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Midline central nervous system lipomas

MedGen UID:
870525
Concept ID:
C4024973
Neoplastic Process
Synonym: Midline CNS lipomas
 
HPO: HP:0006866

Conditions with this feature

Pai syndrome
MedGen UID:
371972
Concept ID:
C1835087
Disease or Syndrome
Pai syndrome is characterized by mild hypertelorism, midline cleft lip, nasal and facial polyps, pericallosal lipoma, ocular anomalies, and normal neuropsychologic development (Guion-Almeida et al., 2007).
Acromelic frontonasal dysostosis
MedGen UID:
350933
Concept ID:
C1863616
Disease or Syndrome
Verloes et al. (1992) described a rare variant of frontonasal dysplasia (see FND1, 136760), designated acromelic frontonasal dysplasia (AFND), in which similar craniofacial anomalies are associated with variable central nervous system malformations and limb defects including tibial hypoplasia/aplasia, talipes equinovarus, and preaxial polydactyly of the feet.

Professional guidelines

PubMed

Sattar TS, Bannister CM, Russell SA, Rimmer S
Eur J Pediatr Surg 1998 Dec;8 Suppl 1:31-3. doi: 10.1055/s-2008-1071249. PMID: 9926321

Recent clinical studies

Etiology

AbdollahiFakhim S, Bayazian G, Notash R
Int J Pediatr Otorhinolaryngol 2014 Apr;78(4):697-700. Epub 2014 Jan 31 doi: 10.1016/j.ijporl.2014.01.027. PMID: 24560239
Lee CS, Phi JH, Kim SK, Cho BK, Wang KC
J Neurosurg Pediatr 2009 May;3(5):407-11. doi: 10.3171/2009.1.PEDS08153. PMID: 19409020
Parmar H, Patkar D, Shah J, Maheshwari M
Clin Imaging 2003 Jan-Feb;27(1):41-3. doi: 10.1016/s0899-7071(02)00522-3. PMID: 12504320
Kieslich M, Ehlers S, Bollinger M, Jacobi G
J Child Neurol 2000 Feb;15(2):85-9. doi: 10.1177/088307380001500205. PMID: 10695892
Kobayashi H, Hayashi M, Kawano H, Handa Y, Caner HH, Ishii H
Zentralbl Neurochir 1989;50(3-4):166-7. PMID: 2642232

Diagnosis

Lederer D, Wilson B, Lefesvre P, Poorten VV, Kirkham N, Mitra D, Verellen-Dumoulin C, Devriendt K
Am J Med Genet A 2012 Nov;158A(11):2899-904. Epub 2012 Sep 17 doi: 10.1002/ajmg.a.35592. PMID: 22987662
Lee CS, Phi JH, Kim SK, Cho BK, Wang KC
J Neurosurg Pediatr 2009 May;3(5):407-11. doi: 10.3171/2009.1.PEDS08153. PMID: 19409020
Vaccarella F, Pini Prato A, Fasciolo A, Pisano M, Carlini C, Seymandi PL
Int J Oral Maxillofac Surg 2008 Nov;37(11):1059-64. Epub 2008 Jul 25 doi: 10.1016/j.ijom.2008.06.007. PMID: 18657395
Kurt G, Dogulu F, Kaymaz M, Emmez H, Onk A, Baykaner MK
Childs Nerv Syst 2002 Dec;18(12):732-4. Epub 2002 Sep 4 doi: 10.1007/s00381-002-0631-y. PMID: 12483362
Eghwrudjakpor PO, Kurisaka M, Fukuoka M, Mori K
Acta Neurochir (Wien) 1991;110(3-4):124-8. doi: 10.1007/BF01400679. PMID: 1927603

Prognosis

AbdollahiFakhim S, Bayazian G, Notash R
Int J Pediatr Otorhinolaryngol 2014 Apr;78(4):697-700. Epub 2014 Jan 31 doi: 10.1016/j.ijporl.2014.01.027. PMID: 24560239
Dehdashti AR, Abouzeid H, Momjian S, Delavelle J, Rilliet B
Childs Nerv Syst 2004 Apr;20(4):225-8; discussion 229-38. Epub 2004 Feb 17 doi: 10.1007/s00381-003-0867-1. PMID: 14986040
Parmar H, Patkar D, Shah J, Maheshwari M
Clin Imaging 2003 Jan-Feb;27(1):41-3. doi: 10.1016/s0899-7071(02)00522-3. PMID: 12504320
Sattar TS, Bannister CM, Russell SA, Rimmer S
Eur J Pediatr Surg 1998 Dec;8 Suppl 1:31-3. doi: 10.1055/s-2008-1071249. PMID: 9926321
Kobayashi H, Hayashi M, Kawano H, Handa Y, Caner HH, Ishii H
Zentralbl Neurochir 1989;50(3-4):166-7. PMID: 2642232

Clinical prediction guides

Karakaş E, Doğan MS, Çullu N, Kocatürk M, Kocatürk Ö, Karakaş Ö, Celik B, Boyaci FN
Clin Ter 2014;165(2):e134-8. doi: 10.7471/CT.2014.1696. PMID: 24770821
Fong KS, Cooper TB, Drumhiller WC, Somponpun SJ, Yang S, Ernst T, Chang L, Lozanoff S
Birth Defects Res A Clin Mol Teratol 2012 Feb;94(2):102-13. Epub 2012 Jan 13 doi: 10.1002/bdra.22878. PMID: 22246904Free PMC Article
Jabot G, Stoquart-Elsankari S, Saliou G, Toussaint P, Deramond H, Lehmann P
J Neurol 2009 Jun;256(6):851-5. Epub 2009 Mar 12 doi: 10.1007/s00415-009-5087-5. PMID: 19280105
Castori M, Rinaldi R, Bianchi A, Caponetti A, Assumma M, Grammatico P
Birth Defects Res A Clin Mol Teratol 2007 Oct;79(10):673-9. doi: 10.1002/bdra.20392. PMID: 17803202
Łebkowski WJ, Dudek H, Łebkowska U, Dziecioł J
Pol J Pathol 2000;51(3):159-63. PMID: 11247399

Recent systematic reviews

Trevisani V, Balestri E, Napoli M, Caraffi SG, Baroni MC, Peluso F, Colonna A, Iughetti L, Gargano G, Superti-Furga A, Garavelli L
Genes (Basel) 2023 Aug 31;14(9) doi: 10.3390/genes14091745. PMID: 37761885Free PMC Article

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