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Fibroblastic neoplasm

MedGen UID:
60198
•Concept ID:
C0206643
•
Neoplastic Process
Synonym: Fibrous tissue neoplasm
 
HPO: HP:0012316
Monarch Initiative: MONDO:0006209

Definition

Any neoplasm composed of fibrous tissue. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFibroblastic neoplasm

Professional guidelines

PubMed

Recent Advances in the Diagnosis, Pathogenesis, and Management of Myxoinflammatory Fibroblastic Sarcoma.
Nishio J, Nakayama S, Aoki M
Int J Mol Sci 2024 Jan 17;25(2) doi: 10.3390/ijms25021127. PMID: 38256198Free PMC Article
Role of Imaging in Management of Desmoid-type Fibromatosis: A Primer for Radiologists.
Braschi-Amirfarzan M, Keraliya AR, Krajewski KM, Tirumani SH, Shinagare AB, Hornick JL, Baldini EH, George S, Ramaiya NH, Jagannathan JP
Radiographics 2016 May-Jun;36(3):767-82. doi: 10.1148/rg.2016150153. PMID: 27163593

Recent clinical studies

Etiology

Wide local excision, Mohs micrographic surgery, and reconstructive options for treatment of dermatofibrosarcoma protuberans of the breast: A retrospective case series from Mayo Clinic.
Saifuddin H, Yan M, Jakub J, Martinez-Jorge J, Roenigk R, Vijayasekaran A
World J Surg Oncol 2023 May 6;21(1):141. doi: 10.1186/s12957-023-03022-9. PMID: 37147611Free PMC Article
Solitary fibrous tumor: An evolving and unifying entity with unsettled issues.
Huang SC, Huang HY
Histol Histopathol 2019 Apr;34(4):313-334. Epub 2018 Nov 15 doi: 10.14670/HH-18-064. PMID: 30431144
USP6 genetic rearrangements in cellular fibroma of tendon sheath.
Carter JM, Wang X, Dong J, Westendorf J, Chou MM, Oliveira AM
Mod Pathol 2016 Aug;29(8):865-9. Epub 2016 Apr 29 doi: 10.1038/modpathol.2016.83. PMID: 27125357
Is wide resection obsolete for desmoid tumors in children and adolescents? Evaluation of histological margins, immunohistochemical markers, and review of literature.
Woltsche N, Gilg MM, Fraissler L, Liegl-Atzwanger B, Beham A, Lackner H, Benesch M, Leithner A
Pediatr Hematol Oncol 2015 Feb;32(1):60-9. Epub 2014 Sep 29 doi: 10.3109/08880018.2014.956905. PMID: 25264623
Massive localised lymphoedema: a clinicopathological study of 22 cases and review of the literature.
Manduch M, Oliveira AM, Nascimento AG, Folpe AL
J Clin Pathol 2009 Sep;62(9):808-11. doi: 10.1136/jcp.2009.066555. PMID: 19734477

Diagnosis

Recent Advances in the Diagnosis, Pathogenesis, and Management of Myxoinflammatory Fibroblastic Sarcoma.
Nishio J, Nakayama S, Aoki M
Int J Mol Sci 2024 Jan 17;25(2) doi: 10.3390/ijms25021127. PMID: 38256198Free PMC Article
CYP1A1 Is a Useful Diagnostic Marker for Angiofibroma of Soft Tissue.
Uemura K, Komatsu M, Hara S, Kawamoto T, Bitoh Y, Itoh T, Hirose T
Am J Surg Pathol 2023 May 1;47(5):547-557. Epub 2023 Mar 6 doi: 10.1097/PAS.0000000000002029. PMID: 36876749
The emerging PRRX1-NCOA fibroblastic neoplasm: a combined reappraisal of published tumors and two new cases.
Chen CH, Chang KC, Chuang CH, Fu JT, Huang HY
Virchows Arch 2022 Jul;481(1):111-116. Epub 2021 Oct 14 doi: 10.1007/s00428-021-03219-x. PMID: 34647172
Solitary fibrous tumor: An evolving and unifying entity with unsettled issues.
Huang SC, Huang HY
Histol Histopathol 2019 Apr;34(4):313-334. Epub 2018 Nov 15 doi: 10.14670/HH-18-064. PMID: 30431144
Role of Imaging in Management of Desmoid-type Fibromatosis: A Primer for Radiologists.
Braschi-Amirfarzan M, Keraliya AR, Krajewski KM, Tirumani SH, Shinagare AB, Hornick JL, Baldini EH, George S, Ramaiya NH, Jagannathan JP
Radiographics 2016 May-Jun;36(3):767-82. doi: 10.1148/rg.2016150153. PMID: 27163593

