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Pituitary carcinoma

MedGen UID:
91096
•Concept ID:
C0346300
•
Neoplastic Process
Synonyms: Cancer of Pituitary; Cancer of the Pituitary; Cancer, Pituitary; Cancers, Pituitary; Carcinoma, Pituitary; Carcinomas, Pituitary; Pituitary Cancer; Pituitary Cancers; Pituitary Carcinoma; Pituitary Carcinomas
SNOMED CT: Pituitary carcinoma (128665000); Pituitary carcinoma (254955001)
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
•Concept ID:
CN307042
•
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
See: This record
Unknown inheritance (Orphanet)
 
HPO: HP:0011763
Monarch Initiative: MONDO:0017582
Orphanet: ORPHA300385

Definition

A pituitary tumor with subarachnoid, brain, or systemic metastasis. The diagnosis of a pituitary carcinoma requires evidence of metastatic disease, either outside the central nervous system (CNS) or as separate noncontiguous foci within the CNS. [from HPO]

Professional guidelines

PubMed

Revised European Society of Endocrinology Clinical Practice Guideline for the management of aggressive pituitary tumours and pituitary carcinomas.
Raverot G, Burman P, Abreu AP, Heaney AP, van Hulsteijn L, Lin AL, Marcus H, McCormack A, Minniti G, Petersenn S, Popovic V, Theodoropoulou M, Trouillas J, Dekkers OM
Eur J Endocrinol 2025 May 30;192(6):R45-R78. doi: 10.1093/ejendo/lvaf100. PMID: 40506054
European Society of Endocrinology Clinical Practice Guidelines for the management of aggressive pituitary tumours and carcinomas.
Raverot G, Burman P, McCormack A, Heaney A, Petersenn S, Popovic V, Trouillas J, Dekkers OM; European Society of Endocrinology
Eur J Endocrinol 2018 Jan;178(1):G1-G24. Epub 2017 Oct 18 doi: 10.1530/EJE-17-0796. PMID: 29046323
Pituitary carcinoma: diagnosis and treatment.
Lopes MB, Scheithauer BW, Schiff D
Endocrine 2005 Oct;28(1):115-21. doi: 10.1385/ENDO:28:1:115. PMID: 16311418

Recent clinical studies

Etiology

The 2022 WHO classification of tumors of the pituitary gland: An update on aggressive and metastatic pituitary neuroendocrine tumors.
Casar-Borota O, Burman P, Lopes MB
Brain Pathol 2025 Jan;35(1):e13302. Epub 2024 Sep 1 doi: 10.1111/bpa.13302. PMID: 39218431Free PMC Article
Genome-wide methylation profiling differentiates benign from aggressive and metastatic pituitary neuroendocrine tumors.
Jotanovic J, Boldt HB, Burton M, Andersen MS, Bengtsson D, Bontell TO, Ekman B, Engström BE, Feldt-Rasmussen U, Heck A, Jakovcevic A, Jørgensen JOL, Kraljevic I, Kunicki J, Lindsay JR, Losa M, Loughrey PB, Maiter D, Maksymowicz M, Manojlovic-Gacic E, Pahnke J, Petersenn S, Petersson M, Popovic V, Ragnarsson O, Rasmussen ÅK, Reisz Z, Saeger W, Schalin-Jäntti C, Scheie D, Terreni MR, Tynninen O, Whitelaw B, Burman P, Casar-Borota O
Acta Neuropathol 2024 Nov 23;148(1):68. doi: 10.1007/s00401-024-02836-5. PMID: 39580368Free PMC Article
Overview of the 2022 WHO Classification of Pituitary Tumors.
Asa SL, Mete O, Perry A, Osamura RY
Endocr Pathol 2022 Mar;33(1):6-26. Epub 2022 Mar 15 doi: 10.1007/s12022-022-09703-7. PMID: 35291028
Invasive, atypical and aggressive pituitary adenomas and carcinomas.
Sav A, Rotondo F, Syro LV, Di Ieva A, Cusimano MD, Kovacs K
Endocrinol Metab Clin North Am 2015 Mar;44(1):99-104. Epub 2014 Nov 4 doi: 10.1016/j.ecl.2014.10.008. PMID: 25732646
Non-functioning pituitary tumors: 2012 update.
Cámara Gómez R
Endocrinol Nutr 2014 Mar;61(3):160-70. Epub 2013 Sep 12 doi: 10.1016/j.endonu.2013.04.009. PMID: 24035732

