Colonic and rectal NET's
ABSTRACT Neuroendocrine tumours of the Colon and Rectum are rare but are increasing in incidence as a result of greater investigation with endoscopy and improved histological reporting. Classification with the 2010 WHO TNM staging system as well as grading based on the Ki-67 index has led to improved prognostic assessment. The use of Endoanal Ultrasound has increased the sensitivity of detection of depth of invasion and lymphovascular involvement, which is associated with a poor prognosis. Standard polypectomy has largely been replaced by endoscopic mucosal resection of smaller polyps, although newer techniques such as band ligation or endoscopic submucosal dissection are likely to be associated with less residual disease. The management of advanced disease remains a challenge but new treatments such as Peptide Receptor Targeted therapy and molecular targeted treatments offer hope of improved progression free survival in non-resectable disease.
SourceAvailable from: Andreas Kjaer[Show abstract] [Hide abstract]
ABSTRACT: To date, empirical literature has generally been considered lacking in relation to neuroendocrine carcinomas (NECs), the highly malignant subgroup of neuroendocrine neoplasms. NECs are often found in the lungs or the gastroenteropancreatic (GEP) system and can be of small or large cell type. Concentrating on GEP-NECs, we can conclude that survival times are poor, with a median of only 4–16 months depending on disease stage and primary site. Further, this aggressive disease appears to be on the rise, with incidence numbers increasing while survival times are stagnant. Treatment strategies concerning surgery are often undecided and second-line chemotherapy is not yet established. After an OPEN ACCESS Diagnostics 2015, 5 120 analysis of over 2600 articles, we can conclude that there is indeed more empirical literature concerning GEP-NECs available than previously assumed. This unique review is based on 333 selected articles and contains detailed information concerning all aspects of GEP-NECs. Namely, the classification, histology, genetic abnormalities, epidemiology, origin, biochemistry, imaging, treatment and survival of GEP-NECs are described. Also, organ-specific summaries with more detail in relation to disease presentation, diagnosis, treatment and survival are presented. Finally, key points are discussed with directions for future research priorities.04/2015; 2015(5):119-176. DOI:10.3390/diagnostics5020119
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ABSTRACT: Lymph node metastasis is rare in small (i.e., <10 mm) rectal neuroendocrine tumors (NETs). In addition to tumor size, pathological features such as the mitotic or Ki-67 proliferation index are associated with lymph node metastasis in rectal NETs. We recently treated a patient who underwent endoscopic treatment of a small, grade 1 rectal NET that recurred in the form of perirectal lymph node metastasis 7 years later. A 7-mm-sized perirectal lymph node was noted at the time of the initial endoscopic treatment. The same lymph node was found to be slightly enlarged on follow-up and finally confirmed as a metastatic NET. Therefore, the perirectal lymph node metastasis might have been present at the time of the initial diagnosis. However, the growth rate of the lymph node was extremely low, and it took 7 years to increase in size from 7 to 10 mm. NETs with low Ki-67 proliferation index and without mitotic activity may grow extremely slowly even if they are metastatic.04/2015; 13(2):175-9. DOI:10.5217/ir.2015.13.2.175
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ABSTRACT: Rare endocrine-metabolic diseases (REMD) represent an important area in the field of medicine and pharmacology. The rare diseases of interest to endocrinologists involve all fields of endocrinology, including rare diseases of the pituitary, thyroid and adrenal glands, paraganglia, ovary and testis, disorders of bone and mineral metabolism, energy and lipid metabolism, water metabolism, and syndromes with possible involvement of multiple endocrine glands, and neuroendocrine tumors. Taking advantage of the constitution of a study group on REMD within the Italian Society of Endocrinology, consisting of basic and clinical scientists, a document on the taxonomy of REMD has been produced.Journal of endocrinological investigation 11/2014; 38(2). DOI:10.1007/s40618-014-0202-6 · 1.55 Impact Factor