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Prezentace aplikace PowerPoint

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Title: Prezentace aplikace PowerPoint Author: Pedro Last modified by: Peter Talar k Created Date: 10/23/2009 6:13:38 PM Document presentation format – PowerPoint PPT presentation

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Title: Prezentace aplikace PowerPoint


1
Prípad SD-IAP c. 522
P.Talarcík Cytopathos
2
Vek 5 rocný chlapec Klinika xeroderma
pigmentosum, mnohopocetné metachrónne/synchrónne
bazocelulárne a squamocelulárne karcinómy,
keratoakantóm, FEP, aktinická keratóza,
dysplastický pigmentový névus, všetky tumory v
oblasti tváre Makro excízia kože 5mm, bez
prominencie z oblasti cutis regio anguli oris
superioris
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Desmoplastic neurotropic angiotropic
melanoma
  • Neurotropic/perineural,endoneural,neural diff./
  • Angiotropic
  • Folliculotropic
  • Syringotropic
  • M.arrector pillorum musculotropic
  • Amelanotic
  • Desmoplastic omnitropic melanoma

12
R.Barnhill et al Less common Variants of
cutaneus melanoma. Emerging cancer therapeutics
33, 421-460, 2012
  • pure DM
  • desmoplastic neurotropic m.
  • pure neurotropic, spindle cell, no desmoplasia,
    epitheliod cell /conventionalneurotropic?/
  • mixed/combined variants of DM/DNM with 10-50 of
    conventional melanoma, e.g. spindle
    cell/epitheliod

13
Desmoplastic neurotropic/angiotropic melanoma.
  • disparate dignity versus conventional melanoma
  • may look deceptively indolent in picture
  • less than 4mm/intermediate grade neurocristic
  • sarcoma??, no correlation Breslow versus sentinel
  • olders 60s-70s, predominance of males, but
    almost any age!!
  • headnneck, but almost everywhere
  • desmoplastic and neurotropic overlap

14
Desmoplastic neurotropic/angiotropic melanoma.
  • p75 NGF-R
  • HMB-45, Melan-A more often negative
  • S100 positive /almost allways/
  • SOX10-all melanomas, CCS, MPnST
  • PnL2, conventional sens.75-100, no DM
  • CD146/Mel-CAM all melanomas
  • Aberrant expression keratins, CD68, CD34, SMA
    etc.

15
Differential diagnosis- do not hesitate to be
paranoic looking at everything spindle
  • scar/cellularity,elastosis, lymph.agg., adnex.,
    lack ofhorizontal fibrosis, extravasation
  • desmoplastic/sclerosing nevi, Spitz, amelanotic
    blue, desmoplastic neurothecoma
  • dermatofibroma, neurofibroma, spindle-cell AFX
  • DFSP, Bednar tumor
  • any cutaneus spindle-cell monomorphic sarcoma
  • spindle cell sarcomatoid sq.cell carcinoma

16
Prognosis, prediction, managment, follow-up-
  • Pure DM/DnM better prognosis?
  • Mixed forms may combine dignity of components
  • 95 of pure DMs BRAF wild-type
  • quick diagnosis, margin 1-3 cm

17
Xeroderma pigmentosum.
  • rare autosomal recessive disorder, consanguinity
  • genetical heterogenous
  • defects in nucleotide excision repair genes
    /NER/
  • Incidence 1/1000 000
  • More common Japan, Middle east, North Africa
  • no sex predilection
  • First describtion by Hebra et Kaposi 1874
  • 1 000-fold increased risk for non-melanomacancer
    under the age of 20
  • median age for non-melanoma skin cancer 8 yrs of
    age
  • 10-20-fold increased risk for internal
    malignancies

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