Prolactinoma pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Faizan Sheraz, M.D. [2]
Overview
Prolactinoma may occur as part of a hereditary disorder called multiple endocrine neoplasia type 1 (MEN 1). A minority of prolactinomas are associated with Carney complex, McCune-Albright Syndrome, MEN like syndrome ( CKDN1B loss of function).[1]
Pathophysiology
Prolactinoma may occur as part of a hereditary disorder called multiple endocrine neoplasia type 1 (MEN 1). A minority of prolactinomas are associated with:
- Multiple endocrine neoplasia type I (MEN I)
- Carney complex
- McCune-Albright Syndrome
- MEN like syndrome ( CKDN1B loss of function)[2]
Familial pituitary adenomas
A pituitary adenoma may be part of a familial syndrome:[3][4]
Syndrome | Gene | Notes |
---|---|---|
Multiple endocrine neoplasia I | MEN1 | characterized by the 3 Ps: pituitary adenoma, parathyroid adenoma, pancreatic neuroendocrine tumour |
MEN-1-like syndrome | CDKN1B[5] | also known as Multiple endocrine neoplasia IV [5] |
Carney syndrome | PRKAR1A | other findings (mnemonic NAME): nevi, atrial myxoma, myxoid neurofibroma, ephelides (freckles) |
Isolated pituitary adenoma[6] | AIP | classically GH-producing adenoma - leads to acromegaly |
Microscopic
Features:[7]
- Loss of fibrous stroma.
- The cells of a normal (anterior) pituitary are nested.
Notes:
- Smears very well.[8]
Images
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Pituitary adenoma - non-functioning. (WC/KGH)
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Extensive interstitial and perivascular fibrosis in a pituitary adenoma (WC/jensflorian)
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Pituitary adenoma - PRL producing, HE. Note the basophilic appearance of the cells (WC/jensflorian)
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Pituitary adenoma - PRL producing, HE. Extensive regressive changes after after dopamine agonist treatment (WC/jensflorian)
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Pituitary adenoma - PRL producing, Prolactin IHC (WC/jensflorian)
Stains
- Reticulin - loss of reticulin between tumour cells.
References
- ↑ http://radiopaedia.org/articles/pituitary-adenoma
- ↑ http://radiopaedia.org/articles/pituitary-adenoma
- ↑ Elston, MS.; McDonald, KL.; Clifton-Bligh, RJ.; Robinson, BG. (2009). "Familial pituitary tumor syndromes". Nat Rev Endocrinol. 5 (8): 453–61. doi:10.1038/nrendo.2009.126. PMID 19564887. Unknown parameter
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ignored (help) - ↑ Template:Ref PCPBoD8
- ↑ 5.0 5.1 Online Mendelian Inheritance in Man (OMIM) 600778
- ↑ Korbonits, M.; Storr, H.; Kumar, AV. (2012). "Familial pituitary adenomas - Who should be tested for AIP mutations?". Clin Endocrinol (Oxf). doi:10.1111/j.1365-2265.2012.04445.x. PMID 22612670. Unknown parameter
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ignored (help) - ↑ Template:Ref PSNP
- ↑ MUN. 24 November 2010.