Multiple endocrine neoplasia

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C-cell hyperplasia, as may be seen in the context of MEN 2A or MEN 2B. H&E stain.

Multiple endocrine neoplasia, abbreviated MEN, refers to collection of different genetic abnormalities that lead to endocrine neoplasms.

Simple

MEN 1

Memory device: 3 Ps.

MEN 2A (II)

Notes - memory device:

  • Both MEN 1 and MEN 2A have parathyroid adenomas.
  • Each has additional neoplasms below and above the diaphragm.

MEN 2B (III)

Details

MEN 1

Gene:

Characteristics:[2]

  • Endocrine tumours usually lead to most of the morbidity.
  • DIPNECH has been described in the context of multiple endocrine neoplasia 1.[4]

MEN 2A

Gene:

Characteristics:[2]

  • Medullary thyroid carcinoma - in almost 100%.

Treatment:

  • Patients are advised to have prophylactic thyroidectomy.

Images:

Trivia:

MEN 2B

Gene:

Characteristics:

Treatment:

  • Patients are advised to have prophylactic thyroidectomy.

References

  1. 1.0 1.1 Online 'Mendelian Inheritance in Man' (OMIM) 131100
  2. 2.0 2.1 2.2 2.3 2.4 Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1162. ISBN 978-1416031215. }}
  3. Zollinger RM, Ellison EH (1955). "Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas". Ann. Surg. 142 (4): 709–23; discussion, 724–8. doi:10.1097/00000658-195510000-00015. PMC 1465210. PMID 13259432. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1465210/.
  4. Davies, SJ.; Gosney, JR.; Hansell, DM.; Wells, AU.; du Bois, RM.; Burke, MM.; Sheppard, MN.; Nicholson, AG. (Mar 2007). "Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognised spectrum of disease.". Thorax 62 (3): 248-52. doi:10.1136/thx.2006.063065. PMID 17099078.
  5. Online 'Mendelian Inheritance in Man' (OMIM) 171400
  6. 6.0 6.1 Online 'Mendelian Inheritance in Man' (OMIM) 162300
  7. Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1160. ISBN 978-1416031215.
  8. Haraguchi M, Kinoshita H, Koori M, et al. (2007). "Multiple rectal carcinoids with diffuse ganglioneuromatosis". World J Surg Oncol 5: 19. doi:10.1186/1477-7819-5-19. PMC 1805501. PMID 17306015. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1805501/.
  9. Iacobuzio-Donahue, Christine A.; Montgomery, Elizabeth A. (2005). Gastrointestinal and Liver Pathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 215. ISBN 978-0443066573.