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Infantile digital fibromatosis

MedGen UID:
232929
Concept ID:
C1318562
Neoplastic Process
Synonyms: Inclusion body fibromatosis; Inclusion body fibromatosis (disease)
SNOMED CT: Infantile digital fibromatosis (399903008); Reye tumor (399903008); Infantile digital fibroma (399903008); Infantile digital myofibroblastoma (399903008); Inclusion body fibromatosis (1290969006)
 
HPO: HP:0025197
Monarch Initiative: MONDO:0016039
Orphanet: ORPHA199267

Definition

A benign tumor made up of mostly myofibroblasts that appears almost exclusively on the digits of the hands and feet, rarely involving the thumb or big toe. The lesion displays a proliferation of bland intradermal spindle cells arranged in whorls, fascicles, or a storiform pattern in a collagenous background of varying degrees. Also usually present are perpendicular tumor cell fascicles that extend to the epidermis. The small intracytoplasmic inclusions are said to appear similar to red blood cells. The inclusion bodies have been shown to be made up of densely packed vimentin and actin filaments. The tumor often causes a dome-shaped elevation of the overlying structures, forming a protuberant or polypoid nodule. The overlying epidermis can display a host of changes, including acanthosis, hyperkeratosis, parakeratosis, rete ridge flattening, entrapment of adnexal structures, and, rarely, ulceration. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVInfantile digital fibromatosis
Follow this link to review classifications for Infantile digital fibromatosis in Orphanet.

Professional guidelines

PubMed

Kelenjian S, Mattjie RA, Franz R, Biedermann T, Brockow K
J Dtsch Dermatol Ges 2019 Apr;17(4):393-397. Epub 2019 Mar 13 doi: 10.1111/ddg.13808. PMID: 30865379
Holmes WJ, Mishra A, McArthur P
J Plast Reconstr Aesthet Surg 2011 May;64(5):632-7. doi: 10.1016/j.bjps.2010.09.004. PMID: 20937573

Recent clinical studies

Etiology

Agarwal H, Singh L, Gupta N, Sengar M
Cytopathology 2020 Mar;31(2):115-121. Epub 2020 Feb 7 doi: 10.1111/cyt.12786. PMID: 32034815
Kelenjian S, Mattjie RA, Franz R, Biedermann T, Brockow K
J Dtsch Dermatol Ges 2019 Apr;17(4):393-397. Epub 2019 Mar 13 doi: 10.1111/ddg.13808. PMID: 30865379
Spingardi O, Zoccolan A, Venturino E
Chir Main 2011 Feb;30(1):62-5. Epub 2011 Jan 12 doi: 10.1016/j.main.2010.12.001. PMID: 21276743
Holmes WJ, Mishra A, McArthur P
J Plast Reconstr Aesthet Surg 2011 May;64(5):632-7. doi: 10.1016/j.bjps.2010.09.004. PMID: 20937573
Coffin CM, Dehner LP
Pediatr Pathol 1991 Jul-Aug;11(4):569-88. doi: 10.3109/15513819109064791. PMID: 1946077

Diagnosis

Rodríguez-Lomba E, Lozano-Masdemont B, Campos-Domínguez M
Actas Dermosifiliogr 2022 Apr;113(4):432-434. Epub 2021 Oct 6 doi: 10.1016/j.ad.2020.05.019. PMID: 35623736
Özdemir ZC, Töret E
Indian Pediatr 2021 Nov 15;58(11):1104. PMID: 34837378
Adegoke OO, Ajao AE, Ano-Edward GH
Afr Health Sci 2020 Dec;20(4):1865-1869. doi: 10.4314/ahs.v20i4.42. PMID: 34394250Free PMC Article
Taylor HO, Gellis SE, Schmidt BA, Upton J, Rogers GF
Ann Plast Surg 2008 Oct;61(4):472-6. doi: 10.1097/SAP.0b013e31816d8236. PMID: 18812724
Heymann WR
J Am Acad Dermatol 2008 Jul;59(1):122-3. doi: 10.1016/j.jaad.2007.11.025. PMID: 18571599

Therapy

Holmes WJ, Mishra A, McArthur P
J Plast Reconstr Aesthet Surg 2011 May;64(5):632-7. doi: 10.1016/j.bjps.2010.09.004. PMID: 20937573
Taylor HO, Gellis SE, Schmidt BA, Upton J, Rogers GF
Ann Plast Surg 2008 Oct;61(4):472-6. doi: 10.1097/SAP.0b013e31816d8236. PMID: 18812724
Shin SJ, Rosen PP
Arch Pathol Lab Med 2007 Jul;131(7):1126-9. doi: 10.5858/2007-131-1126-BPOFWD. PMID: 17617003
Oh CK, Son HS, Kwon YW, Jang HS, Kwon KS
Arch Dermatol 2005 May;141(5):549-50. doi: 10.1001/archderm.141.5.549. PMID: 15897374

Prognosis

Agarwal H, Singh L, Gupta N, Sengar M
Cytopathology 2020 Mar;31(2):115-121. Epub 2020 Feb 7 doi: 10.1111/cyt.12786. PMID: 32034815
Kelenjian S, Mattjie RA, Franz R, Biedermann T, Brockow K
J Dtsch Dermatol Ges 2019 Apr;17(4):393-397. Epub 2019 Mar 13 doi: 10.1111/ddg.13808. PMID: 30865379
Holmes WJ, Mishra A, McArthur P
J Plast Reconstr Aesthet Surg 2011 May;64(5):632-7. doi: 10.1016/j.bjps.2010.09.004. PMID: 20937573
Grenier N, Liang C, Capaldi L, Ney A, Lapidus C, Schappell D, Katarincic J, Robinson-Bostom L
Pediatr Dermatol 2008 Jan-Feb;25(1):72-5. doi: 10.1111/j.1525-1470.2007.00587.x. PMID: 18304159
Coffin CM, Dehner LP
Pediatr Pathol 1991 Jul-Aug;11(4):569-88. doi: 10.3109/15513819109064791. PMID: 1946077

Clinical prediction guides

Holmes WJ, Mishra A, McArthur P
J Plast Reconstr Aesthet Surg 2011 May;64(5):632-7. doi: 10.1016/j.bjps.2010.09.004. PMID: 20937573
Grenier N, Liang C, Capaldi L, Ney A, Lapidus C, Schappell D, Katarincic J, Robinson-Bostom L
Pediatr Dermatol 2008 Jan-Feb;25(1):72-5. doi: 10.1111/j.1525-1470.2007.00587.x. PMID: 18304159
Kawaguchi M, Mitsuhashi Y, Hozumi Y, Kondo S
J Dermatol 1998 Aug;25(8):523-6. doi: 10.1111/j.1346-8138.1998.tb02448.x. PMID: 9769598
Zhu WY, Xia MY, Huang YF, Leonardi C, Penneys NS
Pediatr Dermatol 1991 Jun;8(2):137-9. doi: 10.1111/j.1525-1470.1991.tb00304.x. PMID: 1656415
Iwasaki H, Kikuchi M, Mori R, Miyazono J, Enjoji M, Shinohara N, Matsuzaki A
Cancer 1980 Nov 15;46(10):2238-47. doi: 10.1002/1097-0142(19801115)46:10<2238::aid-cncr2820461021>3.0.co;2-9. PMID: 6253042

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