Update on Pathophysiology, Treatment, and Complications of Carcinoid Syndrome

Clement, Dominique; Ramage, John; Srirajaskanthan, Raj

doi:10.1155/2020/8341426

Cited by 52 publications

(60 citation statements)

References 112 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Carcinoid syndrome is a paraneoplastic syndrome characterized by combination of symptoms which occurs due to various vasoactive substances secreted by the NENs into the systemic circulation[ 57 , 58 ]. Various vasoactive substances include serotonin, histamine, kallikrein, prostaglandins E and F, norepinephrine, motilin, and tachykinins such as substance P. Carcinoid syndrome usually occur in the setting of extensive liver metastasis, where these vasoactive substances cannot be metabolized and enter the systemic circulation through the hepatic veins.…”

Section: Clinical Features Of Gep-nens With Hormone Hypersecretionmentioning

confidence: 99%

Gastroenteropancreatic neuroendocrine neoplasms: A clinical snapshot

Fernandez¹,

Agarwal²,

Pottakkat³

et al. 2021

WJGS

View full text Add to dashboard Cite

Our understanding about the epidemiological aspects, pathogenesis, molecular diagnosis, and targeted therapies of neuroendocrine neoplasms (NENs) have drastically advanced in the past decade. Gastroenteropancreatic (GEP) NENs originate from the enteroendocrine cells of the embryonic gut which share common endocrine and neural differentiation factors. Most NENs are well-differentiated, and slow growing. Specific neuroendocrine biomarkers that are used in the diagnosis of functional NENs include insulin, glucagon, vasoactive intestinal polypeptide, gastrin, somatostatin, adrenocorticotropin, growth hormone releasing hormone, parathyroid hormone-related peptide, serotonin, histamine, and 5-hydroxy indole acetic acid (5-HIAA). Biomarkers such as pancreatic polypeptide, human chorionic gonadotrophin subunits, neurotensin, ghrelin, and calcitonin are used in the diagnosis of non-functional NENs. 5-HIAA levels correlate with tumour burden, prognosis and development of carcinoid heart disease and mesenteric fibrosis, however several diseases, medications and edible products can falsely elevate the 5-HIAA levels. Organ-specific transcription factors are useful in the differential diagnosis of metastasis from an unknown primary of well-differentiated NENs. Emerging novel biomarkers include circulating tumour cells, circulating tumour DNA, circulating micro-RNAs, and neuroendocrine neoplasms test (NETest) (simultaneous measurement of 51 neuroendocrine-specific marker genes in the peripheral blood). NETest has high sensitivity (85%-98%) and specificity (93%-97%) for the detection of gastrointestinal NENs, and is useful for monitoring treatment response, recurrence, and prognosis. In terms of management, surgery, radiofrequency ablation, symptom control with medications, chemotherapy and molecular targeted therapies are all considered as options. Surgery is the mainstay of treatment, but depends on factors including age of the individual, location, stage, grade, functional status, and the heredity of the tumour (sporadic vs inherited). Medical management is helpful to alleviate the symptoms, manage inoperable lesions, suppress postoperative tumour growth, and manage recurrences. Several molecular-targeted therapies are considered second line to somatostatin analogues. This review is a clinical update on the pathophysiological aspects, diagnostic algorithm, and management of GEP NENs.

show abstract

Section: Clinical Features Of Gep-nens With Hormone Hypersecretionmentioning

confidence: 99%

Gastroenteropancreatic neuroendocrine neoplasms: A clinical snapshot

Fernandez¹,

Agarwal²,

Pottakkat³

et al. 2021

WJGS

View full text Add to dashboard Cite

show abstract

“…15 The ideal administration schedule for octreotide IR to prevent carcinoid crisis remains unknown, but guidelines (ENETS, NAN-ETS, and UKNETS) suggest different octreotide dosing schedules, including pre-, intra-and/or postoperative, to prevent symptoms. 52…”

Section: Carcinoid Crisismentioning

confidence: 99%

“…To prevent carcinoid crisis that could be induced by these latter circumstances, patients often use octreotide IR prophylactically [ 15 ]. The ideal administration schedule for octreotide IR to prevent carcinoid crisis remains unknown, but guidelines (ENETS, NANETS, and UKNETS) suggest different octreotide dosing schedules, including pre‐, intra‐, and/or postoperative, to prevent symptoms [ 52 ].…”

