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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

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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