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Desmoplastic Fibroblastoma

MedGen UID:
226977
•Concept ID:
C1300346
•
Neoplastic Process
Synonyms: Collagenous Fibroma; Collagenous fibroma; Desmoplastic fibroblastoma
SNOMED CT: Desmoplastic fibroblastoma (388984008); Collagenous fibroma (388984008)

Definition

A rare benign soft tissue neoplasm characterized by the presence of an abundant collagenous or myxocollagenous matrix, spindle-shaped, and stellate-shaped fibroblasts. It usually presents as an asymptomatic, slowly growing subcutaneous mass. The most common sites of involvement are the upper arm, shoulder, and lower limb. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDesmoplastic Fibroblastoma

Professional guidelines

PubMed

Diagnosis and Differential Diagnosis of Desmoplastic Fibroblastoma by Clinical, Radiological, and Histopathological Analyses.
Gong LH, Liu WF, Ding Y, Geng YH, Sun XQ, Huang XY
Chin Med J (Engl) 2018 Jan 5;131(1):32-36. doi: 10.4103/0366-6999.221274. PMID: 29271377Free PMC Article

Recent clinical studies

Etiology

Invasive desmoplastic fibroblastoma in the chest wall.
Kawaguchi Y, Fujita T, Hanaoka J
Ann Thorac Surg 2015 Apr;99(4):e85-6. doi: 10.1016/j.athoracsur.2015.01.055. PMID: 25841856
Massive desmoplastic fibroblastoma with scapular invasion.
Merriman DJ, Deavers MT, Czerniak BA, Lin PP
Orthopedics 2010 Aug 11;33(8) doi: 10.3928/01477447-20100625-23. PMID: 20704102
Expression of claudin-1, a recently described tight junction-associated protein, distinguishes soft tissue perineurioma from potential mimics.
Folpe AL, Billings SD, McKenney JK, Walsh SV, Nusrat A, Weiss SW
Am J Surg Pathol 2002 Dec;26(12):1620-6. doi: 10.1097/00000478-200212000-00010. PMID: 12459629
Childhood desmoplastic fibroblastoma (collagenous fibroma) with a 12-year follow-up.
Magro G, Venti C
Pediatr Dev Pathol 1999 Jan-Feb;2(1):62-4. doi: 10.1007/s100249900091. PMID: 9841708
Desmoplastic fibroblastoma. A report of seven cases.
Evans HL
Am J Surg Pathol 1995 Sep;19(9):1077-81. PMID: 7661281

Diagnosis

Recurrent FOSL1 rearrangements in desmoplastic fibroblastoma.
De Noon S, Piggott R, Trotman J, Tadross JA, Fittall M, Hughes D, Ye H, Munasinghe E, Murray M, Tirabosco R, Amary F, Coleman N, Watkins J, Hubank M, Tarpey P, Behjati S, Flanagan AM
J Pathol 2023 Feb;259(2):119-124. Epub 2023 Jan 3 doi: 10.1002/path.6038. PMID: 36426824Free PMC Article
A desmoplastic fibroblastoma that developed in the anterior mediastinum: a case report.
Hato T, Kashimada H, Yamaguchi M, Sugiyama A, Inoue Y, Aoki K, Fukuda H, Gika M, Kikuchi J, Fujino T, Yamaguchi T, Tamaru JI, Kohno M, Nakayama M
J Med Case Rep 2021 Oct 18;15(1):525. doi: 10.1186/s13256-021-03014-x. PMID: 34663426Free PMC Article
Primary desmoplastic fibroblastoma of diaphragm.
Guo C, Wang G, Ma D, Li J, Liu H, Li S
Thorac Cancer 2021 Nov;12(21):2961-2963. Epub 2021 Sep 27 doi: 10.1111/1759-7714.14168. PMID: 34581000Free PMC Article
Desmoplastic Fibroblastoma: An Uncommon Tumor With a Relatively Characteristic MRI Appearance.
Kresse ME, Kransdorf MJ, Fox MG, Flug JA, Long JR, Murphey MD
AJR Am J Roentgenol 2020 Jul;215(1):178-183. Epub 2020 May 14 doi: 10.2214/AJR.19.22163. PMID: 32406775
Diagnosis and Differential Diagnosis of Desmoplastic Fibroblastoma by Clinical, Radiological, and Histopathological Analyses.
Gong LH, Liu WF, Ding Y, Geng YH, Sun XQ, Huang XY
Chin Med J (Engl) 2018 Jan 5;131(1):32-36. doi: 10.4103/0366-6999.221274. PMID: 29271377Free PMC Article

