A randomized trial to improve early detection and prevention practices among siblings of melanoma patients

Abstract

Disease overview POEMS syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. Diagnoses are often delayed because the syndrome is rare and can be mistaken for other neurologic disorders, most commonly chronic inflammatory demyelinating polyradiculoneuropathy. POEMS syndrome should be distinguished from the Castleman disease variant of POEMS syndrome, which has no clonal PCD and typically little to no peripheral neuropathy but has several of the minor diagnostic criteria for POEMS syndrome. Diagnosis: The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma cell disorder, and at least one of the minor criteria. Risk stratification: Because the pathogenesis of the syndrome is not well understood, risk stratification is limited to clinical phenotype rather than specific molecular markers. The number of clinical criteria is not prognostic, but the extent of the plasma cell disorder is. Those patients with an iliac crest bone marrow biopsy that does not reveal a plasma cell clone are candidates for local radiation therapy; those with a more extensive or disseminated clone will be candidates for systemic therapy. Risk‐adapted therapy: For those patients with a dominant sclerotic plasmacytoma, first line therapy is irradiation. Patients with diffuse sclerotic lesions or disseminated bone marrow involvement and for those who have progression of their disease 3 to 6 months after completing radiation therapy should receive systemic therapy. Corticosteroids are temporizing, but alkylators are the mainstay of treatment, either in the form of low dose conventional therapy or high dose with stem cell transplantation. The benefit of anti‐VEGF antibodies is conflicting. Lenalidomide shows promise with manageable toxicity. Thalidomide and bortezomib also have activity, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes. Am. J. Hematol. 86:592–601, 2011. © 2011 Wiley‐Liss, Inc.

Screening, early detection, and trends for melanoma: Current status (2000-2006) and future directions

“…The number of participants in both groups that received a skin cancer examination more than doubled, with no differences between intervention and control subjects. 92 Earlier surveys on beliefs and practices regarding skin cancer prevention and detection among 404 siblings of recently diagnosed patients with melanoma found that only 27% had received a skin cancer examination by a dermatologist during the past year; 47% had never received a dermatologic examination. Multivariate analysis found modifiable positive predictors for SSE and dermatologist examinations, including having a clinician with whom to talk about melanoma and believing in the importance of regular skin examinations by a physician.…”

State of the science 60th anniversary review

“…Geller et al demonstrated in a randomized control trial that siblings of melanoma patients who had received an intervention were more likely to examine all their moles 12 months later, including the ones on their backs. 47 Skin selfexamination has a role in reducing melanoma mortality; as Berwick et al demonstrated, it could potentially reduce mortality related to melanoma by 63%. 48 Indeed, in a study in New York, skin selfexamination was found to be a key predictor of presentation with a melanoma <1 mm in thickness.…”

Melanoma survivorship: research opportunities

“…In a study of unaffected first-degree relatives of individuals diagnosed with melanoma, it was reported that less than one-third practiced sun protective behaviors such as using sunscreen routinely, staying in the shade, and wearing protective clothing frequently [7]. Furthermore, in an intervention study in which materials and telephone counseling were provided to first-degree relatives of melanoma patients, Geller et al [40] determined that the intervention lead to significantly higher rates of skin selfexamination among relatives who received the materials and counseling compared with relatives who underwent usual care with no intervention. First-degree relatives of melanoma patients, who are at particularly high risk, warrant targeted public health action.…”