Diagnosis

Revised European Society of Endocrinology Clinical Practice Guideline for the management of aggressive pituitary tumours and pituitary carcinomas.
Raverot G, Burman P, Abreu AP, Heaney AP, van Hulsteijn L, Lin AL, Marcus H, McCormack A, Minniti G, Petersenn S, Popovic V, Theodoropoulou M, Trouillas J, Dekkers OM
Eur J Endocrinol 2025 May 30;192(6):R45-R78. doi: 10.1093/ejendo/lvaf100. PMID: 40506054
The 2022 WHO classification of tumors of the pituitary gland: An update on aggressive and metastatic pituitary neuroendocrine tumors.
Casar-Borota O, Burman P, Lopes MB
Brain Pathol 2025 Jan;35(1):e13302. Epub 2024 Sep 1 doi: 10.1111/bpa.13302. PMID: 39218431Free PMC Article
Overview of the 2022 WHO Classification of Pituitary Tumors.
Asa SL, Mete O, Perry A, Osamura RY
Endocr Pathol 2022 Mar;33(1):6-26. Epub 2022 Mar 15 doi: 10.1007/s12022-022-09703-7. PMID: 35291028
European Society of Endocrinology Clinical Practice Guidelines for the management of aggressive pituitary tumours and carcinomas.
Raverot G, Burman P, McCormack A, Heaney A, Petersenn S, Popovic V, Trouillas J, Dekkers OM; European Society of Endocrinology
Eur J Endocrinol 2018 Jan;178(1):G1-G24. Epub 2017 Oct 18 doi: 10.1530/EJE-17-0796. PMID: 29046323
Pituitary carcinoma.
Sansur CA, Oldfield EH
Semin Oncol 2010 Dec;37(6):591-3. doi: 10.1053/j.seminoncol.2010.10.012. PMID: 21167378

Therapy

Aggressive corticotroph tumors and carcinomas.
Lasolle H, Vasiljevic A, Jouanneau E, Ilie MD, Raverot G
J Neuroendocrinol 2022 Aug;34(8):e13169. Epub 2022 Aug 18 doi: 10.1111/jne.13169. PMID: 35979732Free PMC Article
Immune-checkpoint inhibitors in pituitary malignancies.
Di Nunno V, Franceschi E, Tosoni A, Gatto L, Maggio I, Lodi R, Bartolini S, Brandes AA
Anticancer Drugs 2022 Jan 1;33(1):e28-e35. doi: 10.1097/CAD.0000000000001157. PMID: 34348358
Aggressive pituitary tumours and pituitary carcinomas.
Raverot G, Ilie MD, Lasolle H, Amodru V, Trouillas J, Castinetti F, Brue T
Nat Rev Endocrinol 2021 Nov;17(11):671-684. Epub 2021 Sep 7 doi: 10.1038/s41574-021-00550-w. PMID: 34493834
Aggressive prolactinomas: how to manage?
Lasolle H, Ilie MD, Raverot G
Pituitary 2020 Feb;23(1):70-77. doi: 10.1007/s11102-019-01000-7. PMID: 31617128
Thyrotropin-secreting pituitary carcinoma.
Mixson AJ, Friedman TC, Katz DA, Feuerstein IM, Taubenberger JK, Colandrea JM, Doppman JL, Oldfield EH, Weintraub BD
J Clin Endocrinol Metab 1993 Feb;76(2):529-33. doi: 10.1210/jcem.76.2.8432799. PMID: 8432799

