Merkel cell carcinoma in France: a registries-based, comprehensive epidemiological survey.

BACKGROUND: Merkel cell carcinoma (MCC) is a rare primary cutaneous neuroendocrine carcinoma. Owing to its low incidence, epidemiological data are scarce and have never been analysed in France to identify the main epidemiological trends. METHODS: Data from MCC patients diagnosed between 1998 and 2010 were obtained from 11 French cancer registries in the FRANCIM network. The main epidemiological characteristics of MCC were investigated between 2006 and 2010 because comprehensive data were only available for this period. The main focus was tumour incidence and mortality over time. RESULTS: Between 1998 and 2010, 562 cases of MCC were reported in the registries. From 2006 to 2010 (290 cases), European- and world-standardized incidence rates were 0.26 and 0.43 per 100,000 person-years in men and 0.24 and 0.38 per 100,000 person-years in women. MCC is more frequent in females in France (56.9%) with male/female ratio 1.1. Relative survival rates were 84%, 56% and 42% at one, three and 5 years, respectively. CONCLUSIONS: The incidence of MCC clearly increased over time in all areas under focus. The standardized incidence in France was comparable to the incidence observed in other countries for the same period, but French data are too recent to conclude on an increase in MCC incidence. Prognosis remains poor in all countries in which data are available.

[Haemophagocytic syndrome secondary to Mediterranean spotted fever]. / Syndrome d'activation macrophagique secondaire à une fièvre boutonneuse méditerranéenne.

INTRODUCTION: Haemophagocytic syndrome (HS) is a rare disease with a severe prognosis that is defined by clinical, laboratory and histopathological criteria. Infections represent the classical cause of HS. HS secondary to Mediterranean spotted fever (MSF) is rare with only a few cases being reported in the literature. OBSERVATIONS: We report two cases of HS secondary to MSF in 2 men aged 77 and 63 years presenting a febrile maculo-purpuric eruption with inoculation ulcer associated with laboratory abnormalities (cytopenia, elevated ferritin, hypertriglyceridaemia). Haemophagocytosis was present in 2 cases. Serology and PCR for Rickettsia conorii were positive and militated in favour of recent infection responsible for the diagnosis of MSF. DISCUSSION: The first case of HS was described in 1979. Sixteen cases of HS secondary to MSF are described in the literature. Cytopenia associated with hyperferritinaemia and hypertriglyceridaemia strongly suggests MSF complicated by HS. The prognosis depends on the time elapsed since diagnosis and host-specific factors. Immunosuppressants and antibiotics may be necessary to ensure healing. CONCLUSION: Rickettsioses can induce HS, and this potential complication with a severe prognosis must be known.