Prognosis

Bilateral collagenous fibroma of the hard palate: a case report and review of the literature.
El-Naggar HA, El-Mahallawy YA, Harby MI, Abou Madawi NA
J Med Case Rep 2023 Jan 7;17(1):5. doi: 10.1186/s13256-022-03691-2. PMID: 36609451Free PMC Article
Unusual Appearance of Nasal Collagenous Fibroma (Desmoplastic Fibroblastoma) on Imaging.
Liu X, Guo X, Wang L, Chen Y, Yu H, Sun Z
J Coll Physicians Surg Pak 2022 Aug;32(8):1064-1066. doi: 10.29271/jcpsp.2022.08.1064. PMID: 35932135
Desmoplastic Fibroblastoma (Collagenous Fibroma) of the Foot: A Case Report of a Rare Benign Tumor in an Atypical Patient.
Allen R, Parks C, Dini M
J Am Podiatr Med Assoc 2022 May-Jun;112(3) doi: 10.7547/19-020. PMID: 35797230
Diagnosis and Differential Diagnosis of Desmoplastic Fibroblastoma by Clinical, Radiological, and Histopathological Analyses.
Gong LH, Liu WF, Ding Y, Geng YH, Sun XQ, Huang XY
Chin Med J (Engl) 2018 Jan 5;131(1):32-36. doi: 10.4103/0366-6999.221274. PMID: 29271377Free PMC Article
Invasive desmoplastic fibroblastoma in the chest wall.
Kawaguchi Y, Fujita T, Hanaoka J
Ann Thorac Surg 2015 Apr;99(4):e85-6. doi: 10.1016/j.athoracsur.2015.01.055. PMID: 25841856

Clinical prediction guides

Recurrent FOSL1 rearrangements in desmoplastic fibroblastoma.
De Noon S, Piggott R, Trotman J, Tadross JA, Fittall M, Hughes D, Ye H, Munasinghe E, Murray M, Tirabosco R, Amary F, Coleman N, Watkins J, Hubank M, Tarpey P, Behjati S, Flanagan AM
J Pathol 2023 Feb;259(2):119-124. Epub 2023 Jan 3 doi: 10.1002/path.6038. PMID: 36426824Free PMC Article
A desmoplastic fibroblastoma that developed in the anterior mediastinum: a case report.
Hato T, Kashimada H, Yamaguchi M, Sugiyama A, Inoue Y, Aoki K, Fukuda H, Gika M, Kikuchi J, Fujino T, Yamaguchi T, Tamaru JI, Kohno M, Nakayama M
J Med Case Rep 2021 Oct 18;15(1):525. doi: 10.1186/s13256-021-03014-x. PMID: 34663426Free PMC Article
Diagnosis and Differential Diagnosis of Desmoplastic Fibroblastoma by Clinical, Radiological, and Histopathological Analyses.
Gong LH, Liu WF, Ding Y, Geng YH, Sun XQ, Huang XY
Chin Med J (Engl) 2018 Jan 5;131(1):32-36. doi: 10.4103/0366-6999.221274. PMID: 29271377Free PMC Article
Collagenous fibroma (desmoplastic fibroblastoma).
Dagli M, Eryilmaz A, Acar A, Kulacoglu S, Akmansu H
Yonsei Med J 2004 Oct 31;45(5):941-3. doi: 10.3349/ymj.2004.45.5.941. PMID: 15515209
Desmoplastic fibroblastoma (collagenous fibroma).
Jang JG, Jung HH, Suh KS, Kim ST
Am J Dermatopathol 1999 Jun;21(3):256-8. doi: 10.1097/00000372-199906000-00009. PMID: 10380048

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