Dermatologic Clinic, Department of Biomedical Sciences and Human Oncology, University of Turin, Turin, Italy.
Cancer. 2012 Dec 1;118(23):5830-9. doi: 10.1002/cncr.27627. Epub 2012 Jun 6.
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.
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.
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 .
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.
蕈样肉芽肿（MF）是一种惰性原发性皮肤 T 细胞淋巴瘤。据作者所知，目前尚无关于随着时间推移，疾病进展特定途径的风险演变的数据。
本回顾性研究分析了 1975 年至 2010 年间在 27 个意大利皮肤淋巴瘤研究组中心诊断并随访的 1422 例 MF 患者。主要目的是确定皮肤/皮肤外疾病进展的时间过程、途径和危险风险趋势；评估不同的肿瘤-淋巴结-转移-血液（TNMB）分期是否具有不同的疾病进展途径；并分析肿瘤分期和红皮病 MF 之间在临床发病、疾病演变和预后方面的差异。次要目标是为修订后的国际皮肤淋巴瘤学会（ISCL）和欧洲癌症研究与治疗组织（EORTC）皮肤淋巴瘤工作组（CTF）分类提供进一步验证。
中位随访时间为 14.5 年；29.7%的患者发生了疾病进展，血液受累是最常见的皮肤外疾病进展部位。IA 期至 IB 期疾病患者肿瘤期疾病进展的年发生率稳定在 1%-2%；IIA 期疾病患者在前几年的风险较高（高达 9.4%）。红皮病从斑块/斑块（13.9%/28.2%）演变而来的频率明显高于肿瘤（P =.028 和 P =.013）。皮肤外受累的危险率较低（<1%）。T 评分与皮肤外受累部位、肿瘤期疾病伴淋巴结/内脏病变、红皮病伴血液受累有关。TNMB 分类和疾病进展是独立的预后因素，在多变量分析中被检测到；皮肤外受累的类型被发现影响生存。
本研究的数据支持需要进行基于分期的随访，建议对低于红皮病的肿瘤期疾病的分类进行修改，并为修订后的 TNMB 分类提供进一步验证。