Section: Rationale For Use Of Octreotide Ir In Current Clinical Practicementioning

confidence: 99%

Opportunities to Improve Symptom Control with Somatostatin Congeners in GEP-NETs: A Review of Key Issues

Anthony

O’Dorisio

2021

The Oncologist

View full text Add to dashboard Cite

Octreotide acetate (octreotide) is the most prescribed and most studied somatostatin congener, or analog, for gastroenteropancreatic neuroendocrine tumors (GEP‐NETs) and carcinoid syndrome, the latter of which may be characterized by debilitating diarrhea and flushing. Approved in the U.S. more than 30 years ago, octreotide is widely used to control the symptoms of carcinoid syndrome and has been shown to demonstrate antiproliferative activity. The two formulations available in the U.S. include a subcutaneous immediate‐release (IR) injection introduced in 1989 and a long‐acting repeatable (LAR) intramuscular injection approved in 1999. Lanreotide depot (lanreotide), a more recent somatostatin congener, has been available in the U.S. since 2014. Despite widespread use of octreotide LAR, several key challenges exist with the current depot‐based treatment paradigm. Studies indicate that LAR formulations are associated with continued unmet patient needs, owing in part to a loss of bioactivity over time that may necessitate progressive supplemental treatment with IR octreotide to adequately control symptoms. Clinicians should understand the key differences in the pharmacokinetic profiles of the LAR and IR formulations that may contribute to bioactivity loss and somatostatin receptor desensitization. In addition, there is a need to re‐evaluate the role of IR octreotide in combination with depot therapy to provide consistent bioavailability and better control of carcinoid syndrome symptoms. The purpose of this review is to explore all these issues and to re‐establish a rationale for the IR formulation, particularly with respect to novel use cases and its use during the COVID‐19 pandemic. Implications for Practice There is a need to re‐evaluate the role of immediate‐release octreotide in combination with depot therapy to provide consistent bioavailability and better control of carcinoid syndrome symptoms.

show abstract

“…It is found in up to 45% of patients and may be caused by LN and/or distant metastasis [ 22 ]. Serotonin and other cytokines released from the tumour cells may induce fibrosis, leading to carcinoid heart disease and abdominal fibrotic reactions (desmoplasia) [ 23 •, 24 ].…”

Section: Functionalitymentioning

confidence: 99%

How to Manage Small Intestine (Jejunal and Ileal) Neuroendocrine Neoplasms Presenting with Liver Metastases?

2021

View full text Add to dashboard Cite

Purpose of Review Small intestinal neuroendocrine neoplasms (siNENs) are slowly growing tumours with a low malignant potential. However, more than half of the patients present with distant metastases (stage IV) and nearly all with locoregional lymph node (LN) metastases at the time of surgery. The value of locoregional treatment is discussed controversially. Recent Findings In stage I to III disease, locoregional surgery was currently shown to be curative prolonging survival. In stage IV disease, surgery may prolong survival in selected patients with the chance to cure locoregional disease besides radical/debulking liver surgery. It may improve the quality of life and may prevent severe local complications resulting in a state of chronic malnutrition and severe intestinal ischaemia or bowel obstruction. Summary Locoregional tumour resection offers the opportunity to be curative or to focus therapeutically on liver metastasis, facilitating various other therapeutic modalities. Risks and benefits of the surgical intervention need to be balanced individually.

show abstract

Update on Pathophysiology, Treatment, and Complications of Carcinoid Syndrome

Cited by 52 publications

References 112 publications

Gastroenteropancreatic neuroendocrine neoplasms: A clinical snapshot

Gastroenteropancreatic neuroendocrine neoplasms: A clinical snapshot

Opportunities to Improve Symptom Control with Somatostatin Congeners in GEP-NETs: A Review of Key Issues

How to Manage Small Intestine (Jejunal and Ileal) Neuroendocrine Neoplasms Presenting with Liver Metastases?

Contact Info

Product

Resources

About