60 y.o. Female. Tumour on the right upper arm. Non-melanoma skin cancer? CASE 27.
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Transcript of 60 y.o. Female. Tumour on the right upper arm. Non-melanoma skin cancer? CASE 27.
British Association of Dermatology July 2007
CK20: cytokeratin 20CK7: cytokeratin 7NSE: neurone-specific enolaseNFP: neurofilament proteinS100: S100 proteinLCA: leucocyte common antigenCD99: cluster-of-differentiation antigen 99TTF1: thyroid transcription factor 1+: positive stain –: negative stain
Tumour CK20 CK7 NSE NFP S100 LCA CD99 TTF1
Merkel-cell carcinoma + - + + - - Rare -
Small-cell carcinoma of lung - + + +/- - - Rare +
Lymphoma - - - - - + - -
Peripheral primitive neuro-ectodermal tumour
- - + Rare - - + -
Small-cell melanoma - - + - - - - -
Immunolabelling Profiles
Merkel Cell Carcinoma
• Rare highly malignant neuroendocrine skin tumour
• USA - 0.23 / 100 000
•Male predominance
•Average age at presentation – 69y
Merkel Cell Carcinoma
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
• Clinical diagnosis is difficult
• Red / violaceous nodule
• May resemble an SCC / BCC or amelanotic melanoma
Clinical Diagnosis
Prognostic markers
• Advanced stage at presentation
• Immunosupression
Poor Prognostic Markers
Good Prognostic Markers
• CD+ Lymphocyte invasion of the tumour
• Prescence of Polyomavirus
Results: Demographics
9 female, 5 male
Mean age of presentation: 73years (range 54 to 87years)
21% presented with metastatic disease
25%
17%50%
8%
Buttock
Head
Upper limbs
Lower Limbs
Results: TreatmentINTERVENTION 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
British Association of Dermatology July 2007
Radiotherapy induced skin cancers
• BCC - 3.6%
• SCC - 0.003%
• Angiosarcoma
• Fibrosarcoma
• Melanoma
• Merkel cell carcinoma
Guidelines
Germany France USA UK
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
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