months. There was no alteration in carcinoid blood markers and no CT changes. In July 2018, she was commenced on secukinumab, which has allowed reduction in steroids. The patient is aware of lack of safety data in her circumstances. Regular surveillance has shown no recurrence of carcinoid with serial negative 5HIAA and chromogranin levels. There are no progressive CT changes at 1 year. Conclusion This is a case of carcinoid tumour occurring on TNF-alpha blockers and may represent a rare complication. Screening biomarkers including 5HIAA and chromogranin levels can be useful if disease is suspected. We could not find other similar case reports to guide further management. Within time limited data available - ustekinumab had no effect on the carcinoid. Th17 inhibitors can be safe options for treating psoriatic arthritis and psoriasis with highly sustained efficacy and favourable safety profile seen in large clinical trials. In this case, after > one year of secukinumab treatment - there is no adverse effect on carcinoid syndrome. Disclosures A.S.F. Sheikh None. S.G. Srivastva None. F. Wood None.

scholarly journals Carcinoid Syndrome and Hyperinsulinemic Hypoglycemia Associated with Neuroendocrine Neoplasms: A Critical Review on Clinical and Pharmacological Management

2021 ◽  
Vol 14 (6) ◽  
pp. 539
Author(s):  
Francesca Spada ◽  
Roberta E. Rossi ◽  
Elda Kara ◽  
Alice Laffi ◽  
Sara Massironi ◽  
...  
Keyword(s):  
Carcinoid Syndrome ◽  
Treatment Options ◽  
Therapeutic Strategies ◽  
Hyperinsulinemic Hypoglycemia ◽  
Neuroendocrine Neoplasms ◽  
Pharmacological Management ◽  
Serotonin Secretion ◽  
Clinical Syndromes ◽  
Pancreatic Insulinoma

The carcinoid syndrome (CS) and hyperinsulinemic hypoglycemia (HH) represent two of the most common clinical syndromes associated with neuroendocrine neoplasms (NENs). The former is mainly related to the serotonin secretion by a small bowel NEN, whereas the latter depends on an insulin hypersecretion by a pancreatic insulinoma. Both syndromes/conditions can affect prognosis and quality of life of patients with NENs. They are often diagnosed late when patients become strongly symptomatic. Therefore, their early detection and management are a critical step in the clinical management of NEN patients. A dedicated and experienced multidisciplinary team with appropriate therapeutic strategies is needed and should be encouraged to optimize clinical outcomes. This review aims to critically analyze clinical features, evidence and treatment options of CS and HH and therefore to improve their management.

scholarly journals Bone Metastases in Neuroendocrine Neoplasms: From Pathogenesis to Clinical Management

Cancers ◽  
2019 ◽  
Vol 11 (9) ◽  
pp. 1332 ◽  
Author(s):  
Barbara Altieri ◽  
Carla Di Dato ◽  
Chiara Martini ◽  
Concetta Sciammarella ◽  
Antonella Di Sarno ◽  
...  
Keyword(s):  
Bone Metastases ◽  
Molecular Mechanisms ◽  
Current Knowledge ◽  
Imaging Techniques ◽  
Case Reports ◽  
Treatment Options ◽  
Neuroendocrine Neoplasms ◽  
Positron Emission ◽  
Late Event ◽  
Skeletal Complications

Bone represents a common site of metastases for several solid tumors. However, the ability of neuroendocrine neoplasms (NENs) to localize to bone has always been considered a rare and late event. Thanks to the improvement of therapeutic options, which results in longer survival, and of imaging techniques, particularly after the introduction of positron emission tomography (PET) with gallium peptides, the diagnosis of bone metastases (BMs) in NENs is increasing. The onset of BMs can be associated with severe skeletal complications that impair the patient’s quality of life. Moreover, BMs negatively affect the prognosis of NEN patients, bringing out the lack of curative treatment options for advanced NENs. The current knowledge on BMs in gastro-entero-pancreatic (GEP) and bronchopulmonary (BP) NENs is still scant and is derived from a few retrospective studies and case reports. This review aims to perform a critical analysis of the evidence regarding the role of BMs in GEP- and BP-NENs, focusing on the molecular mechanisms underlining the development of BMs, as well as clinical presentation, diagnosis, and treatment of BMs, in an attempt to provide suggestions that can be used in clinical practice.

scholarly journals Glioblastoma and MiRNAs

Cancers ◽  
2021 ◽  
Vol 13 (7) ◽  
pp. 1581
Author(s):  
Swalih P. Ahmed ◽  
Javier S. Castresana ◽  
Mehdi H. Shahi
Keyword(s):  
Brain Tumors ◽  
Human Body ◽  
Diagnostic Tool ◽  
Poor Prognosis ◽  
Treatment Options ◽  
Diagnosis And Treatment ◽  
Therapeutic Success ◽  
Knowing That ◽  
Almost All ◽  
Cell Signaling Pathways

