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Sclerotic Fibroma

MedGen UID:
98364
•Concept ID:
C0406815
•
Neoplastic Process
Synonyms: Circumscribed storiform collagenoma; Circumscribed Storiform Collagenoma
SNOMED CT: Circumscribed storiform collagenoma (239137003)

Definition

A benign skin tumor characterized as a small, dome-shaped dermal nodule composed of fibroblasts in a dense collagenous background with storiform organization. Rarely, multiple lesions can occur in connection with Cowden''s disease. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSclerotic Fibroma

Professional guidelines

PubMed

The differential diagnosis between sclerotic fibroma and sclerosing perineurioma: An unresolved challenge.
Saggini A, Baciorri F
J Cutan Pathol 2020 Feb;47(2):195-196. Epub 2019 Dec 9 doi: 10.1111/cup.13620. PMID: 31782178

Recent clinical studies

Etiology

Superficial fibromas with CTNNB1 mutation.
Kuntze A, Meliß RR, Ermert L, Falkenberg KD, Puller AC, Trautmann M, Hartmann W, Wardelmann E
Genes Chromosomes Cancer 2024 May;63(5):e23247. doi: 10.1002/gcc.23247. PMID: 38757718
Utilizing PTEN immunohistochemistry as a screening test for Cowden syndrome.
Hartsough E, DeSimone MS, Lorenzo ME, Dias-Santagata D, Nose V, Hoang MP
Am J Clin Pathol 2024 May 2;161(5):490-500. doi: 10.1093/ajcp/aqad177. PMID: 38206110
Cd34+ Stromal Cells/Telocytes in Normal and Pathological Skin.
Díaz-Flores L, Gutiérrez R, García MP, González-Gómez M, Rodríguez-Rodriguez R, Hernández-León N, Díaz-Flores L Jr, Carrasco JL
Int J Mol Sci 2021 Jul 8;22(14) doi: 10.3390/ijms22147342. PMID: 34298962Free PMC Article
Benign mesenchymal tumors of the external ear: A series of 14 cases.
AbdullGaffar B, Manzlgi M, Saleem N
Ann Diagn Pathol 2019 Aug;41:62-68. Epub 2019 May 20 doi: 10.1016/j.anndiagpath.2019.05.011. PMID: 31132654
Sclerotic fibroma-like change in various neoplastic and inflammatory skin lesions: is sclerotic fibroma a distinct entity?
High WA, Stewart D, Essary LR, Kageyama NP, Hoang MP, Cockerell CJ
J Cutan Pathol 2004 May;31(5):373-8. doi: 10.1111/j.0303-6987.2004.00199.x. PMID: 15059222

Diagnosis

Utilizing PTEN immunohistochemistry as a screening test for Cowden syndrome.
Hartsough E, DeSimone MS, Lorenzo ME, Dias-Santagata D, Nose V, Hoang MP
Am J Clin Pathol 2024 May 2;161(5):490-500. doi: 10.1093/ajcp/aqad177. PMID: 38206110
Paravertebral fibrous pseudotumor: Four cases of a distinctive tumefactive lesion overlapping with eosinophilic angiocentric fibrosis and tumoral erythema elevatum diutinum.
Agaimy A, Förster S, Kalla J, Stacher-Priehse E, Titze U, Vieth M
Ann Diagn Pathol 2023 Feb;62:152073. Epub 2022 Dec 5 doi: 10.1016/j.anndiagpath.2022.152073. PMID: 36502765
Storiform collagenoma (sclerotic fibroma) of the oral mucosa.
Elledge R, Nandra B, Bates T, Zardo D, Parmar S
Br J Oral Maxillofac Surg 2020 Feb;58(2):231-233. Epub 2019 Nov 21 doi: 10.1016/j.bjoms.2019.11.005. PMID: 31761569
Two Cases of Sclerotic Fibroma of the Skin that Mimicked Keloids Clinically.
Tosa M, Ansai SI, Kuwahara H, Akaishi S, Ogawa R
J Nippon Med Sch 2018;85(5):283-286. doi: 10.1272/jnms.JNMS.2018_85-45. PMID: 30464146
Late-stage nodular erythema elevatum diutinum mimicking sclerotic fibroma.
Shi KY, Vandergriff T
J Cutan Pathol 2018 Jan;45(1):94-96. Epub 2017 Nov 22 doi: 10.1111/cup.13066. PMID: 29068068

