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Prípad SD-IAP č. 522

Prípad SD-IAP č. 522. P.Talarčík Cytopathos. Vek 5 ročný chlapec Klinika: xeroderma pigmentosum, mnohopočetné metachrónne/synchrónne bazocelulárne a squamocelulárne karcinómy, keratoakantóm, FEP, aktinická keratóza, dysplastický pigmentový névus, všetky tumory v oblasti tváre

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Prípad SD-IAP č. 522

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  1. Prípad SD-IAP č. 522 P.Talarčík Cytopathos

  2. Vek 5 ročný chlapec Klinika: xeroderma pigmentosum, mnohopočetné 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

  3. Desmoplastic neurotropic angiotropic melanoma • Neurotropic/perineural,endoneural,neuraldiff./ • Angiotropic • Folliculotropic • Syringotropic • M.arrectorpillorummusculotropic • Amelanotic • Desmoplasticomnitropicmelanoma

  4. R.Barnhill et al : Less common Variants of cutaneus melanoma. Emerging cancer therapeutics 3:3, 421-460, 2012 • pure DM • desmoplastic neurotropic m. • pure neurotropic, spindle cell, no desmoplasia, epitheliod cell /conventional+neurotropic?/ • mixed/combined variants of DM/DNM with 10-50% of conventional melanoma, e.g. spindle cell/epitheliod

  5. 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!! • head´n´neck, but almost everywhere • desmoplastic and neurotropic overlap

  6. 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.

  7. 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

  8. 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

  9. 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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