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Difference between revisions of "Neuroendocrine tumour of the pancreas"
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Neuroendocrine tumour of the pancreas (view source)
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**[[Neurofibromatosis type 1]].<ref name=pmid15249710>{{Cite journal | last1 = Alexakis | first1 = N. | last2 = Connor | first2 = S. | last3 = Ghaneh | first3 = P. | last4 = Lombard | first4 = M. | last5 = Smart | first5 = HL. | last6 = Evans | first6 = J. | last7 = Hughes | first7 = M. | last8 = Garvey | first8 = CJ. | last9 = Vora | first9 = J. | title = Hereditary pancreatic endocrine tumours. | journal = Pancreatology | volume = 4 | issue = 5 | pages = 417-33; discussion 434-5 | month = | year = 2004 | doi = 10.1159/000079616 | PMID = 15249710 }}</ref> | **[[Neurofibromatosis type 1]].<ref name=pmid15249710>{{Cite journal | last1 = Alexakis | first1 = N. | last2 = Connor | first2 = S. | last3 = Ghaneh | first3 = P. | last4 = Lombard | first4 = M. | last5 = Smart | first5 = HL. | last6 = Evans | first6 = J. | last7 = Hughes | first7 = M. | last8 = Garvey | first8 = CJ. | last9 = Vora | first9 = J. | title = Hereditary pancreatic endocrine tumours. | journal = Pancreatology | volume = 4 | issue = 5 | pages = 417-33; discussion 434-5 | month = | year = 2004 | doi = 10.1159/000079616 | PMID = 15249710 }}</ref> | ||
===Classification=== | ===WHO classification of 2017=== | ||
Five categories:<!-- https://www.esp-congress.org/fileadmin/user_upload/Congress_2016/IAP_ESP_Presentations/Mon/0830-1200/SY-02/SY-02-004-Kl%C3%B6ppel-Update%20of%20WHO%20classification%20of%20endocrine%20pancreatic%20tumours.pdf --> | |||
*NET G1. Ki67 Index < 3%, Mitotic Index < 2/10HPF | |||
*NET G2. Ki67 Index 3-20%, Mitotic Index 2-20/10HPF | |||
*NET G3. Ki67 Index > 20%, Mitotic Index > 20/10 HPF AND well-differentiated, expressing neuroendocrine differentiation and hormones | |||
*Neuroendocrine carcinoma (NEC G3). Ki 67 Index > 20%, Mitotic Index > 20/10 HPF Poorly differentiated, expressing neuroendocrine differentiation but lacking exocrine markers. | |||
*[[MiNEN]]. mixed endocrine-nonendocrine neoplasm: components of a non-endocrine carcinoma (mostly ductal adenocarcinoma or acinar cell carcinoma) combined with a neuroendocrine neoplasm | |||
===Classification by product=== | |||
Based on peptide produced in the pancreatic islets: | Based on peptide produced in the pancreatic islets: | ||
#Glucagon from alpha cells ([[glucagonoma]]). | #Glucagon from alpha cells ([[glucagonoma]]). | ||
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==Gross== | ==Gross== | ||
*Usually in the head of the pancreas - 68% in one series,<ref name=pmid22869477>{{Cite journal | last1 = Oh | first1 = TG. | last2 = Chung | first2 = MJ. | last3 = Park | first3 = JY. | last4 = Bang | first4 = SM. | last5 = Park | first5 = SW. | last6 = Chung | first6 = JB. | last7 = Song | first7 = SY. | title = Prognostic factors and characteristics of pancreatic neuroendocrine tumors: single center experience. | journal = Yonsei Med J | volume = 53 | issue = 5 | pages = 944-51 | month = Sep | year = 2012 | doi = 10.3349/ymj.2012.53.5.944 | PMID = 22869477 | PMC = 3423842}}</ref> and 50% in another series.<ref name=pmid22869477 | *Usually in the head of the pancreas - 68% in one series,<ref name=pmid22869477>{{Cite journal | last1 = Oh | first1 = TG. | last2 = Chung | first2 = MJ. | last3 = Park | first3 = JY. | last4 = Bang | first4 = SM. | last5 = Park | first5 = SW. | last6 = Chung | first6 = JB. | last7 = Song | first7 = SY. | title = Prognostic factors and characteristics of pancreatic neuroendocrine tumors: single center experience. | journal = Yonsei Med J | volume = 53 | issue = 5 | pages = 944-51 | month = Sep | year = 2012 | doi = 10.3349/ymj.2012.53.5.944 | PMID = 22869477 | PMC = 3423842}}</ref> and 50% in another series.<ref name=pmid22869477/> | ||
==Microscopic== | ==Microscopic== | ||
Features: | Features: | ||
| Line 92: | Line 99: | ||
Image: Pancreatic neuroendocrine tumour - 2 -- high mag.jpg | Pancreatic NET - high mag. (WC/Nephron) | Image: Pancreatic neuroendocrine tumour - 2 -- high mag.jpg | Pancreatic NET - high mag. (WC/Nephron) | ||
</gallery> | </gallery> | ||
[[File: PANC NET LG 1 sl 1.png| Low grade pancreatic neuroendocrine tumor]] | |||
[[File: PANC NET LG 1 sl 2.png| Low grade pancreatic neuroendocrine tumor]] | |||
[[File: PANC NET LG 1 sl 3.png| Low grade pancreatic neuroendocrine tumor]] | |||
[[File: PANC NET LG 1 sl 4.png| Low grade pancreatic neuroendocrine tumor]] | |||
[[File: PANC NET LG 1 sl 5.png| Low grade pancreatic neuroendocrine tumor]] | |||
Low grade pancreatic neuroendocrine tumor. A. Stromal trabeculums (black arrows) support the uniform, non-necrotic tumor that has sharp edges (green arrows). B. Modestly variable, rounded nuclei show open chromatin and nucleoli. Individual pyknotic nuclei (black arrows) are insufficient as evidence of necrosis sufficient to increase grade. C. Immunostain. Cytoplasm is diffusely synaptophysin positive. D. Immunostain. Cytoplasm shows stippled chromogranin positivity. E. Immunostain. Only scattered nuclei are Ki67 positive. | |||
www: | www: | ||
*[http://path.upmc.edu/cases/case172/micro.html Islet cell tumour (upmc.edu)]. | *[http://path.upmc.edu/cases/case172/micro.html Islet cell tumour (upmc.edu)]. | ||
| Line 103: | Line 120: | ||
*Synaptophysin +ve. | *Synaptophysin +ve. | ||
*CD56 +ve. | *CD56 +ve. | ||
*[[PAX8]] +ve (74% of cases<ref name=pmid20890270>{{Cite journal | last1 = Sangoi | first1 = AR. | last2 = Ohgami | first2 = RS. | last3 = Pai | first3 = RK. | last4 = Beck | first4 = AH. | last5 = McKenney | first5 = JK. | last6 = Pai | first6 = RK. | title = PAX8 expression reliably distinguishes pancreatic well-differentiated neuroendocrine tumors from ileal and pulmonary well-differentiated neuroendocrine tumors and pancreatic acinar cell carcinoma. | journal = Mod Pathol | volume = 24 | issue = 3 | pages = 412-24 | month = Mar | year = 2011 | doi = 10.1038/modpathol.2010.176 | PMID = 20890270 }}</ref>). | |||
Functional tumours: | Functional tumours: | ||