Update of the diagnostic and therapeutic guidelines for gastro-entero-pancreatic neuroendocrine neoplasms (recommended by the Polish Network of Neuroendocrine Tumours) [Aktualizacja zaleceń ogólnych dotyczących postępowania diagnostyczno-terapeutycznego w nowotworach neuroendokrynnych układu pokarmowego (rekomendowane przez Polską Sieć Guzów Neuroendokrynnych)]

Abstract

This article is available in open access under Creative Common Attribution-Non-Commercial-No Derivatives 4.0 International (CC BY-NC-ND 4.0) license, allowing to download articles and share them with others as long as they credit the authors and the publisher, but without permission to change them in any way or use them commercially  388 Guidelines Beata Kos-Kudła et al. Guidelinespersons/year, with the primary lesion most frequently found in the small intestine (37.4%). Since 2000, rectal NENs have been diagnosed more frequently than small intestine NENs [2, 6-8]. The incidence of GEP-NETs in the USA, based on SEER data, was 3.56/100,000 persons/year. In Europe, the incidence of GEP-NETs is also increasing -from 1.33 to 2.33/100,000 persons/year; however, these data come from different registries and are mainly retrospective. Higher incidence is observed among men (5.35/100,000 persons/year) compared to women (4.76/100,000 persons/year) [1, 5-7]. The vast majority of NENs are sporadic, well-differentiated tumours. However, GEP-NETs originating from the pancreas, duodenum, stomach, and much less frequently, from the thymus and lungs sometimes constitute an element of multiple endocrine neoplasia type 1 (MEN-1) syndrome. Pancreatic neuroendocrine tumours (PanNETs) may also be associated with von Hippel-Lindau (VHL) syndrome, tuberous sclerosis complex (TSC), and neurofibromatosis (NF). In these congenital diseases, NETs can be multifocal and occur 10-20 years earlier than in sporadic cases. The frequency of the hereditary causes (MEN-1, VHL) is estimated at about 5%. Genome studies revealed the presence of germline mutations in, e.g., MUTYH, CHEK2, and BRCA2 and a propensity to PanNETs in approximately 17% of the studied population [1]. Diagnostics Biochemical diagnosticsBiochemical diagnostics of NENs involves the following: A. Non-specific markersThe most frequently used diagnostic method is determination of the chromogranin A (CgA) concentration in the serum (less frequently in the plasma) [1,8,9]. CgA is a relatively stable protein in blood. However, there are two different methods for determining the concentration of CgA: radioimmunoassay (RIA) and enzyme-linked immunosorbent assay (ELISA) in Neuroendocrine neoplasms (NENs) arise from the disseminated system of neuroendocrine cells and can occur in various parts of the body. However, they are most often found in the gastrointestinal tract and lungs. The term NENs includes both well-differentiated neuroendocrine tumours and neuroendocrine carcinomas (NECs), which account for 10-20% of all NENs. The following characteristics of NENs should be considered in the diagnostic and therapeutic process: proliferative activity, presence of somatostatin receptors (SSTRs), tumour growth rate, and extent of the neoplastic disease [1].

Long-Term Complications of Radioligand Therapy with Lutetium-177 and Yttrium-90 in Patients with Neuroendocrine Neoplasms

“…Detailed inclusion criteria were as follows: Well- and moderately-differentiated unresectable metastatic progressive neuroendocrine neoplasm (defined as Ki-67 < 20%, progression according to the RECIST 1.1 (Response Evaluation Criteria In Solid Tumors) criteria, over the previous 12 months); Good expression of somatostatin receptors in qualifying somatostatin receptor scintigraphy (SRS) (SPECT/CT) (radiotracer uptake in the majority of the lesions higher than in normal liver (Krenning scale 3)) or in Gallium-68-PET/CT (SUVmax in the majority of the lesions higher than SUVmax in normal liver); No possibilities of surgical treatment; Chronic treatment with long-acting somatostatin analogues. Exclusion criteria was lack of consent, pregnancy or lactation, Karnofsk’y scale <60, WHO/ECOG 3 or 4, no tracer uptake in SRI, myelosuppression (understood as hemoglobin <8 g/L, or platelets <80.000/µL, or leukocytes <2000/µL, or lymphocytes <500/µL, or neutrophils <1000/µL), renal disfunction (eGFR <30 mL/min, or serum Creatinine >1.8 mg/dL) and liver diseases (ALT 3× over upper limit) [ 2 ]. …”

“…Chronic treatment with long-acting somatostatin analogues. Exclusion criteria was lack of consent, pregnancy or lactation, Karnofsk’y scale <60, WHO/ECOG 3 or 4, no tracer uptake in SRI, myelosuppression (understood as hemoglobin <8 g/L, or platelets <80.000/µL, or leukocytes <2000/µL, or lymphocytes <500/µL, or neutrophils <1000/µL), renal disfunction (eGFR <30 mL/min, or serum Creatinine >1.8 mg/dL) and liver diseases (ALT 3× over upper limit) [ 2 ].…”

“…They can be found in almost every part of human body, but mainly in gastrointestinal system. The most common location of NENs is small intestine [ 2 ]. Majority of NENs are non-functioning tumors, with slow growth, and no clinical symptoms in early stages.…”

“…Majority of NENs are non-functioning tumors, with slow growth, and no clinical symptoms in early stages. They are recognized accidentally, the most often when the liver metastases appear [ 2 , 3 ].…”

“…Radioligand therapy (RLT), previously defined as peptide receptor radionuclide therapy (PRRT), is usually second line of treatment, introduced after inefficiency or no possibility of primary treatment, and with disease progression [ 2 ]. RLT is typically recommended in G1 and G2 grading of NEN.…”