Prognosis

Revised European Society of Endocrinology Clinical Practice Guideline for the management of aggressive pituitary tumours and pituitary carcinomas.
Raverot G, Burman P, Abreu AP, Heaney AP, van Hulsteijn L, Lin AL, Marcus H, McCormack A, Minniti G, Petersenn S, Popovic V, Theodoropoulou M, Trouillas J, Dekkers OM
Eur J Endocrinol 2025 May 30;192(6):R45-R78. doi: 10.1093/ejendo/lvaf100. PMID: 40506054
The 2022 WHO classification of tumors of the pituitary gland: An update on aggressive and metastatic pituitary neuroendocrine tumors.
Casar-Borota O, Burman P, Lopes MB
Brain Pathol 2025 Jan;35(1):e13302. Epub 2024 Sep 1 doi: 10.1111/bpa.13302. PMID: 39218431Free PMC Article
Aggressive corticotroph tumors and carcinomas.
Lasolle H, Vasiljevic A, Jouanneau E, Ilie MD, Raverot G
J Neuroendocrinol 2022 Aug;34(8):e13169. Epub 2022 Aug 18 doi: 10.1111/jne.13169. PMID: 35979732Free PMC Article
Aggressive Pituitary Adenomas and Carcinomas.
Ilie MD, Jouanneau E, Raverot G
Endocrinol Metab Clin North Am 2020 Sep;49(3):505-515. Epub 2020 Jul 8 doi: 10.1016/j.ecl.2020.05.008. PMID: 32741485
Pituitary carcinomas.
Colao A, Ochoa AS, Auriemma RS, Faggiano A, Pivonello R, Lombardi G
Front Horm Res 2010;38:94-108. Epub 2010 Jul 5 doi: 10.1159/000318499. PMID: 20616500

Clinical prediction guides

Therapeutic targeting of the pituitary tumor microenvironment.
Ilie MD, De Alcubierre D, Carretti AL, Jouanneau E, Raverot G
Pharmacol Ther 2023 Oct;250:108506. Epub 2023 Aug 9 doi: 10.1016/j.pharmthera.2023.108506. PMID: 37562699
Pituitary carcinoma - case series and review of the literature.
Du Four S, Van Der Veken J, Duerinck J, Vermeulen E, Andreescu CE, Bruneau M, Neyns B, Velthoven V, Velkeniers B
Front Endocrinol (Lausanne) 2022;13:968692. Epub 2022 Sep 8 doi: 10.3389/fendo.2022.968692. PMID: 36157469Free PMC Article
Aggressive pituitary tumours and pituitary carcinomas.
Raverot G, Ilie MD, Lasolle H, Amodru V, Trouillas J, Castinetti F, Brue T
Nat Rev Endocrinol 2021 Nov;17(11):671-684. Epub 2021 Sep 7 doi: 10.1038/s41574-021-00550-w. PMID: 34493834
Aggressive Pituitary Adenomas and Carcinomas.
Ilie MD, Jouanneau E, Raverot G
Endocrinol Metab Clin North Am 2020 Sep;49(3):505-515. Epub 2020 Jul 8 doi: 10.1016/j.ecl.2020.05.008. PMID: 32741485
Invasive, atypical and aggressive pituitary adenomas and carcinomas.
Sav A, Rotondo F, Syro LV, Di Ieva A, Cusimano MD, Kovacs K
Endocrinol Metab Clin North Am 2015 Mar;44(1):99-104. Epub 2014 Nov 4 doi: 10.1016/j.ecl.2014.10.008. PMID: 25732646

Recent systematic reviews

Outcome and prognostic factors for pituitary carcinomas: lessons from a systematic review.
Raymond P, Raverot G, Ilie MD
Endocr Relat Cancer 2023 May 1;30(5) Epub 2023 Mar 31 doi: 10.1530/ERC-22-0338. PMID: 36852675
Immunotherapy in aggressive pituitary tumors and carcinomas: a systematic review.
Ilie MD, Vasiljevic A, Jouanneau E, Raverot G
Endocr Relat Cancer 2022 May 27;29(7):415-426. doi: 10.1530/ERC-22-0037. PMID: 35521777
Distant Pituitary Adenoma Spread: A Systematic Review and Report of 2 Cases.
Azizkhanian I, El Ahmadieh TY, Palmisciano P, Abou-Mrad Z, Daoud EV, Essibayi MA, Connors S, Aoun SG, Kim J, Hatanpaa KJ, Garzon-Muvdi T, Barnett SL, Patel T, Raisanen JM, Mickey BE
Oper Neurosurg 2022 Mar 1;22(3):131-143. doi: 10.1227/ONS.0000000000000089. PMID: 35030115
Immune-checkpoint inhibitors in pituitary malignancies.
Di Nunno V, Franceschi E, Tosoni A, Gatto L, Maggio I, Lodi R, Bartolini S, Brandes AA
Anticancer Drugs 2022 Jan 1;33(1):e28-e35. doi: 10.1097/CAD.0000000000001157. PMID: 34348358
Genetic aspects of pituitary carcinoma: A systematic review.
Yang Z, Zhang T, Gao H
Medicine (Baltimore) 2016 Nov;95(47):e5268. doi: 10.1097/MD.0000000000005268. PMID: 27893664Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

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