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Fibromatosis, gingival, 2(HGF2; GINGF2)

MedGen UID:: 344226
•Concept ID:: C1854181
•: Disease or Syndrome

Synonyms:	GINGF2; Hereditary gingival fibromatosis, 2

Monarch Initiative:	MONDO:0011563
OMIM®:	605544

Definition

Hereditary gingival fibromatosis is a benign disorder manifested by a slowly progressive enlargement of the oral gingival tissues (summary by Xiao et al., 2001). For general phenotypic information and a discussion of genetic heterogeneity of hereditary gingival fibromatosis, see GINGF (135300). [from OMIM]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVGingival overgrowth
- CROGVEpulis
  - CROGVCongenital epulis
  - CROGVGingival fibroepithelial polyp
- CROGVHereditary gingival fibromatosis
  - CROGVFibromatosis, gingival, 1
  - CROGVFibromatosis, gingival, 2
  - CROGVFibromatosis, gingival, 3
  - CROGVFibromatosis, gingival, 4
  - CROGVFibromatosis, gingival, 5
- CROGVHypertrophy of gingiva

Abnormal oral morphology
- Abnormal oral cavity morphology
  - Abnormal oral mucosa morphology
    - Abnormality of the gingiva
      - Gingival overgrowth
        Hereditary gingival fibromatosis
        Fibromatosis, gingival, 2

Professional guidelines

PubMed

Anesthetic Management of a Juvenile Hyaline Fibromatosis Patient With Trismus and Cervical Movement Limitation.

Yasuda A, Miyazawa N, Inoue E, Imai T, Shionoya Y, Nakamura K
Anesth Prog 2021 Jun 1;68(2):117-118. doi: 10.2344/anpr-68-01-04. PMID: 34185859 Free PMC Article

Hyaline fibromatosis syndrome: Clinical update and phenotype-genotype correlations.

Casas-Alba D, Martínez-Monseny A, Pino-Ramírez RM, Alsina L, Castejón E, Navarro-Vilarrubí S, Pérez-Dueñas B, Serrano M, Palau F, García-Alix A
Hum Mutat 2018 Dec;39(12):1752-1763. Epub 2018 Sep 17 doi: 10.1002/humu.23638. PMID: 30176098

Diagnosis and treatment of a hereditary gingival fibromatosis case.

Zhou M, Xu L, Meng HX
Chin J Dent Res 2011;14(2):155-8. PMID: 22319759

See all (7)

Recent clinical studies

Etiology

Surgical and prosthetic treatment in an elderly patient affected by unilateral idiopathic gingival fibromatosis: a case report.

Millet C, Rodier P, Farges JC, Labert N, Duprez JP
Gerodontology 2012 Jun;29(2):e1185-9. doi: 10.1111/j.1741-2358.2011.00564.x. PMID: 22612835

Infantile systemic hyalinosis.

Giri PP, Raushan R, Ghosh A, Pal P
Indian Pediatr 2012 Jan;49(1):62-4. PMID: 22318104

Role of smoking and type 2 diabetes in the immunobalance of advanced chronic periodontitis.

Duarte PM, Santos VR, Dos Santos FA, de Lima Pereira SA, Rodrigues DB, Napimoga MH
J Periodontol 2011 Mar;82(3):429-38. Epub 2010 Aug 23 doi: 10.1902/jop.2010.100215. PMID: 20731586

Case report: Rutherfurd syndrome associated with Marfan syndrome.

Raja TA, Albadri S, Hood C
Eur Arch Paediatr Dent 2008 Sep;9(3):138-41. doi: 10.1007/BF03262625. PMID: 18793596

Fibromatoses of the oral and paraoral soft tissues and jaws. Review of the literature and report of 12 new cases.

Vally IM, Altini M
Oral Surg Oral Med Oral Pathol 1990 Feb;69(2):191-8. doi: 10.1016/0030-4220(90)90327-o. PMID: 2406675

See all (20)

Diagnosis

Hyaline fibromatosis syndrome: a rare, yet recognizable syndrome.

Daşar T, Gönen HN, Kösemehmetoğlu K, Tekşam Ö, Boduroğlu K, Utine GE, Şimşek Kiper PÖ
Turk J Pediatr 2024 May 23;66(2):205-214. doi: 10.24953/turkjpediatr.2024.4511. PMID: 38814306

Hyaline fibromatosis syndrome: a case presenting with gingival enlargement as the only clinical manifestation and a report of two new mutations in the ANTXR2 gene.

Liu Y, Zeng X, Ding Y, Xu Y, Duan D
BMC Oral Health 2021 Oct 9;21(1):508. doi: 10.1186/s12903-021-01840-5. PMID: 34627224 Free PMC Article

Association of 17q24.2-q24.3 deletions with recognizable phenotype and short telomeres.

Hancarova M, Malikova M, Kotrova M, Drabova J, Trkova M, Sedlacek Z
Am J Med Genet A 2018 Jun;176(6):1438-1442. Epub 2018 Apr 25 doi: 10.1002/ajmg.a.38711. PMID: 29696806

Epilepsy in KCNH1-related syndromes.

