Insulinoma classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Amandeep Singh M.D.[2]

Overview

Insulinoma may be classified according to their malignant potential into 2 sub-types: Benign (90%) and malignant (10%). It is also classified into 2 subtypes based on the number: solitary (90%) and multiple (10%). Previously insulinoma was classified into 2 subtypes based on hormonal level as determined by radioimmunoassay into group A and group B. The staging of malignant insulinoma is based on the AJCC 2010, ENETS and modified ENETS staging classification.

Classification

Classification of insulinoma
Criteria Classification Features
Malignancy potential Benign
  • 90% of insulinomas are benign in nature.
Malignant
  • 10% of Insulinomas have a malignant potential to invade adjacent soft tissues or structures.
  • The malignant type is mostly associated with MEN 1 syndrome.
  • They also have a recurrence rate which is higher in those with MEN1 (21% at 10 and 20 years) than without it (5% at 10 and 7% at 20 years).[1] In one of the recent research papers, the recurrence was described as 4 times more common in individuals with MEN 1.[2]
Based on number Solitary
Multiple
  • 10% of insulinomas can be multiple in number.
Based on the functionality

(clinical manifestations)

Functional
  1. Well-differentiated endocrine tumors, with benign or uncertain behavior.
  2. Well-differentiated endocrine carcinomas with low-grade malignant behavior.
  3. Poorly differentiated endocrine carcinomas with high-grade malignant behavior.
Note: Most insulinomas are classified as well-differentiated endocrine tumors (WHO 1) but occasionally they belong to WHO 2 or 3. [3][4][5][6]
Non-functional[7]
Based on hormonal level determined by radioimmunoassay[8]

(previously used)

Group A
Group B

American Joint Cancer Committee (AJCC) 7th edition 2010 calssification

The staging of malignant insulinoma being a pancreatic neuroendocrine tumor may be classified into several subtypes based on American Joint Cancer Committee (AJCC) 7th edition 2010: [9][10]

Stage T N M
IA T1 N0 M0
IB T2 N0 M0
IIA T3 N0 M0
IIB T1-3 N1 M0
III T4 Any N M0
IV Any T Any N M1
AJCC 2010
T T1 <2 cm in greatest dimension
T2 >2 cm in greatest dimension
T3 Beyond the pancreas but without involvement of the superior mesenteric artery
T4 Involvement of the celiac axis or superior mesenteric artery (unresectable tumor)
N N0 No regional lymph node metastasis
N1 Regional lymph node metastasis
M M0 No distant metastasis
M1 Distant metastasis

European Neuroendocrine Tumor Society (ENETS) classification:

Being a pancreatic neuroendocrine tumor, it is also staged by European Neuroendocrine Tumor Society (ENETS) as: [9][10]

Stage T N M
I T1 N0 M0
IIA T2 N0 M0
IIB T3 N0 M0
IIIA T4 N0 M0
III B Any T N1 M0
IV Any T Any N M1
ENETS
T T1 Tumor limited to pancreas, <2 cm
T2 Tumor limited to pancreas, 2-4 cm
T3 >4cm, or invading the duodenum or common bile duct
T4 Tumor invades adjacent structures
N N0 No regional lymph node metastasis
N1 Regional lymph node metastasis
M M0 No distant metastasis
M1 Distant metastasis

WHO 2010 classification system

  • WHO classification system combined differentiation and grading characteristics to classify the belligerence of a pancreatic neuroendocrine tumor.
  • The aggressiveness of tumor was expressed in form of mitotic count and staining of a nuclear antigen called Ki-67: [11][6]
Grade of tumor Mitotic Count(Mitoses per 10 high powerfields) Expression of Ki 67
Grade 1 <2 ≤3%
Grade 2 2-10 3-20%
Grade 3 >20 >20%
  • Grade 1 and 2 tumors were classified as neuroendocrine neoplasm (NET) and grade 3 were classified as neuroendocrine carcinoma (NEC).
  • In its new 8th edition of AJCC which is planned to be published on January 1, 2018; AJCC had developed a modified ENETS (mENETS) staging classification:[9]
Stage T N M
IA T1 N0 M0
IB T2 N0 M0
IIA T3 N0 M0
IIB T1-3 N1 M0
III T4 Any N M0
IV Any T Any N M1
mENETS
T T1 Tumor limited to pancreas, <2 cm
T2 Tumor limited to pancreas, 2-4 cm
T3 >4cm, or invading the duodenum or common bile duct
T4 Tumor invades adjacent structures
N N0 No regional lymph node metastasis
N1 Regional lymph node metastasis
M M0 No distant metastasis
M1 Distant metastasis

