U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Infantile myofibromatosis(IMF)

MedGen UID:
140933
Concept ID:
C0432284
Neoplastic Process
Synonyms: Congenital generalized fibromatosis; IMF
SNOMED CT: Infantile myofibromatosis (254146000); Congenital generalized fibromatosis (254146000); Infantile myofibromatosis (1285183007); Congenital generalized fibromatosis (1285183007)
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
Concept ID:
C0441748
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in individuals with two pathogenic alleles, either homozygotes (two copies of the same mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Related genes: PDGFRB, NOTCH3
 
Monarch Initiative: MONDO:0016824
OMIM®: 228550
OMIM® Phenotypic series: PS228550
Orphanet: ORPHA2591

Definition

Infantile myofibromatosis is a rare mesenchymal disorder characterized by the onset of nodules in the skin, striated muscles, bones, and, more rarely, visceral organs. Subcutaneous or soft tissue nodules commonly involve the skin of the head, neck, and trunk. Skeletal and muscular lesions occur in about 50% of patients. Lesions may be solitary or multicentric, and they may be present at birth or become apparent in early infancy or occasionally in adult life (summary by Arcangeli and Calista, 2006). Genetic Heterogeneity of Infantile Myofibromatosis See also IMF2 (615293), caused by mutation in the NOTCH3 gene (600276). [from OMIM]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
Follow this link to review classifications for Infantile myofibromatosis in Orphanet.

Professional guidelines

PubMed

Manisterski M, Benish M, Levin D, Shiran SI, Sher O, Gortzak Y, Elhasid R
Pediatr Blood Cancer 2021 Feb;68(2):e28769. Epub 2020 Oct 16 doi: 10.1002/pbc.28769. PMID: 33063933
Pekar-Zlotin M, Levinsohn-Tavor O, Livneh A, Sher O, Melcer Y, Maymon R
Obstet Gynecol Surv 2019 Oct;74(10):607-610. doi: 10.1097/OGX.0000000000000717. PMID: 31670833
Parham DM
F1000Res 2018;7 Epub 2018 Dec 20 doi: 10.12688/f1000research.16236.1. PMID: 30613391Free PMC Article

Recent clinical studies

Etiology

Manisterski M, Benish M, Levin D, Shiran SI, Sher O, Gortzak Y, Elhasid R
Pediatr Blood Cancer 2021 Feb;68(2):e28769. Epub 2020 Oct 16 doi: 10.1002/pbc.28769. PMID: 33063933
Parham DM
F1000Res 2018;7 Epub 2018 Dec 20 doi: 10.12688/f1000research.16236.1. PMID: 30613391Free PMC Article
Weaver MS, Navid F, Huppmann A, Meany H, Angiolillo A
J Pediatr Hematol Oncol 2015 Apr;37(3):237-41. doi: 10.1097/MPH.0000000000000286. PMID: 25389917
Parker RK, Mallory SB, Baker GF
Pediatr Dermatol 1991 Jun;8(2):129-32. doi: 10.1111/j.1525-1470.1991.tb00302.x. PMID: 1923981
Chung EB, Enzinger FM
Cancer 1981 Oct 15;48(8):1807-18. doi: 10.1002/1097-0142(19811015)48:8<1807::aid-cncr2820480818>3.0.co;2-g. PMID: 7284977

Diagnosis

Zhao G, Zhu M, Qin C, Liu X, Zhao X
J Pediatr Hematol Oncol 2020 Nov;42(8):495-498. doi: 10.1097/MPH.0000000000001603. PMID: 31764512
Pekar-Zlotin M, Levinsohn-Tavor O, Livneh A, Sher O, Melcer Y, Maymon R
Obstet Gynecol Surv 2019 Oct;74(10):607-610. doi: 10.1097/OGX.0000000000000717. PMID: 31670833
Larralde M, Ferrari B, Martinez JP, Barbieri MAF, Méndez JH, Casas J
An Bras Dermatol 2017 Nov-Dec;92(6):854-857. doi: 10.1590/abd1806-4841.20175001. PMID: 29364448Free PMC Article
Gopal M, Chahal G, Al-Rifai Z, Eradi B, Ninan G, Nour S
Pediatr Surg Int 2008 Mar;24(3):287-91. Epub 2007 Dec 12 doi: 10.1007/s00383-007-2091-7. PMID: 18074145
Johnson GL, Baisden BL, Fishman EK
Skeletal Radiol 1997 Oct;26(10):611-4. doi: 10.1007/s002560050295. PMID: 9361358

