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60 y.o. Female. Tumour on the right upper arm. Non-melanoma skin cancer? CASE 27.

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Presentation on theme: "60 y.o. Female. Tumour on the right upper arm. Non-melanoma skin cancer? CASE 27."— Presentation transcript:

1 60 y.o. Female. Tumour on the right upper arm. Non-melanoma skin cancer? CASE 27

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8 Rare Aggressive No consensus on management

9 Keratin 20 Synapsin CD 56Ki67

10 British Association of Dermatology July 2007

11 CK20: cytokeratin 20 CK7: cytokeratin 7 NSE: neurone-specific enolase NFP: neurofilament protein S100: S100 protein LCA: leucocyte common antigen CD99: cluster-of-differentiation antigen 99 TTF1: thyroid transcription factor 1 +: positive stain –: negative stain Tumour CK20CK7NSENFPS100LCACD99TTF1 Merkel-cell carcinoma +-++--Rare- Small-cell carcinoma of lung -+++/---Rare+ Lymphoma -----+-- Peripheral primitive neuro- ectodermal tumour --+Rare--+- Small-cell melanoma --+----- Immunolabelling Profiles

12 Cytokeratin 20 Immunohistochemistry

13 Merkel Cell Carcinoma Rare highly malignant neuroendocrine skin tumour USA - 0.23 / 100 000 Male predominance Average age at presentation – 69y Merkel Cell Carcinoma

14 Associations UV light – most commonly seen on the head and neck region and the lower leg Immunosuppression –Post organ transplant (0.13 / 1000 patient yrs) –HIV –Haematological malignancies Associations

15 Clinical diagnosis is difficult Red / violaceous nodule May resemble an SCC / BCC or amelanotic melanoma Clinical Diagnosis

16 Prognostic markers Advanced stage at presentation Immunosupression Poor Prognostic Markers Good Prognostic Markers CD+ Lymphocyte invasion of the tumour Prescence of Polyomavirus

17 MCV MCPyV Clonally integrated in 80% of MCC

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19 Retrospective Analysis of MCC over 10years 14 cases identified SSMDT Barts Health NHS Trust

20 Results: Demographics 9 female, 5 male Mean age of presentation: 73years (range 54 to 87years) 21% presented with metastatic disease

21 Results: Treatment INTERVENTIONFrequency WLE with 1-3cm marginsALL Adjunctive RadiotherapyMajority (57%) Sentinel Lymph Node BiopsyFew (14%) Adjunctive Chemotherapy Enrolled onto MCC-1 Trial 1

22 Polyomavirus 10/14 tested 80% had positive polyomavirus Positive virus did not correlate with outcome

23 Results: Survival Rates All patients who presented with metastases died within a year (3/14) Of the remaining 11/14 patients, 1 died within a year Other comorbidities 10/14 patients are still alive to date Date range from 2005- 2012

24 British Association of Dermatology July 2007 Radiotherapy induced skin cancers BCC - 3.6% SCC - 0.003% Angiosarcoma Fibrosarcoma Melanoma Merkel cell carcinoma

25 Guidelines GermanyFranceUSAUK WLE margin3cm2-3cmNot specified Radiotherapy to surgical bed Yes SLNDYes Not obligatory Adjunct Chemotherapy No Yes- enrol in Clinical trials Chemotherapy in Metastases Yes Follow-up5-years5yearsMinimum 3years

26 Suggestions for UK pathway Histopathological criteria and Staging J Diaz- Perez et al. “Merkel cell carcinoma: a clinicopathological study of 11 cases.” JEADV, 2005 WLE with 2-3cm or Mohs S. J. Miller, M. Alam, J. Andersen, et al., “Merkel cell carcinoma,” Journal of the National Comprehensive Cancer Network, vol. 7, no. 3, pp. 322–332, 2009. Adjunctive Radiotherapy L. Mortier, X. Mirabel, C. Fournier, F. Piette, and E. Lartigau, “Radiotherapy alone for primary Merkel cell carcinoma,” Archives of Dermatology, vol. 139, pp. 1587–1590, 2003 Sentinel lymph node biopsy L. K. E. Rodrigues, S. P. L. Leong, M. Kashani-Sabet, and J. H. Wong, “Early experience with sentinel lymph node mapping for Merkel cell carcinoma,” Journal of the American Academy of Dermatology, vol. 45, no. 2, pp. 303–308, 2001. ?Role of Adjunctive chemotherapy


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