References

  1. F. J. Service, M. M. McMahon, P. C. O'Brien & D. J. Ballard (1991). "Functioning insulinoma--incidence, recurrence, and long-term survival of patients: a 60-year study". Mayo Clinic proceedings. 66 (7): 711–719. PMID 01677058.
  2. Ahmad N, Almutawa AM, Abubacker MZ, Elzeftawy HA, Bawazir OA (2017). "Recurrent insulinoma in a 10-year-old boy with Down's syndrome". Endocrinol Diabetes Metab Case Rep. 2017. doi:10.1530/EDM-16-0155. PMC 5445445. PMID 28567298.
  3. de Herder, Wouter W.; Niederle, Bruno; Scoazec, Jean-Yves; Pauwels, Stanislas; Klöppel, Günter; Falconi, Massimo; Kwekkeboom, Dik J.; Öberg, Kjel; Eriksson, Barbro; Wiedenmann, Bertram; Rindi, Guido; O’Toole, Dermot; Ferone, Diego (2007). "Well-Differentiated Pancreatic Tumor/Carcinoma: Insulinoma". Neuroendocrinology. 84 (3): 183–188. doi:10.1159/000098010. ISSN 0028-3835.
  4. Schott M, Klöppel G, Raffel A, Saleh A, Knoefel WT, Scherbaum WA (2011). "Neuroendocrine neoplasms of the gastrointestinal tract". Dtsch Arztebl Int. 108 (18): 305–12. doi:10.3238/arztebl.2011.0305. PMC 3103981. PMID 21629514.
  5. Bosman, F. T. (2010). WHO classification of tumours of the digestive system. Lyon: International Agency for Research on Cancer. ISBN 978-9283224327.
  6. 6.0 6.1 Sun J (2017). "Pancreatic neuroendocrine tumors". Intractable Rare Dis Res. 6 (1): 21–28. doi:10.5582/irdr.2017.01007. PMC 5359348. PMID 28357177.
  7. Mittendorf EA, Liu YC, McHenry CR (2005). "Giant insulinoma: case report and review of the literature". J Clin Endocrinol Metab. 90 (1): 575–80. doi:10.1210/jc.2004-0825. PMID 15522939.
  8. Berger M, Bordi C, Cüppers HJ, Berchtold P, Gries FA, Münterfering H; et al. (1983). "Functional and morphologic characterization of human insulinomas". Diabetes. 32 (10): 921–31. PMID 6311653.
  9. 9.0 9.1 9.2 Luo G, Javed A, Strosberg JR, Jin K, Zhang Y, Liu C; et al. (2017). "Modified Staging Classification for Pancreatic Neuroendocrine Tumors on the Basis of the American Joint Committee on Cancer and European Neuroendocrine Tumor Society Systems". J Clin Oncol. 35 (3): 274–280. doi:10.1200/JCO.2016.67.8193. PMID 27646952.
  10. 10.0 10.1 Yang M, Zeng L, Zhang Y, Wang WG, Wang L, Ke NW; et al. (2015). "TNM staging of pancreatic neuroendocrine tumors: an observational analysis and comparison by both AJCC and ENETS systems from 1 single institution". Medicine (Baltimore). 94 (12): e660. doi:10.1097/MD.0000000000000660. PMC 4554009. PMID 25816036.
  11. Bosman, F. T. (2010). WHO classification of tumours of the digestive system. Lyon: International Agency for Research on Cancer. ISBN 978-9283224327.


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