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Craniopharyngioma

MedGen UID:
41339
Concept ID:
C0010276
Neoplastic Process
Synonyms: Adamantinomatous tumor; Craniopharyngeal duct tumor; Dysodontogenic epithelial tumor; Rathke's pouch tumor
SNOMED CT: Craniopharyngioma (189179009); Craniopharyngioma (40009002); Rathke's pouch tumor (40009002)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
HPO: HP:0030062
Monarch Initiative: MONDO:0018907
Orphanet: ORPHA54595

Definition

A benign pituitary-region neoplasm that originates from Rathke's pouch. Craniopharyngiomas are benign slow growing tumors that are located within the sellar and para sellar region of the central nervous system. [from HPO]

Conditions with this feature

Silver-Russell syndrome 1
MedGen UID:
1718472
Concept ID:
C5393125
Disease or Syndrome
Silver-Russell Syndrome (SRS) is typically characterized by asymmetric gestational growth restriction resulting in affected individuals being born small for gestational age, with relative macrocephaly at birth (head circumference =1.5 SD above birth weight and/or length), prominent forehead usually with frontal bossing, and frequently body asymmetry. This is followed by postnatal growth failure, and in some cases progressive limb length discrepancy and feeding difficulties. Additional clinical features include triangular facies, fifth-finger clinodactyly, and micrognathia with narrow chin. Except for the limb length asymmetry, the growth failure is proportionate and head growth normal. The average adult height in untreated individuals is ~3.1±1.4 SD below the mean. The Netchine-Harbison Clinical Scoring System (NH-CSS) is a sensitive diagnostic scoring system. Clinical diagnosis can be established in an individual who meets at least four of the NH-CSS clinical criteria – prominent forehead/frontal bossing and relative macrocephaly at birth plus two additional findings – and in whom other disorders have been ruled out.

Professional guidelines

PubMed

Diaz MJ, Kwak SH, Root KT, Fadil A, Nguyen A, Ladehoff L, Batchu S, Lucke-Wold B
Front Biosci (Landmark Ed) 2022 Dec 21;27(12):328. doi: 10.31083/j.fbl2712328. PMID: 36624954
Müller HL
Neuroendocrinology 2020;110(9-10):753-766. Epub 2019 Nov 4 doi: 10.1159/000504512. PMID: 31678973
Di Iorgi N, Napoli F, Allegri AE, Olivieri I, Bertelli E, Gallizia A, Rossi A, Maghnie M
Horm Res Paediatr 2012;77(2):69-84. Epub 2012 Mar 16 doi: 10.1159/000336333. PMID: 22433947

Recent clinical studies

Etiology

Salmon MK, Kshirsagar RS, Eide JG
Curr Opin Otolaryngol Head Neck Surg 2023 Feb 1;31(1):45-52. Epub 2022 Nov 14 doi: 10.1097/MOO.0000000000000856. PMID: 36730658
Madsen PJ, Lang SS, Adappa ND, Palmer JN, Storm PB
Otolaryngol Clin North Am 2022 Apr;55(2):477-491. Epub 2022 Mar 4 doi: 10.1016/j.otc.2021.12.017. PMID: 35256170
Henderson F Jr, Schwartz TH
J Neurooncol 2022 Jan;156(1):97-108. Epub 2021 Nov 22 doi: 10.1007/s11060-021-03906-4. PMID: 34807341
Kiliç M, Can SM, Özdemir B, Tanik C
J Craniofac Surg 2019 Mar/Apr;30(2):e178-e183. doi: 10.1097/SCS.0000000000005136. PMID: 30640850
Iughetti L, Bruzzi P
Ital J Pediatr 2011 Aug 16;37:38. doi: 10.1186/1824-7288-37-38. PMID: 21846381Free PMC Article

Diagnosis

Otte A, Müller HL
J Clin Endocrinol Metab 2021 Sep 27;106(10):e3820-e3836. doi: 10.1210/clinem/dgab397. PMID: 34086900
Müller HL
Neuroendocrinology 2020;110(9-10):753-766. Epub 2019 Nov 4 doi: 10.1159/000504512. PMID: 31678973
Müller HL, Merchant TE, Warmuth-Metz M, Martinez-Barbera JP, Puget S
Nat Rev Dis Primers 2019 Nov 7;5(1):75. doi: 10.1038/s41572-019-0125-9. PMID: 31699993
Drapeau A, Walz PC, Eide JG, Rugino AJ, Shaikhouni A, Mohyeldin A, Carrau RL, Prevedello DM
Childs Nerv Syst 2019 Nov;35(11):2133-2145. Epub 2019 Aug 5 doi: 10.1007/s00381-019-04300-2. PMID: 31385085
Müller HL
Endocr Rev 2014 Jun;35(3):513-43. Epub 2014 Jan 27 doi: 10.1210/er.2013-1115. PMID: 24467716