Therapy

Selected case from the Arkadi M. Rywlin International Pathology Slide Seminar: sclerotic (fibroma-like) lipoma, dorsum of right hand.
Allen PW
Adv Anat Pathol 2013 Jan;20(1):68-72. doi: 10.1097/PAP.0b013e31827b65e3. PMID: 23232574
Sclerotic (fibroma-like) lipoma: a distinctive lipoma variant with a predilection for the distal extremities.
Laskin WB, Fetsch JF, Michal M, Miettinen M
Am J Dermatopathol 2006 Aug;28(4):308-16. doi: 10.1097/00000372-200608000-00003. PMID: 16871033

Prognosis

Superficial fibromas with CTNNB1 mutation.
Kuntze A, Meliß RR, Ermert L, Falkenberg KD, Puller AC, Trautmann M, Hartmann W, Wardelmann E
Genes Chromosomes Cancer 2024 May;63(5):e23247. doi: 10.1002/gcc.23247. PMID: 38757718
Germline mutation of the PTEN gene in a Japanese patient with Cowden's disease.
Kato N, Kimura K, Sugawara H, Aoyagi S, Ikeda T, Horii A
Int J Oncol 2001 May;18(5):1017-22. doi: 10.3892/ijo.18.5.1017. PMID: 11295050
Sclerosing perineurioma: a clinicopathologic study of 19 cases of a distinctive soft tissue lesion with a predilection for the fingers and palms of young adults.
Fetsch JF, Miettinen M
Am J Surg Pathol 1997 Dec;21(12):1433-42. doi: 10.1097/00000478-199712000-00005. PMID: 9414186

Clinical prediction guides

Utility of LEF1 to differentiate desmoid fibromatosis from its histologic mimics.
Jobbagy S, Lozano-Calderon S, Mullen JT, Nielsen GP, Hung YP, Chebib I
Virchows Arch 2024 May;484(5):807-813. Epub 2024 Mar 19 doi: 10.1007/s00428-024-03782-z. PMID: 38503969
Giant cell collagenomas associated with Cowden syndrome: A case report.
Salusti-Simpson M, Madrid M, Cook D
J Cutan Pathol 2024 May;51(5):345-347. Epub 2024 Feb 9 doi: 10.1111/cup.14595. PMID: 38337148
Sclerosing Dermatofibrosarcoma Protuberans Shows Significant Overlap With Sclerotic Fibroma in Both Routine and Immunohistochemical Analysis: A Potential Diagnostic Pitfall.
Abdaljaleel MY, North JP
Am J Dermatopathol 2017 Feb;39(2):83-88. doi: 10.1097/DAD.0000000000000584. PMID: 28134725
Sclerotic fibroma-like change in various neoplastic and inflammatory skin lesions: is sclerotic fibroma a distinct entity?
High WA, Stewart D, Essary LR, Kageyama NP, Hoang MP, Cockerell CJ
J Cutan Pathol 2004 May;31(5):373-8. doi: 10.1111/j.0303-6987.2004.00199.x. PMID: 15059222
Histopathologic diagnosis of superficial soft tissue tumors, related lesions, and simulators: an algorithmic approach based on colors: tumors with predominance of pink.
Diaz-Cascajo C
Am J Dermatopathol 2000 Apr;22(2):191-6. doi: 10.1097/00000372-200004000-00020. PMID: 10770445

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