Therapy

Gehle S, Quinsey C, McShane D, Thompson P, Quezada G
J Pediatr Hematol Oncol 2023 Oct 1;45(7):e921-e923. Epub 2023 Jul 13 doi: 10.1097/MPH.0000000000002717. PMID: 37526359
Manisterski M, Benish M, Levin D, Shiran SI, Sher O, Gortzak Y, Elhasid R
Pediatr Blood Cancer 2021 Feb;68(2):e28769. Epub 2020 Oct 16 doi: 10.1002/pbc.28769. PMID: 33063933
Bidadi B, Watson A, Weigel B, Oliveira A, Kirkham J, Arndt C
Pediatr Blood Cancer 2020 Jun;67(6):e28288. Epub 2020 Apr 19 doi: 10.1002/pbc.28288. PMID: 32307894
Larralde M, Ferrari B, Martinez JP, Barbieri MAF, Méndez JH, Casas J
An Bras Dermatol 2017 Nov-Dec;92(6):854-857. doi: 10.1590/abd1806-4841.20175001. PMID: 29364448Free PMC Article
Weaver MS, Navid F, Huppmann A, Meany H, Angiolillo A
J Pediatr Hematol Oncol 2015 Apr;37(3):237-41. doi: 10.1097/MPH.0000000000000286. PMID: 25389917

Prognosis

Zhao G, Zhu M, Qin C, Liu X, Zhao X
J Pediatr Hematol Oncol 2020 Nov;42(8):495-498. doi: 10.1097/MPH.0000000000001603. PMID: 31764512
Gopal M, Chahal G, Al-Rifai Z, Eradi B, Ninan G, Nour S
Pediatr Surg Int 2008 Mar;24(3):287-91. Epub 2007 Dec 12 doi: 10.1007/s00383-007-2091-7. PMID: 18074145
Linder JS, Harris GJ, Segura AD
Arch Ophthalmol 1996 Feb;114(2):219-22. doi: 10.1001/archopht.1996.01100130213022. PMID: 8573030
Kim KH, Park YN, Joo HJ, Lee KG, Park C, Kim DS
Yonsei Med J 1989 Dec;30(4):376-82. doi: 10.3349/ymj.1989.30.4.376. PMID: 2626841
Chung EB, Enzinger FM
Cancer 1981 Oct 15;48(8):1807-18. doi: 10.1002/1097-0142(19811015)48:8<1807::aid-cncr2820480818>3.0.co;2-g. PMID: 7284977

Clinical prediction guides

Dachy G, Fraitag S, Boulouadnine B, Cordi S, Demoulin JB
J Cell Mol Med 2021 May;25(9):4387-4394. Epub 2021 Apr 8 doi: 10.1111/jcmm.16502. PMID: 33830670Free PMC Article
Zhao G, Zhu M, Qin C, Liu X, Zhao X
J Pediatr Hematol Oncol 2020 Nov;42(8):495-498. doi: 10.1097/MPH.0000000000001603. PMID: 31764512
Weaver MS, Navid F, Huppmann A, Meany H, Angiolillo A
J Pediatr Hematol Oncol 2015 Apr;37(3):237-41. doi: 10.1097/MPH.0000000000000286. PMID: 25389917
Giannakopoulou C, Hatzidaki E, Giannakopoulos K, Matalliotakis I, Koumantakis E, Kalmanti M
J Dermatol 1999 Sep;26(9):595-8. PMID: 10535254
Smith KJ, Skelton HG, Barrett TL, Lupton GP, Graham JH
Mod Pathol 1989 Nov;2(6):603-9. PMID: 2587567

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...