Time course, clinical pathways, and long‐term hazards risk trends of disease progression in patients with classic mycosis fungoides

Abstract

BACKGROUND: Mycosis fungoides (MF) is an indolent primary cutaneous T‐cell lymphoma. To the authors' knowledge, no data currently are available regarding the evolution over time of the risk of developing specific pathways of disease progression. METHODS: This retrospective study analyzed 1422 patients with MF who were diagnosed and followed from 1975 through 2010 in 27 Italian Study Group for Cutaneous Lymphoma centers. The primary objectives were to ascertain the time course, pathways, and hazards risk trends of cutaneous/extracutaneous disease progression; to evaluate whether different tumor‐lymph node‐metastasis‐blood (TNMB) stages have different pathways of disease progression; and to analyze differences between tumor‐stage and erythrodermic MF with regard to clinical onset, disease evolution, and prognosis. The secondary objective was to provide a further validation for the revised International Society for Cutaneous Lymphomas and the Cutaneous Lymphoma Task Force of the European Organization of Research and Treatment of Cancer (ISCL/EORTC) classification. RESULTS: The median follow‐up was 14.5 years; stage progression occurred in 29.7% of patients and blood involvement was the most frequent extracutaneous site of disease progression. Patients with stage IA to stage IB disease demonstrated a steady low annual incidence of disease progression to tumor‐stage (1%‐2%); patients with stage IIA disease had a higher risk within the first years (up to 9.4%). Erythroderma evolved with a significantly higher frequency from patches/plaques (13.9%/28.2%) than tumors (P = .028 and P = .013, respectively). Hazards rates of extracutaneous involvement were low (< 1%). The T‐score was found to be associated with extracutaneous involvement site, tumor‐stage disease with lymph node/visceral lesions, and erythroderma with blood involvement. TNMB classification and stage progression resulted as independent prognostic variables being detected on multivariate analysis; the type of extracutaneous involvement was found to affect survival . CONCLUSIONS: The data from the current study support the need for a stage‐tailored follow‐up, suggest that the classification of tumor‐stage disease at a stage below erythroderma could be modified, and offer a further validation for the revised TNMB classification. Cancer 2012. © 2012 American Cancer Society.

Prognostic indicators for mycosis fungoides in a Greek population

“…According to the largest studies to date, early‐stage disease affects 71·5–76% of patients diagnosed with MF. Our findings are in line with those of Quaglino et al ., in which 87·1% of their Italian cohort presented with early‐stage disease at the time of diagnosis . It should be further investigated whether these results are due to possible genetic differences of Mediterranean populations or whether environmental parameters – such as high ambient sun exposure – may suppress disease appearance, especially in younger patients.…”

miR‐155 is involved in tumor progression of mycosis fungoides

“…The reason for this discrepancy with our data can be attributed to different patients selection, among which is that only 7 tumor-stage cases where included (15), while the vast majority of the remaining 18 cases of the advance stage consisted of erythrodermic MF and SS. It should be noted that the progression from patch/plaque stage is almost always to tumor stage, while other advanced stages of MF, specifically, eryhtrodermic MF and SS usually develop de novo (20).…”

Doxycycline is an NF-κB inhibitor that induces apoptotic cell death in malignant T-cells

“…Although the median survival can vary from 10-35 years in those with early stage disease, this drastically reduces to only 1.5 years for those with advanced staged disease [3]. Additionally, one in four people with early stage disease progress to more advanced stages with a drastic reduction in overall survival rate to 4 years [4, 5]. …”