Glioblastoma (GB) is one of the most common types of lethal brain tumors. Although several treatment options are available including surgery, along with adjuvant chemo and radiotherapy, the disease has a poor prognosis and patients generally die within 14 months of diagnosis. GB is chemo and radio resistant. Thus, there is a critical need for new insights into GB treatment to increase the chance of therapeutic success. This is why microRNA (miRNA) is being potentially considered in the diagnosis and treatment of glioblastoma. The objective of our review is to provide a holistic picture of GB up-regulated and down-regulated miRNA, in relationship with the expression of other genes, cell signaling pathways, and their role in GB diagnosis and treatment. MiRNA treatment is being considered to be used against GB together with radiotherapy and chemotherapy. Moreover, the use of miRNA as a diagnostic tool has also begun. Knowing that miRNAs are isolated in almost all human body fluids and that there are more than 3000 miRNAs in the human genome, plus the fact that each miRNA controls hundreds of different mRNAs, there is still much study needed to explore how miRNAs relate to GB for its proliferation, progression, and inhibition.

scholarly journals Epilepsy in Mitochondrial Diseases—Current State of Knowledge on Aetiology and Treatment

Children ◽  
2021 ◽  
Vol 8 (7) ◽  
pp. 532
Author(s):  
Dorota Wesół-Kucharska ◽  
Dariusz Rokicki ◽  
Aleksandra Jezela-Stanek
Keyword(s):  
Oxidative Phosphorylation ◽  
Mitochondrial Dysfunction ◽  
Mitochondrial Disease ◽  
Clinical Picture ◽  
Poor Prognosis ◽  
Treatment Options ◽  
Mitochondrial Diseases ◽  
Energy Deficit ◽  
Atp Production ◽  
Current State

Mitochondrial diseases are a heterogeneous group of diseases resulting from energy deficit and reduced adenosine triphosphate (ATP) production due to impaired oxidative phosphorylation. The manifestation of mitochondrial disease is usually multi-organ. Epilepsy is one of the most common manifestations of diseases resulting from mitochondrial dysfunction, especially in children. The onset of epilepsy is associated with poor prognosis, while its treatment is very challenging, which further adversely affects the course of these disorders. Fortunately, our knowledge of mitochondrial diseases is still growing, which gives hope for patients to improve their condition in the future. The paper presents the pathophysiology, clinical picture and treatment options for epilepsy in patients with mitochondrial disease.

Primary carcinoid tumour of nasal septum

2008 ◽  
Vol 123 (7) ◽  
pp. 789-792 ◽  
Author(s):  
T Galm ◽  
N Turner
Keyword(s):  
Nasal Septum ◽  
World Literature ◽  
Carcinoid Syndrome ◽  
Carcinoid Tumour ◽  
Primary Tumour ◽  
Neck Region ◽  
Head And Neck Region ◽  
Carcinoid Tumours ◽  
History Of

AbstractObjective:We present the first reported case of primary carcinoid tumour of the nasal septum.Method:Case report of our experience of a carcinoid tumour of the nasal septum. We discuss our clinical, radiological and pathological findings.Result:An 83-year-old woman presented with a history of left-sided nasal blockage. Clinical examination showed a unilateral, left-sided nasal polyp. Further imaging and histological analysis confirmed this to be a carcinoid tumour. Carcinoid tumours outside the gastrointestinal tract are rare. There have been reports of carcinoid tumours in the head and neck region, but no published cases occurring in the nasal septum. Our management involved wide surgical resection with regular follow up to monitor for recurrence and for the development of carcinoid syndrome. Four years from initial presentation, the patient remained free of the primary tumour and had displayed no signs or symptoms suggestive of carcinoid syndrome.Conclusion:To the authors' best knowledge, and after searching the world literature, the presented case represents the first report of primary carcinoid tumour of the nasal septum. Despite its rarity, this tumour should be considered as part of the differential diagnosis, as timely recognition and intervention are critical for successful treatment.

scholarly journals Wilkie’s Syndrome Following Weight Loss in a Trauma Patient

2020 ◽  
pp. 1-3
Author(s):  
George Oosthuizen ◽  
George Oosthuizen
Keyword(s):  
Weight Loss ◽  
Trauma Patient ◽  
Superior Mesenteric Artery ◽  
Mesenteric Artery ◽  
Treatment Options ◽  
Rare Condition ◽  
Feeding Tube ◽  
Significant Weight Loss ◽  
The Third ◽  
Wilkie’S Syndrome

Wilkie’s syndrome, or superior mesenteric artery syndrome, is a rare condition of duodenal obstruction caused by compression of the superior mesenteric artery on the third part of the duodenum. The diagnosis should be considered in a patient who has experienced significant weight loss and now presents with persistent vomiting, especially if the vomiting occurs with the patient in the supine position and is alleviated by the lateral or prone position. The diagnosis can be confirmed by imaging studies demonstrating compression of the third part of the duodenum, and the main aim of treatment should be to pass a feeding tube beyond the point of obstruction to allow enteral feeding. The condition improves spontaneously with weight gain. Further treatment options include parenteral feeding and operative bypass in select cases. Here we present a case of Wilkie’s syndrome in a trauma patient with significant weight loss, together with a review of the literature on this interesting topic.

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