Nowotwory neuroendokrynne jelita cienkiego i wyrostka robaczkowego — zasady postępowania (rekomendowane przez Polską Sieć Guzów Neuroendokrynnych)

Abstract

 224Neuroendocrine neoplasms of the small intestine and appendix -management guidelines Bednarczuk Tomasz et al. SZKOLENIE PODYPLOMOWE AbstractThis study presents the revised Polish guidelines regarding the management of patients suffering from neuroendocrine neoplasms (NENs) of the small intestine and appendix. The small intestine, especially the ileum, is the most common location for these neoplasms. Most are well differentiated and slow growing. Their symptoms may be atypical, which can result in delayed or accidental diagnosis. Appendicitis is usually the first manifestation of NEN in this location. Typical symptoms of carcinoid syndrome occur in approximately 20-30% of patients suffering from small intestinal NENs with distant metastases. The main cause of death in patients with carcinoid syndrome is carcinoid heart disease.The most useful laboratory test is the determination of chromogranin A, while concentration of 5-hydroxyindoleacetic acid is helpful in the diagnostics of carcinoid syndrome. For visualisation, ultrasound, computed tomography, magnetic resonance imaging, colonoscopy, video capsule endoscopy, double-balloon enteroscopy, and somatostatin receptor scintigraphy may be used. A detailed histological report is crucial for the proper diagnostics and therapy of NENs of the small intestine and appendix. The treatment of choice is surgical management, either radical or palliative. The pharmacological treatment of the hormonally active and non-active small intestinal NENs as well as NENs of the appendix is based on long-acting somatostatin analogues. In patients with generalised NENs of the small intestine in progress during the SSA treatment, with good expression of somatostatin receptors, the first-line treatment should be radioisotope therapy, while targeted therapies, such as everolimus, should be considered afterwards. When the above therapies are exhausted, in certain cases chemotherapy may be considered.(Endokrynol Pol 2017; 68 (2): 223-236)

Somatostatin receptor scintigraphy in the follow up of neuroendocrine neoplasms of appendix

“…According to Bednarczuk et al[ 14 ], SRS is more sensitive than radiological methods with the sensitivity around 80% for the detection of the primary tumor site. Investigations with positron emitting radiopharmaceuticals [Gallium-68( 68 Ga)-peptides] are the preferred imaging method, particularly if the lesions are smaller than 1 cm[ 4 ].…”

“…Investigations with positron emitting radiopharmaceuticals [Gallium-68( 68 Ga)-peptides] are the preferred imaging method, particularly if the lesions are smaller than 1 cm[ 4 ]. For localization of primary tumor and assess the disease stage, SRS in addition to CT and MRI is recommended, but in the case of incomplete surgery of the ANET or if distant metastases are suspected, SRS should be performed[ 14 ]. According to other investigations sensitivity, specificity, accuracy, positive and negative predictive values of 99m Tc- SRS were; 96%, 100%, 97%, 100% and 94% respectively, in NENs in general, with high negative predictive value in ANET[ 15 ], which is in concordance with our results.…”

Impact of enteroscopy on diagnosis and management of small bowel tumors

“…Somatostatin receptor imaging seems to be useful pre-operatively, since it confirms the presence and functionality of the disease, however it is limited to provide the exact location. CE should be the endoscopic method of choice in order to guide the route of DAE ( 78 - 80 ). DAE should be preferentially performed in patients with previous positive exams, for biopsies sampling, and marking the exact location of the lesion ( 81 - 84 ) ( Figure 5 ).…”

Tandem peptide receptor radionuclide therapy using 90Y/177Lu-DOTATATE for neuroendocrine tumors efficacy and side-effects - polish multicenter experience

“…Neuroendocrine tumors are a heterogeneous group of neoplasms, arising from cells of the endocrine system, with various clinical behaviors. Although they may show hormonal activity (known as hormonally active tumors), a significant proportion do not produce enough hormones and/or biogenic amines to cause clinical symptoms (classed as hormonally inactive tumors) [1][2][3][4]. Although these neoplasms are considered rare, a significant increase in the incidence and detectability of NET has been noted in many epidemiological studies in recent years [2][3][4][5].…”

“…The data of single center trial using simultaneous 90 Y/ 177 Lu-DOTATATE was published in 2017 [20]. These results enabled inclusion of PRRT using 90 Y/ 177 Lu-DOTATATE tandem therapy to the treatment protocols used in Poland and the recommendations of the Polish Network of Neuroendocrine Tumors [1,2,21].…”