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60 y.o. Female. Tumour on the right upper arm. Non-melanoma skin cancer? CASE 27 Rare Aggressive No consensus on management Keratin 20 CD 56 Synapsin Ki67 British Association of Dermatology July 2007 Immunolabelling Profiles Tumour CK20 CK7 NSE NFP S100 LCA CD99 TTF1 Merkel-cell carcinoma + - + + - - Rare - Small-cell carcinoma of lung - + + +/- - - Rare + Lymphoma - - - - - + - - Peripheral primitive neuroectodermal tumour - - + Rare - - + - Small-cell melanoma - - + - - - - - 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 British Association of Dermatology July 2007 Immunohistochemistry Cytokeratin 20 MerkelCell Cell Carcinoma Carcinoma Merkel • Rare highly malignant neuroendocrine skin tumour • USA - 0.23 / 100 000 •Male predominance •Average age at presentation – 69y 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 Clinical Diagnosis • Clinical diagnosis is difficult • Red / violaceous nodule • May resemble an SCC / BCC or amelanotic melanoma Prognostic markers Poor Prognostic Markers • Advanced stage at presentation Good Prognostic Markers • CD+ Lymphocyte invasion of the tumour • Immunosupression • Prescence of Polyomavirus MCPyV MCV Clonally integrated in 80% of MCC Retrospective Analysis of MCC over 10years 14 cases identified SSMDT Barts Health NHS Trust Results: Demographics 8% 25% Buttock Head Upper limbs Lower Limbs 50% 17% 9 female, 5 male Mean age of presentation: 73years (range 54 to 87years) 21% presented with metastatic disease Results: Treatment INTERVENTION Frequency WLE with 1-3cm margins ALL Adjunctive Radiotherapy Majority (57%) Sentinel Lymph Node Biopsy Few (14%) Adjunctive Chemotherapy •Enrolled onto MCC-1 Trial 1 Polyomavirus • 10/14 tested • 80% had positive polyomavirus • Positive virus did not correlate with outcome 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 Radiotherapy induced skin cancers • BCC - 3.6% • SCC - 0.003% • Angiosarcoma • Fibrosarcoma • Melanoma • Merkel cell carcinoma British Association of Dermatology July 2007 Guidelines Germany France USA WLE margin 3cm 2-3cm Not specified Radiotherapy to surgical bed Yes Yes Yes SLND Yes Yes Not obligatory Adjunct Chemotherapy No No Yes- enrol in Clinical trials Chemotherapy in Metastases Yes Yes Yes Follow-up 5-years 5years Minimum 3years UK 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