Campbell Belinda A, Prince H Miles, Thursky Karin, Dabaja Bouthaina, Hoppe Richard, Specht Lena, Morris Stephen, Porceddu Sandro V
Department of Radiation Oncology, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia.; The Sir Peter MacCallum Department of Oncology, The University of Melbourne, Parkville, Victoria, Australia; Department of Clinical Pathology, The University of Melbourne, Parkville, Victoria, Australia.
The Sir Peter MacCallum Department of Oncology, The University of Melbourne, Parkville, Victoria, Australia; Department of Haematology, Peter MacCallum Cancer Centre and Royal Melbourne Hospital, Melbourne, Victoria, Australia.
Semin Radiat Oncol. 2025 Jan;35(1):110-125. doi: 10.1016/j.semradonc.2024.08.005.
Cutaneous T-cell lymphomas (CTCL) are a rare collection of diseases, frequently associated with diagnostic challenges and complex management dilemmas. The multidisciplinary team is vital for accurate clinico-pathological diagnoses and for collaborative therapeutic decisions throughout the management journey, which frequently involves multiple lines of therapy. Radiotherapy (RT) is a highly effective skin-directed therapy for CTCL, commonly delivered as localised fields or as total skin electron beam therapy (TSEBT). Mycosis fungoides (MF) is the most common of the CTCL, and patients typically experience high rates of morbidity and long natural histories of relapse and progression. Patients with MF typically present with incurable disease; in these patients, RT has an established role in symptom- and disease-control, achieving excellent response rates and proven therapeutic benefits. The role of RT continues to evolve, with modern practices favouring lower doses to reduce toxicity risks and allow for re-irradiation. Less commonly, there are situations where RT has an integral role in the potential cure of patients with MF: firstly, in the setting of unilesional MF where localised RT alone may be curative, and secondly, in the setting of preconditioning prior to curative-intent allogeneic hematopoietic stem cell transplant for patients with advanced MF/Sezary syndrome, where conventional-dose TSEBT is indicated as the most effective single agent for maximal debulking of skin disease. Radiotherapy also has an important role in the management of the less common CTCL, including the curative treatment of localised primary cutaneous anaplastic large cell lymphoma. Despite proven efficacy and quality of life benefits, disparity exists in access to RT and TSEBT. World-wide, stronger multidisciplinary collaborations and greater patient advocacy are required to increase access to RT and improve equity of care for our patients with CTCL.
皮肤T细胞淋巴瘤(CTCL)是一类罕见的疾病,常伴有诊断挑战和复杂的管理难题。多学科团队对于准确的临床病理诊断以及在整个管理过程中的协作治疗决策至关重要,这通常涉及多种治疗方案。放射治疗(RT)是一种针对CTCL的高效皮肤定向治疗方法,通常以局部照射野或全身皮肤电子束治疗(TSEBT)的形式进行。蕈样肉芽肿(MF)是CTCL中最常见的类型,患者通常发病率高,复发和进展的自然病程长。MF患者通常表现为无法治愈的疾病;在这些患者中,RT在症状控制和疾病控制方面具有既定作用,能取得优异的缓解率并证明有治疗益处。RT的作用不断演变,现代实践倾向于采用较低剂量以降低毒性风险并允许再次照射。较少见的情况是,RT在MF患者的潜在治愈中也起着不可或缺的作用:首先,在单病灶MF的情况下,单独的局部RT可能具有治愈效果;其次,在晚期MF/Sezary综合征患者进行根治性异基因造血干细胞移植前的预处理中,传统剂量的TSEBT被认为是最大程度减轻皮肤疾病负担的最有效单一药物。放射治疗在较罕见的CTCL管理中也具有重要作用,包括局部原发性皮肤间变性大细胞淋巴瘤的根治性治疗。尽管已证明其疗效和对生活质量的益处,但在获得RT和TSEBT方面仍存在差距。在全球范围内,需要加强多学科合作并加大患者宣传力度,以增加RT的可及性并改善我们CTCL患者的护理公平性。