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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

Etiology

Sloan EA, Chiang J, Villanueva-Meyer JE, Alexandrescu S, Eschbacher JM, Wang W, Mafra M, Ud Din N, Carr-Boyd E, Watson M, Punsoni M, Oviedo A, Gilani A, Kleinschmidt-DeMasters BK, Coss DJ, Lopes MB, Raffel C, Berger MS, Chang SM, Reddy A, Ramani B, Ferris SP, Lee JC, Hofmann JW, Cho SJ, Horvai AE, Pekmezci M, Tihan T, Bollen AW, Rodriguez FJ, Ellison DW, Perry A, Solomon DA
Brain Pathol 2021 Jul;31(4):e12918. Epub 2021 Jan 28 doi: 10.1111/bpa.12918. PMID: 33141488Free 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
Thway K, Fisher C
Arch Pathol Lab Med 2015 May;139(5):674-82. doi: 10.5858/arpa.2014-0234-RA. PMID: 25927151
Bohman SL, Goldblum JR, Rubin BP, Tanas MR, Billings SD
Pathology 2014 Apr;46(3):199-204. doi: 10.1097/PAT.0000000000000073. PMID: 24614712
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

Diagnosis

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
Fischer GM, Papke DJ Jr
Semin Diagn Pathol 2023 Jul;40(4):246-257. Epub 2023 Apr 25 doi: 10.1053/j.semdp.2023.04.014. PMID: 37156707
Boland JM
Mod Pathol 2022 Jan;35(Suppl 1):57-65. Epub 2021 Sep 13 doi: 10.1038/s41379-021-00914-7. PMID: 34518633
Sloan EA, Chiang J, Villanueva-Meyer JE, Alexandrescu S, Eschbacher JM, Wang W, Mafra M, Ud Din N, Carr-Boyd E, Watson M, Punsoni M, Oviedo A, Gilani A, Kleinschmidt-DeMasters BK, Coss DJ, Lopes MB, Raffel C, Berger MS, Chang SM, Reddy A, Ramani B, Ferris SP, Lee JC, Hofmann JW, Cho SJ, Horvai AE, Pekmezci M, Tihan T, Bollen AW, Rodriguez FJ, Ellison DW, Perry A, Solomon DA
Brain Pathol 2021 Jul;31(4):e12918. Epub 2021 Jan 28 doi: 10.1111/bpa.12918. PMID: 33141488Free PMC Article
Thway K, Fisher C
Arch Pathol Lab Med 2015 May;139(5):674-82. doi: 10.5858/arpa.2014-0234-RA. PMID: 25927151

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
Song JY, Lee SK, Kim SG, Rotaru H, Baciut M, Dinu C
Oral Maxillofac Surg 2012 Jun;16(2):237-42. Epub 2011 Oct 1 doi: 10.1007/s10006-011-0297-2. PMID: 21965132

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

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    Clinical resources

    Practice guidelines

    • PubMed
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      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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