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Terminology, classification and diagnosis of NET-s („carcinoid-tumours“) Lukáš Plank, Karol Kajo

General medicine undergraduate (VLs) PA II March 2008. Terminology, classification and diagnosis of NET-s („carcinoid-tumours“) Lukáš Plank, Karol Kajo ÚPA JLF UK a MFN v Martine. NET and its definition: PES, APUD and a concept of NETs. 1. „neuro + endocrine“:

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Terminology, classification and diagnosis of NET-s („carcinoid-tumours“) Lukáš Plank, Karol Kajo

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  1. General medicine undergraduate (VLs) PA IIMarch 2008 Terminology, classification and diagnosis of NET-s („carcinoid-tumours“) Lukáš Plank, Karol Kajo ÚPA JLF UK a MFN v Martine

  2. NET and its definition: PES, APUD and a concept of NETs 1. „neuro + endocrine“: Gastro-intestinal , respiratory and other neuroendocrine system (NES): - cells and their products have „endocrine“ properties + (paracrine and a autocrine), - peptides and amines of GI-NES communicate with enteric NS, serve as neurotransmitters and neuromodulators in CNS as well as in enteric and very probably in other NS. 2. neuroendocrine tumour (NET) arises by neoplastic transformation of NES cell: a./ undifferentiated progenitor (pluripotent stem) cells, or b./ differentiated NES cells (of GIT, respiratory system, etc)

  3. NET: a „specific“ tumour in oncology • NETs • rare tumours - low incidence, • diagnostics is „therapeutically orientated“ • problems: • terminology and classification (typing), • biolog. properties and grading, • staging • insufficient experiences with their dg. = • „specialization“ and centralization (E-NET)

  4. NETs: problems of terminology and classification I. • confusion („historical“), for one Tu different names, • e.g. pancreatic NET: „islet cell tumour“, nesidioma, • nesidioblastoma, apudoma, adenoma, carcinoma, etc. • „correct“ name NET-s = spectrum: • classic „carcinoids“ (slow growth) up to highly malignant undifferent. NE Ca • ff. active NET-s could be designated according to products: • (insulinoma, glucagonoma) • possible academically correct but complicated combined names, • related to function + biolog. properties, e.g.: • primary pulmonary NET (or NE Ca), with production of somatostatin,...

  5. NETs: problems of terminology and classification II. Classification WHO (2000, 2004): • compilation of morfol. a ff. properties for definition • common schematical instructions for typing, grading a staging (not TNM) • combined patol./morphol. + clin. criterias: localisation, size, invasivity (and angioinvasivity), MTS, ff. state, prolif. activity (MAI, Ki-67 index)

  6. NETs acc. WHO classification 2000: biolog. behaviour and grade of malignancy (grading) I. well differentiated endocrine tumour (WDET) • „benign“ • with uncertain biological behaviour II. well differentiated endocrine carcinoma (WDEC) = low grade NE carcinoma III. poorly differentiated endocrine carcinoma (PDEC) = high grade G I. G II. G III.

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