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Angiomatoid fibrous histiocytoma

MedGen UID:
226845
Concept ID:
C1266127
Neoplastic Process
Synonym: Histiocytoma, Angiomatoid Fibrous
SNOMED CT: Angiomatoid fibrous histiocytoma (128744003); Angiomatoid fibrous histiocytoma (1293020005)
 
Gene (location): CREB1 (2q33.3)
 
Monarch Initiative: MONDO:0012809
OMIM®: 612160
Orphanet: ORPHA569164

Definition

A rare soft tissue tumor characterized by a slow-growing, usually painless, subcutaneous nodule, predominantly located in the extremities, less frequently the trunk or head and neck region. Histopathologically, the lesion is well-circumscribed, lobulated, and composed of epitheloid, ovoid, or spindle cells arranged in a nodular and often syncytial pattern, with pseudoangiomatoid spaces and a peripheral fibrous pseudocapsule with a prominent lymphoplasmacytic cuff. The tumor is most common in the first two decades of life and usually follows an indolent course, although local recurrence may occur, while metastasis is rare. [from ORDO]

Clinical features

From HPO
Histiocytoma
MedGen UID:
267592
Concept ID:
C1509147
Neoplastic Process
A neoplasm containing histiocytes.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAngiomatoid fibrous histiocytoma

Professional guidelines

PubMed

Chokoeva AA, Tchernev G, Cardoso JC, Patterson JW, Dechev I, Valkanov S, Zanardelli M, Lotti T, Wollina U
Int J Immunopathol Pharmacol 2015 Jun;28(2):178-86. Epub 2015 Mar 26 doi: 10.1177/0394632015575977. PMID: 25816393

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

Recent clinical studies

Therapy

Potter SL, Quintanilla NM, Johnston DK, Naik-Mathuria B, Venkatramani R
Pediatr Blood Cancer 2018 Oct;65(10):e27291. Epub 2018 Jun 22 doi: 10.1002/pbc.27291. PMID: 29932283
Ogden S, Harave S, McPartland J, Brennan B, Jeys L, Losty P, Pizer B
Pediatr Blood Cancer 2017 Jun;64(6) Epub 2016 Dec 24 doi: 10.1002/pbc.26376. PMID: 28012233
Kohorst MA, Tran CL, Folpe AL, Sethi S, Arndt CA
Pediatr Nephrol 2014 Nov;29(11):2221-4. Epub 2014 Jun 1 doi: 10.1007/s00467-014-2852-1. PMID: 24880818

Prognosis

Boland JM
Mod Pathol 2022 Jan;35(Suppl 1):57-65. Epub 2021 Sep 13 doi: 10.1038/s41379-021-00914-7. PMID: 34518633
Wang Z, Zhang L, Ren L, Liu D, Du J, Zhang M, Lou G, Song Y, Wang Y, Wu C, Han G
Thorac Cancer 2021 Feb;12(3):314-323. Epub 2020 Dec 12 doi: 10.1111/1759-7714.13727. PMID: 33314685Free PMC Article
Saito K, Kobayashi E, Yoshida A, Araki Y, Kubota D, Tanzawa Y, Kawai A, Yanagawa T, Takagishi K, Chuman H
BMC Musculoskelet Disord 2017 Jan 23;18(1):31. doi: 10.1186/s12891-017-1390-y. PMID: 28114920Free PMC Article
Kao YC, Lan J, Tai HC, Li CF, Liu KW, Tsai JW, Fang FM, Yu SC, Huang HY
J Clin Pathol 2014 Mar;67(3):210-5. Epub 2013 Sep 16 doi: 10.1136/jclinpath-2013-201857. PMID: 24043718
Pratibha R, Ahmed S
Int J Paediatr Dent 2006 Sep;16(5):363-9. doi: 10.1111/j.1365-263X.2006.00734.x. PMID: 16879335

Clinical prediction guides

Miettinen M, Abdullaev Z, Turakulov R, Quezado M, Luiña Contreras A, Curcio CA, Rys J, Chlopek M, Lasota J, Aldape KD
Am J Surg Pathol 2024 Jan 1;48(1):112-122. Epub 2023 Nov 3 doi: 10.1097/PAS.0000000000002138. PMID: 37921028Free PMC Article
Kim NR, Kim SI, Park JW, Park CK, Chung CK, Choi SH, Yun H, Park SH
Neuropathology 2022 Aug;42(4):257-268. Epub 2022 Jun 22 doi: 10.1111/neup.12814. PMID: 35730186
Wang Z, Zhang L, Ren L, Liu D, Du J, Zhang M, Lou G, Song Y, Wang Y, Wu C, Han G
Thorac Cancer 2021 Feb;12(3):314-323. Epub 2020 Dec 12 doi: 10.1111/1759-7714.13727. PMID: 33314685Free PMC Article
Abrahao-Machado LF, Bacchi LM, Fernandes IL, Costa FD, Bacchi CE
Appl Immunohistochem Mol Morphol 2020 Sep;28(8):641-645. doi: 10.1097/PAI.0000000000000816. PMID: 31652145
Kao YC, Lan J, Tai HC, Li CF, Liu KW, Tsai JW, Fang FM, Yu SC, Huang HY
J Clin Pathol 2014 Mar;67(3):210-5. Epub 2013 Sep 16 doi: 10.1136/jclinpath-2013-201857. PMID: 24043718

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.

    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

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