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Notes: Summary Primary cutaneous medium and large cell lymphomas (MLCL) other than mycosis fungoides (MF) are rare, and their prognosis and treatment are controversial. The clinical, immunohistological and follow-up data of 54 well-documented cases of primary cutaneous MLCL other than MF, seen in our institutions over a 14-year period, were retrospectively reviewed, in order to determine the prognostic factors related to these lymphomas, and to analyse the results obtained with different treatment regimens. Forty-six patients presented with a solitary tumour or with localized lesions. and eight had disseminated cutaneous lesions. According to the updated Kiel classification, 45 cases (83%) corresponded to B-cell lymphomas: centroblastic lymphomas, 32 cases; centroblastic-centrocytic lymphomas, 11 cases; immunoblastic lymphomas, two cases. Nine cases (17%) were classified as T-cell lymphomas: pleomorphic medium and large cell lymphomas, eight cases; anaplastic large cell lymphoma. one case. Four of eight patients with disseminated skin lesions had a T-cell lymphoma. whereas 41 of 46 patients with a solitary tumour had a B-cell lymphoma. Patients with disseminated skin lesions and elevated serum lactate dehydrogenase (LDH) levels had a poor prognosis. Comparison of patients' overall survival, depending on immunohistological subtype, showed that the median survival of patients with pleomorphic T-cell lymphoma was 2·5 years, whereas it was not reached at 12 years for patients with centroblastic centrocytic and centroblastic lymphoma. The eight patients with disseminated skin lesions were treated with polychemotherapy. Most patients with a solitary tumour or with localized lesions of low tumour bulk were treated by surgical excision or radiotherapy alone, and nine other patients with localized lesions of high tumour bulk were treated with initial polychemotherapy. Clinical presentation (i.e. solitary or disseminated lesions), serum LDH levels, and the immunohistological subtype, are important prognostic factors in cutaneous MLCL. Patients with disseminated skin lesions have a poor prognosis, and should be treated with intensive polychemotherapy regimens, whereas those with a solitary tumour, or with localized lesions of low tumour bulk, are adequately treated by radiotherapy.

Notes: Background  Some cases of dermatofibrosarcoma protuberans (DFSP) do not protrude above the skin.Objectives  To assess the prevalence of these DFSPs and further to describe their presentation and course.Methods  One hundred and forty-three patients were retrospectively collected. They were asked to complete a standardized questionnaire indicating the history and appearance of the DFSP from the first skin changes identified to the time of diagnosis.Results  Eighty-one DFSPs were described as protuberant ab initio, and 62 as initially nonprotuberant (npDFSP). The latter remained at this stage for a mean period of 7·6 years. Twenty-nine per cent of npDFSPs were ‘morphoea-like’, 19% were ‘atrophoderma-like’ and 42% were ‘angioma-like’. Age at diagnosis was similar for both initial presentations. npDFSPs were most often misdiagnosed by physicians.Conclusions  Nearly half the patients first identified their early DFSP-related skin changes as patches. Both this frequency and the long duration at this preprotuberant stage should prompt dermatologists to consider the diagnosis of DFSP earlier, in order to make surgical treatment easier.