Mastrangelo M, Scheffer IE, Bramswig NC, Nair LD, Myers CT, Dentici ML, Korenke GC, Schoch K, Campeau PM, White SM, Shashi V, Kansagra S, Van Essen AJ, Leuzzi V
Epileptic Disord 2016 Jun 1;18(2):123-36. doi: 10.1684/epd.2016.0830. PMID: 27267311

FAM20A mutations can cause enamel-renal syndrome (ERS).

Wang SK, Aref P, Hu Y, Milkovich RN, Simmer JP, El-Khateeb M, Daggag H, Baqain ZH, Hu JC
PLoS Genet 2013;9(2):e1003302. Epub 2013 Feb 28 doi: 10.1371/journal.pgen.1003302. PMID: 23468644 Free PMC Article

See all (55)

Therapy

Enamel Renal Gingival Syndrome in an Adolescent.

Koul R, Chengappa D, Mathur VP, Singh TA, Chopra SS
J Dent Child (Chic) 2024 Jan 15;91(1):38-42. PMID: 38671566

Epilepsy in KCNH1-related syndromes.

An update on crown lengthening. Part 1: Gingival tissue excess.

Kalsi HJ, Hussain Z, Darbar U
Dent Update 2015 Mar;42(2):144-6, 149-50, 153. doi: 10.12968/denu.2015.42.2.144. PMID: 26058228

Drug-induced gingival enlargement: biofilm control and surgical therapy with gallium-aluminum-arsenide (GaAlAs) diode laser-A 2-year follow-up.

de Oliveira Guaré R, Costa SC, Baeder F, de Souza Merli LA, Dos Santos MT
Spec Care Dentist 2010 Mar-Apr;30(2):46-52. doi: 10.1111/j.1754-4505.2009.00126.x. PMID: 20415800

Pericoronal hamartomatous lesions in the opercula of teeth delayed in eruption: an immunohistochemical study of the extracellular matrix.

Yonemochi H, Noda T, Saku T
J Oral Pathol Med 1998 Oct;27(9):441-52. doi: 10.1111/j.1600-0714.1998.tb01982.x. PMID: 9790098

See all (10)

Prognosis

Hub Genes, Possible Pathways and Predicted Drugs in Hereditary Gingival Fibromatosis by Bioinformatics Analysis.

Yang RX, Shi F, Du SN, Luo XY, Wang WQ, Yuan ZL, Chen D
Chin J Dent Res 2024 Mar 28;27(1):101-109. doi: 10.3290/j.cjdr.b5128671. PMID: 38546525

Hyaline fibromatosis syndrome: Clinical update and phenotype-genotype correlations.

Mutations in KCNH1 and ATP6V1B2 cause Zimmermann-Laband syndrome.

Kortüm F, Caputo V, Bauer CK, Stella L, Ciolfi A, Alawi M, Bocchinfuso G, Flex E, Paolacci S, Dentici ML, Grammatico P, Korenke GC, Leuzzi V, Mowat D, Nair LD, Nguyen TT, Thierry P, White SM, Dallapiccola B, Pizzuti A, Campeau PM, Tartaglia M, Kutsche K
Nat Genet 2015 Jun;47(6):661-7. Epub 2015 Apr 27 doi: 10.1038/ng.3282. PMID: 25915598

Juvenile hyaline fibromatosis: a case report.

Mestiri S, Labaied N, Mama N, Ayadi A, Ladib M, Sriha B, Krifa H, Mokni M
Pathologica 2014 Jun;106(2):70-2. PMID: 25291871

Surgical and prosthetic treatment in an elderly patient affected by unilateral idiopathic gingival fibromatosis: a case report.

Millet C, Rodier P, Farges JC, Labert N, Duprez JP
Gerodontology 2012 Jun;29(2):e1185-9. doi: 10.1111/j.1741-2358.2011.00564.x. PMID: 22612835

See all (27)

Clinical prediction guides

Hub Genes, Possible Pathways and Predicted Drugs in Hereditary Gingival Fibromatosis by Bioinformatics Analysis.

Yang RX, Shi F, Du SN, Luo XY, Wang WQ, Yuan ZL, Chen D
Chin J Dent Res 2024 Mar 28;27(1):101-109. doi: 10.3290/j.cjdr.b5128671. PMID: 38546525

Clinics and genetic background of hereditary gingival fibromatosis.

Strzelec K, Dziedzic A, Łazarz-Bartyzel K, Grabiec AM, Gutmajster E, Kaczmarzyk T, Plakwicz P, Gawron K
Orphanet J Rare Dis 2021 Nov 24;16(1):492. doi: 10.1186/s13023-021-02104-9. PMID: 34819125 Free PMC Article

Association of 17q24.2-q24.3 deletions with recognizable phenotype and short telomeres.

Hancarova M, Malikova M, Kotrova M, Drabova J, Trkova M, Sedlacek Z
Am J Med Genet A 2018 Jun;176(6):1438-1442. Epub 2018 Apr 25 doi: 10.1002/ajmg.a.38711. PMID: 29696806

Epilepsy in KCNH1-related syndromes.

Juvenile hyaline fibromatosis: a case report.

Mestiri S, Labaied N, Mama N, Ayadi A, Ladib M, Sriha B, Krifa H, Mokni M
Pathologica 2014 Jun;106(2):70-2. PMID: 25291871

See all (24)

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Fibromatosis, gingival, 2(HGF2; GINGF2)

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Supplemental Content

Table of contents

Clinical resources

Practice guidelines

Molecular resources

Consumer resources

Reviews

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