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Plaque-Like CD34-Positive Dermal Fibroma

MedGen UID:
1693456
•Concept ID:
C5230986
•
Neoplastic Process
Synonyms: Medallion-Like Dermal Dendrocyte Hamartoma; Plaque-like CD34 positive dermal fibroma
SNOMED CT: Plaque-like CD34 positive dermal fibroma (817948000)

Definition

A congenital or acquired benign dermal spindle cell neoplasm with histopathological features mimicking those of superficial dermal dermatofibrosarcoma protuberans. (WHO 2018) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPlaque-Like CD34-Positive Dermal Fibroma

Recent clinical studies

Etiology

Hypocellular Plaque-Like CD34-Positive Dermal Fibroma (Medallion-Like Dermal Dendrocyte Hamartoma) Presenting as a Skin-Colored Dermal Nodule.
Mutgi KA, Chitgopeker P, Ciliberto H, Stone MS
Pediatr Dermatol 2016 Jan-Feb;33(1):e16-9. Epub 2015 Dec 8 doi: 10.1111/pde.12726. PMID: 26645569

Diagnosis

ALK-rearranged, CD34-positive spindle cell neoplasms resembling dermatofibrosarcoma protuberans: a study of seven cases.
Agrawal S, Ameline B, Folpe AL, Azzato E, Astbury C, Mentzel T, Knapp C, Rütten A, Creytens D, Sukov W, Baumhoer D, Billings SD, Fritchie KJ
Histopathology 2024 Oct;85(4):649-659. Epub 2024 Jun 13 doi: 10.1111/his.15239. PMID: 38867577
Novel KHDRBS1-NTRK3 rearrangement in a congenital pediatric CD34-positive skin tumor: a case report.
Tallegas M, Fraitag S, Binet A, Orbach D, Jourdain A, Reynaud S, Pierron G, Machet MC, Maruani A
Virchows Arch 2019 Jan;474(1):111-115. Epub 2018 Sep 6 doi: 10.1007/s00428-018-2415-0. PMID: 30187166
Hypocellular Plaque-Like CD34-Positive Dermal Fibroma (Medallion-Like Dermal Dendrocyte Hamartoma) Presenting as a Skin-Colored Dermal Nodule.
Mutgi KA, Chitgopeker P, Ciliberto H, Stone MS
Pediatr Dermatol 2016 Jan-Feb;33(1):e16-9. Epub 2015 Dec 8 doi: 10.1111/pde.12726. PMID: 26645569
Hypocellular medallion-like dermal dendrocyte hamartoma (plaque-like CD34-positive dermal fibroma).
Bagazgoitia L, Moreno C
Dermatol Online J 2011 Sep 15;17(9):8. PMID: 21971273
Plaque-like CD34-positive dermal fibroma ("medallion-like dermal dendrocyte hamartoma"): clinicopathologic, immunohistochemical, and molecular analysis of 5 cases emphasizing its distinction from superficial, plaque-like dermatofibrosarcoma protuberans.
Kutzner H, Mentzel T, Palmedo G, Hantschke M, Rütten A, Paredes BE, Schärer L, Guillen CS, Requena L
Am J Surg Pathol 2010 Feb;34(2):190-201. doi: 10.1097/PAS.0b013e3181c7cf11. PMID: 20061935

Prognosis

Novel KHDRBS1-NTRK3 rearrangement in a congenital pediatric CD34-positive skin tumor: a case report.
Tallegas M, Fraitag S, Binet A, Orbach D, Jourdain A, Reynaud S, Pierron G, Machet MC, Maruani A
Virchows Arch 2019 Jan;474(1):111-115. Epub 2018 Sep 6 doi: 10.1007/s00428-018-2415-0. PMID: 30187166

Clinical prediction guides

Novel KHDRBS1-NTRK3 rearrangement in a congenital pediatric CD34-positive skin tumor: a case report.
Tallegas M, Fraitag S, Binet A, Orbach D, Jourdain A, Reynaud S, Pierron G, Machet MC, Maruani A
Virchows Arch 2019 Jan;474(1):111-115. Epub 2018 Sep 6 doi: 10.1007/s00428-018-2415-0. PMID: 30187166

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