Therapy

Merchant TE, Hoehn ME, Khan RB, Sabin ND, Klimo P, Boop FA, Wu S, Li Y, Burghen EA, Jurbergs N, Sandler ES, Aldana PR, Indelicato DJ, Conklin HM
Lancet Oncol 2023 May;24(5):523-534. Epub 2023 Apr 18 doi: 10.1016/S1470-2045(23)00146-8. PMID: 37084748Free PMC Article
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
Müller HL
Endocrinol Metab Clin North Am 2020 Sep;49(3):533-552. Epub 2020 Jul 15 doi: 10.1016/j.ecl.2020.05.009. PMID: 32741487
Müller HL
Handb Clin Neurol 2014;124:235-53. doi: 10.1016/B978-0-444-59602-4.00016-2. PMID: 25248591
Müller HL
Endocr Rev 2014 Jun;35(3):513-43. Epub 2014 Jan 27 doi: 10.1210/er.2013-1115. PMID: 24467716

Prognosis

Korula S, Beryl S, Kamath V, Mathew SS, Joseph V
Indian J Pediatr 2022 Feb;89(2):181-183. Epub 2021 Nov 29 doi: 10.1007/s12098-021-03990-4. PMID: 34843061
Otte A, Müller HL
J Clin Endocrinol Metab 2021 Sep 27;106(10):e3820-e3836. doi: 10.1210/clinem/dgab397. PMID: 34086900
Jensterle M, Jazbinsek S, Bosnjak R, Popovic M, Zaletel LZ, Vesnaver TV, Kotnik BF, Kotnik P
Radiol Oncol 2019 Oct 25;53(4):388-396. doi: 10.2478/raon-2019-0036. PMID: 31652121Free PMC Article
Yu X, Liu R, Wang Y, Wang H, Zhao H, Wu Z
Clin Neurol Neurosurg 2012 Feb;114(2):112-9. Epub 2011 Oct 21 doi: 10.1016/j.clineuro.2011.09.010. PMID: 22018920
Iughetti L, Bruzzi P
Ital J Pediatr 2011 Aug 16;37:38. doi: 10.1186/1824-7288-37-38. PMID: 21846381Free PMC Article

Clinical prediction guides

Nguyen Quoc A, Beccaria K, González Briceño L, Pinto G, Samara-Boustani D, Stoupa A, Beltrand J, Besançon A, Thalassinos C, Puget S, Blauwblomme T, Alapetite C, Bolle S, Doz F, Grill J, Dufour C, Bourdeaut F, Abbou S, Guerrini-Rousseau L, Leruste A, Brabant S, Cavadias I, Viaud M, Boddaert N, Polak M, Kariyawasam D
J Clin Endocrinol Metab 2023 Jul 14;108(8):1929-1936. doi: 10.1210/clinem/dgad079. PMID: 36794424
Otte A, Müller HL
J Clin Endocrinol Metab 2021 Sep 27;106(10):e3820-e3836. doi: 10.1210/clinem/dgab397. PMID: 34086900
Martinez-Barbera JP, Andoniadou CL
Neuroendocrinology 2020;110(9-10):797-804. Epub 2020 Mar 4 doi: 10.1159/000506904. PMID: 32126562
Yu X, Liu R, Wang Y, Wang H, Zhao H, Wu Z
Clin Neurol Neurosurg 2012 Feb;114(2):112-9. Epub 2011 Oct 21 doi: 10.1016/j.clineuro.2011.09.010. PMID: 22018920
Karavitaki N, Cudlip S, Adams CB, Wass JA
Endocr Rev 2006 Jun;27(4):371-97. Epub 2006 Mar 16 doi: 10.1210/er.2006-0002. PMID: 16543382

Recent systematic reviews

Pang JC, Chung DD, Wang J, Abiri A, Lien BV, Himstead AS, Ovakimyan A, Kim MG, Hsu FPK, Kuan EC
Neurosurgery 2023 Jun 1;92(6):1112-1129. Epub 2023 Jan 13 doi: 10.1227/neu.0000000000002346. PMID: 36639856
Castle-Kirszbaum M, Shi MDY, Goldschlager T
World Neurosurg 2022 Aug;164:424-435.e2. Epub 2022 May 14 doi: 10.1016/j.wneu.2022.05.038. PMID: 35580780
Guimarães MM, Cardeal DD, Teixeira MJ, Lucio JEDC, Sanders FH, Kuromoto RK, Matushita H
Childs Nerv Syst 2022 Feb;38(2):253-262. Epub 2021 Oct 7 doi: 10.1007/s00381-021-05378-3. PMID: 34618201
Dandurand C, Sepehry AA, Asadi Lari MH, Akagami R, Gooderham P
Neurosurgery 2018 Oct 1;83(4):631-641. doi: 10.1093/neuros/nyx570. PMID: 29267973
Jasim S, Alahdab F, Ahmed AT, Tamhane S, Prokop LJ, Nippoldt TB, Murad MH
Endocrine 2017 Apr;56(1):33-42. Epub 2016 Nov 5 doi: 10.1007/s12020-016-1159-3. PMID: 27817141

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