Connective tissue responses in blacks in relation to disease: further observations

Additional information is presented in support of the hypothesis (Polednak: Am. J. Phys. Anthropol. 41:49-58, 1974) that in some black populations certain connective-tissue responses, which are involved in protection against infection and repair after injury, also may predispose to specific chronic diseases. These diseases include some autoimmune disorders (i.e., systemic lupus erythematosus, sarcoidosis, and scleroderma) and various benign and malignant tumors involving connective-tissue cells. Complex interactions between genetic factors (HLA and non-HLA loci) and environmental agents may be involved both in the etiology of these autoimmune diseases and in population differences in the incidence of these diseases. A framework is reviewed whereby cellular responses to infectious agents, involving chiefly immunoglobulin-producing cells and macrophages, may have consequences in terms of pathogenesis of specific chronic diseases more common in some black populations. The possible role of natural selection in maintaining some of these diseases is also considered, along with the need for involvement of biomedical anthropologists in their investigation.     

Trends in incidence and survival analysis in non-melanoma skin cancer from 1994 to 2012 in Girona,Spain: A population-based study

IntroductionWe present an epidemiological study focused on Non-melanoma skin cancers (NMSC), including squamous cell carcinoma (SCC), basal cell carcinoma (BCC), Merkel cell carcinoma (MCC), dermatofibrosarcoma protuberans (DFS) and adnexal and skin appendages neoplasm (ASAN), a neoplasm understudied in cancer registries.Material and methodsWe analyze trends of incidence and survival of NMSC registered with the Cancer Registry of Girona, Spain.ResultsWe found 14389 cases of NMSC, accounting 3,474 SCC, 10729 BCC, 33 MCC, 61 DFSP and 71 ASAN. Incidence increased significantly in SCC and BCC with annual percentage of change of 1.6 and 1.5, respectively, but not in MCC, DFS or ASAN. Five-year relative survival for both sexes was 90.1% in SCC, 99.8% in BCC, 44.2% in MCC, 93.7% in DFS and 84% in ASAN.ConclusionsOur study confirms the increasing incidence and good survival of SCC and BCC and enhances knowledge on the epidemiology of the less incidental NMSC.     

Genetics of rare mesenchymal tumors: Implications for targeted treatment in DFSP,ASPS, CCS,GCTB and PEComa

Soft tissue and bone sarcomas comprise a heterogeneous group of mesenchymal tumors that include roughly 130 distinct diagnostic entities. Many of them are exceptionally rare, with only few cases diagnosed worldwide each year. Development of novel targeted treatment in this group of tumors is of special importance since many sarcoma subtypes are resistant to conventional chemotherapy and the effective therapeutic options are limited. In this review we aim to discuss the molecular implications for targeted therapy in selected rare soft tissue and bone sarcoma subtypes, including dermatofibrosarcoma protuberans (DFSP), alveolar soft part sarcoma (ASPS), clear cell sarcoma (CCS), giant cell tumor of bone (GCTB) and perivascular epithelioid cell neoplasms (PEComas).This article is part of a Directed Issue entitled: Rare cancers.     

Cryotherapy for local recurrent dermatofibrosarcoma protuberans: Experience in 19 patients

Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive, cutaneous, malignant tumor characterized by a high propensity for local relapse. Wide and deep local excision with reconstructive surgery is the current standard therapy for DFSP, with a local recurrence rate (LRR) of nearly 40%. In this study, we cured 19 patients with local recurrence of DFSP with 39 sessions of percutaneous cryoablation performed between July 2004 and August 2008. The LRRs after one, two and three cryosurgery sessions per patient were 68%, 54% and 0%, respectively. Moreover, the LRR did not differ with tumor location or size. Furthermore, all patients had a progression-free survival of >5 years. Only minor complications such as fever, local edema, mild nerve injury and local pain occurred, and were resolved within 1 week with symptomatic treatment. In our experience, percutaneous cryoablation is a relatively safe and efficient technique for the treatment of local recurrence of DFSPs.