Therapy

Wide local excision, Mohs micrographic surgery, and reconstructive options for treatment of dermatofibrosarcoma protuberans of the breast: A retrospective case series from Mayo Clinic.
Saifuddin H, Yan M, Jakub J, Martinez-Jorge J, Roenigk R, Vijayasekaran A
World J Surg Oncol 2023 May 6;21(1):141. doi: 10.1186/s12957-023-03022-9. PMID: 37147611Free PMC Article
The Activity and Safety of Anlotinib for Patients with Extremity Desmoid Fibromatosis: A Retrospective Study in a Single Institution.
Zheng C, Zhou Y, Wang Y, Luo Y, Tu C, Min L
Drug Des Devel Ther 2020;14:3941-3950. Epub 2020 Sep 25 doi: 10.2147/DDDT.S271008. PMID: 33061299Free PMC Article
An Intra-abdominal Solid-cystic Desmoid That Emerged after Distal Gastrectomy.
Takinami M, Matsubayashi H, Ishiwatari H, Uesaka K, Okamura Y, Sasaki K, Ohike N, Hirabayashi K, Ono H
Intern Med 2019;58(24):3525-3529. Epub 2019 Dec 15 doi: 10.2169/internalmedicine.3252-19. PMID: 31839672Free PMC Article
Long-Term Follow-Up of Desmoid Fibromatosis Treated with PF-03084014, an Oral Gamma Secretase Inhibitor.
Villalobos VM, Hall F, Jimeno A, Gore L, Kern K, Cesari R, Huang B, Schowinsky JT, Blatchford PJ, Hoffner B, Elias A, Messersmith W
Ann Surg Oncol 2018 Mar;25(3):768-775. Epub 2017 Sep 8 doi: 10.1245/s10434-017-6082-1. PMID: 28887726

Prognosis

Solitary fibrous tumor: An evolving and unifying entity with unsettled issues.
Huang SC, Huang HY
Histol Histopathol 2019 Apr;34(4):313-334. Epub 2018 Nov 15 doi: 10.14670/HH-18-064. PMID: 30431144
ALK Is a Specific Diagnostic Marker for Inflammatory Myofibroblastic Tumor of the Uterus.
Mohammad N, Haimes JD, Mishkin S, Kudlow BA, Leong MY, Chew SH, Koay E, Whitehouse A, Cope N, Ali RH, Köbel M, Stewart CJR, McCluggage WG, Lee CH
Am J Surg Pathol 2018 Oct;42(10):1353-1359. doi: 10.1097/PAS.0000000000001120. PMID: 30015720
Desmoid Tumours of the extremity and trunk. A retrospective study of 44 patients.
Wirth L, Klein A, Baur-Melnyk A, Knösel T, Lindner LH, Roeder F, Jansson V, Dürr HR
BMC Musculoskelet Disord 2018 Jan 5;19(1):2. doi: 10.1186/s12891-017-1924-3. PMID: 29304783Free PMC Article
Is wide resection obsolete for desmoid tumors in children and adolescents? Evaluation of histological margins, immunohistochemical markers, and review of literature.
Woltsche N, Gilg MM, Fraissler L, Liegl-Atzwanger B, Beham A, Lackner H, Benesch M, Leithner A
Pediatr Hematol Oncol 2015 Feb;32(1):60-9. Epub 2014 Sep 29 doi: 10.3109/08880018.2014.956905. PMID: 25264623
Massive localised lymphoedema: a clinicopathological study of 22 cases and review of the literature.
Manduch M, Oliveira AM, Nascimento AG, Folpe AL
J Clin Pathol 2009 Sep;62(9):808-11. doi: 10.1136/jcp.2009.066555. PMID: 19734477

Clinical prediction guides

A Rare Case of Breast fat-Forming Solitary Fibrous Tumor With Molecular Confirmation.
Liu Y, Zhang M, Wang D, Cao C, Yao Z, Pan J
Int J Surg Pathol 2023 Aug;31(5):830-838. Epub 2022 Dec 7 doi: 10.1177/10668969221143475. PMID: 36476128
ALK Is a Specific Diagnostic Marker for Inflammatory Myofibroblastic Tumor of the Uterus.
Mohammad N, Haimes JD, Mishkin S, Kudlow BA, Leong MY, Chew SH, Koay E, Whitehouse A, Cope N, Ali RH, Köbel M, Stewart CJR, McCluggage WG, Lee CH
Am J Surg Pathol 2018 Oct;42(10):1353-1359. doi: 10.1097/PAS.0000000000001120. PMID: 30015720
Is wide resection obsolete for desmoid tumors in children and adolescents? Evaluation of histological margins, immunohistochemical markers, and review of literature.
Woltsche N, Gilg MM, Fraissler L, Liegl-Atzwanger B, Beham A, Lackner H, Benesch M, Leithner A
Pediatr Hematol Oncol 2015 Feb;32(1):60-9. Epub 2014 Sep 29 doi: 10.3109/08880018.2014.956905. PMID: 25264623
STAT6 immunohistochemistry is helpful in the diagnosis of solitary fibrous tumors.
Yoshida A, Tsuta K, Ohno M, Yoshida M, Narita Y, Kawai A, Asamura H, Kushima R
Am J Surg Pathol 2014 Apr;38(4):552-9. doi: 10.1097/PAS.0000000000000137. PMID: 24625420
MUC4 is a sensitive and extremely useful marker for sclerosing epithelioid fibrosarcoma: association with FUS gene rearrangement.
Doyle LA, Wang WL, Dal Cin P, Lopez-Terrada D, Mertens F, Lazar AJ, Fletcher CD, Hornick JL
Am J Surg Pathol 2012 Oct;36(10):1444-51. doi: 10.1097/PAS.0b013e3182562bf8. PMID: 22982887

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