- [Show abstract] [Hide abstract] ABSTRACT: Merkel cell carcinoma (MCC) is a rare neuroendocrine tumor of the skin. The epidemiological factors strongly associated with this tumor are: age over 65 years, fair skin, chronic sun exposure and immune suppression. Data are sparse in the literature and many questions remain unanswered regarding the diagnosis and treatment of this tumor. To provide clinical practice guidelines for the diagnosis and treatment of MCC. The literature data were analyzed and the current American and German practice guidelines were compared. Consensus items between these two guidelines were adopted as recommendations. Regarding discordant points, a formalized expert consensus process was devised. The guidelines were then written up by an editorial panel and validated by the Cutaneous Oncology Group of the French Society of Dermatology. The guidelines were drawn up according to three levels of scientific evidence: (a) complete agreement between the American and German guidelines; (b) the results of the formalized expert consensus process; and (c) the expert opinion of the steering group, based on analysis of the available evidence. The guidelines presented here are up-to-date recommendations on the clinical and pathological procedures for MCC diagnosis, staging, surgical treatment, sentinel node biopsy, radiotherapy and follow-up. These guidelines for diagnosis and treatment of MCC should standardize MCC management, which may not be optimal in France today.
- [Show abstract] [Hide abstract] ABSTRACT: Photodynamic therapy (PDT) using 5-aminolevulinic acid (5-ALA) is an effective treatment for several conditions such as Bowen's disease, subsets of basal cell carcinomas and actinic keratosis. Surgical resection is the first-choice therapy for extramammary Paget's disease (EMPD), but extensive resection is highly invasive and recurrences are frequent. We report two cases of genital EMPD treated by PDT with partial efficacy. The first patient, a 78-year-old male, suffered from pubic and scrotal Paget's disease for 6 years despite numerous treatments. The second patient, a 78-year-old female, had vulvar involvement for 2 years that was resistant to multiple treatments. The disease was recurrent and chronic with important pruritus and significant impact on the quality of life. Methyl 5-aminolevulinate was applied for 3 h, and irradiation was applied with red light (630 nm) using a total light dose of 37 J/cm(2) for a period of 10 min. The patients were treated every 2 to 4 weeks for a total of at least three treatments. Both patients experienced a partial transient reduction in their symptoms. One patient had a partial transient remission (< 50% reduction of the involved surface), whereas in the other patient, PDT failed to reduce the surface area of the lesions.
- [Show abstract] [Hide abstract] ABSTRACT: Merkel cell carcinoma (MCC) is a rare neuroendocrine carcinoma that originates in the skin. Epidemiological factors strongly associated with MCC are: age over 65 years, fair skin, history of extensive sun exposure and chronic immune suppression. The literature is sparse and many questions currently remain unanswered regarding optimal treatment of MCC. To provide clinical practice guidelines for the diagnosis and treatment of MCC. Literature data were analysed and the existing German and American practice guidelines were compared. Where German and US guidelines were identical, the recommendations in question were adopted. Regarding discordant points, a formalized consensus process was devised. The guidelines were then written by an editorial group and validated by the cutaneous oncology group of the French Society of Dermatology. The guidelines were drawn up according to three levels of scientific evidence: a: complete agreement between the German and American guidelines; b: results of the formalized consensus process; c: expert opinion of the steering group based on available evidence. Our guidelines present up-to-date recommendations on clinical and pathological procedures for diagnosis, staging, surgical treatment, sentinel lymph node biopsy, radiotherapy and follow-up. We created a set of diagnostic and treatment guidelines in order to standardize management of MCC, which may be not optimal throughout France.
- [Show abstract] [Hide abstract] ABSTRACT: Le carcinome à cellules de Merkel est une tumeur neuroendocrine cutanée rare et agressive du sujet âgé, favorisée par le soleil et l’immunodépression. La littérature étant pauvre, la prise en charge diagnostique et thérapeutique est controversée et donc hétérogène.
- [Show abstract] [Hide abstract] ABSTRACT: Introduction: Merkel cell carcinoma (MCC) is a rare form of tumour that develops from neuroendocrine skin cells. There is no consensus concerning diagnosis and therapy for this disease. To establish recommendations, the Skin Oncology Group of the French Dermatology Society drew on existing recommendations in the US and Germany. Concordance between the two sets of recommendations was validated by the group, who, in view of the large number of discrepancies, decided to use the formalised expert consensus method. Methods: Twelve practitioners known for their experience in the management of Merkel cell carcinoma completed a standardised questionnaire. This group consisted of seven dermatologists, three radiotherapists and two plastic surgeons. They answered a total of 82 questions concerning the diagnosis, treatment and follow-up of MCC. A strong consensus was declared where all answers were in the same third of the table; a weak or relative consensus was declared where answers overlapped for adjacent scores, and an absence of consensus was declared in the event of dispersal of values. Results: On completion of this process, the method revealed strong concordance on the following points: value of CK7 and CK20 immunolabelling; lack of value of chest radiography in the initial work-up, but value of lymph node ultrasound; inadequacy of an excision margin of 1cm but lack of any need for a margin greater than 3cm; absence of any value of chemotherapy for inoperable tumours; value of seeking sentinel nodes in tumours measuring less than 2cm outside the head and neck and more than 2cm for head and neck tumours; value of additional radiotherapy at the initial tumour site. Relative agreement was reached concerning the value of thoracic-abdominal-pelvic computed tomography (CT) scans in all cases, together with a head CT scan for cervical-facial tumour sites. Conclusion: The creation of a formalised expert consensus helps complete the existing recommendations, particularly on those points where no consensus exists between the American and German recommendations and where no strong evidence is to be found in the literature.
Hôpital Ambroise Paré – Hôpitaux universitaires Paris Ile-de-France OuestBillancourt, Île-de-France, France
Hôpital Ambroise Paré Paul DesbiefMarsiglia, Provence-Alpes-Côte d'Azur, France