Pathogenesis and Clinical Management of Mesenteric Fibrosis in Small Intestinal Neuroendocine Neoplasms: A Systematic Review

Koumarianou, Anna; Alexandraki, Krystallenia Ι.; Wallin, Göran; Kaltsas, Gregory; Daskalakis, Kosmas

doi:10.3390/jcm9061777

Cited by 22 publications

(28 citation statements)

References 116 publications

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“…Nearly 20% of small intestinal NENs are associated with release of amines and peptides to induce carcinoid syndrome. Moreover, they are associated with fibrotic complications such as mesenteric fibrosis and carcinoid heart disease (CaHD)[ 20 ].…”

Section: Clinical Profiles Of Gep-nens Based On the Location Of Tumormentioning

confidence: 99%

“…These growth factors include transforming growth factor (TGF)-β, connective tissue growth factor, platelet-derived growth factor (PDGF), insulin-like growth factor 1, epidermal growth factor (EGF), and TGF-α. The mesenteric fibrosis can present as acute intestinal obstruction, acute mesenteric ischaemia or with chronic symptoms including prandial abdominal pain, venous stasis, malabsorption, and malnutrition[ 20 ]. The pathogenesis of CaHD, characterized by right heart involvement that occurs in nearly 60% of carcinoid syndrome[ 21 ], is mediated by high circulating serotonin levels[ 20 ].…”

Section: Clinical Profiles Of Gep-nens Based On the Location Of Tumormentioning

confidence: 99%

“…However, they can sometimes present with bowel obstruction. The duodenal NECs are rare aggressive neoplasms with invasion into lymphovascular structures or duodenal wall and can present with distant metastasis[ 20 ]. Somatostati-nomas, originating from D cells of the duodenum near the ampulla, can be mostly sporadic or can be rarely associated with MEN1 or NF1[ 22 , 23 ].…”

Section: Clinical Profiles Of Gep-nens Based On the Location Of Tumormentioning

confidence: 99%

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Gastroenteropancreatic neuroendocrine neoplasms: A clinical snapshot

Fernandez¹,

Agarwal²,

Pottakkat³

et al. 2021

WJGS

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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.

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Section: Clinical Profiles Of Gep-nens Based On the Location Of Tumormentioning

confidence: 99%

Section: Clinical Profiles Of Gep-nens Based On the Location Of Tumormentioning

confidence: 99%

Section: Clinical Profiles Of Gep-nens Based On the Location Of Tumormentioning

confidence: 99%

See 1 more Smart Citation

Gastroenteropancreatic neuroendocrine neoplasms: A clinical snapshot

Fernandez¹,

Agarwal²,

Pottakkat³

et al. 2021

WJGS

View full text Add to dashboard Cite

show abstract

“…The pathophysiologic mechanisms underlying the occurrence of diarrhea include secretory, osmotic, inflammatory pathways and altered motility [17]. Other more specific causes can be found in patients with NET, e.g., the development of mesenteric fibrosis that can contribute to profound diarrhea by partial obstruction-knicking of the bowel and induce venous stasis, thus leading to malabsorption [18].…”

Section: Etiology Criteriamentioning

confidence: 99%

Differential Diagnosis and Management of Diarrhea in Patients with Neuroendocrine Tumors

Pusceddu

Rossi

Torchio

et al. 2020

JCM

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Diarrhea is a recurrent symptom in patients with neuroendocrine tumors (NETs) and can represent different etiologies; thus, differential diagnosis is challenging. This paper distinguishes the different causes of chronic diarrhea in patients with gastroenteropancreatic NETs, with the aim to identify the most appropriate therapeutic approach. Underlying causes of diarrhea can be multifactorial, including not only diarrhea that is related to specific hormonal hypersecretory syndromes, but also diarrhea that is secondary to the following: extensive surgery which can cause pancreatic exocrine insufficiency or short bowel syndrome, treatment with somatostatin analogs or other antineoplastic agents, and bile acid malabsorption. After initial management of diarrhea with general treatments (dietary modification, use of antidiarrheals), a proper differential diagnosis is necessary to treat patients with specific etiology-driven therapeutic approaches, such as somatostatin analogs, pancreatic enzyme replacement therapy, and tryptophan hydroxylase inhibitors. In conclusion, NETs should be considered in the differential diagnosis of patients suffering from chronic diarrhea, after the exclusion of more common etiologies. Furthermore, physicians should keep in mind that several different etiologies might be responsible for diarrhea occurrence in NET patients. A prompt diagnosis of the actual cause of diarrhea is necessary to guide the treatment and a multidisciplinary approach is mandatory.

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“…Several interesting recommendations are proposed in the review manuscripts presented in this Special Issue: (i) Pusceddu et al [ 7 ] highlighted that diarrhea, although expected in the context of a small-bowel NET—particularly if associated with carcinoid syndrome—may have other etiologies, including previous surgery, concomitant medical treatments, and exocrine pancreatic insufficiency, which may develop in some patients receiving somatostatin analogs [ 8 ] or with mesenteric fibrosis. This last issue was intensely analyzed by Koumarianou [ 9 ] et al, who performed a systematic review of the literature concerning the pathogenesis and clinical management of mesenteric fibrosis, which may lead to severe symptoms, a deteriorated quality of life, and significant morbidity in patients with small-intestine NETs.…”

mentioning

confidence: 99%