Johnstone Philippa, Higgins Martin, Prince H Miles, Lade Stephen, McCormack Chris, van der Weyden Carrie, Bhabha Friyana, Buelens Odette, Blombery Piers, Campbell Belinda A
Department of Radiation Oncology, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia.
Sir Peter MacCallum Department of Oncology, University of Melbourne, Parkville, Victoria, Australia.
Br J Haematol. 2025 Sep;207(3):824-833. doi: 10.1111/bjh.20225. Epub 2025 Jun 25.
Large-cell transformation of mycosis fungoides (LCTMF) is rare, histologically distinct, with an aggressive clinical course; yet is not recognised as an independent entity in classification systems nor in staging systems for mycosis fungoides and Sézary syndrome (MF/SS). Herein, the patterns of care and survival outcomes for patients with LCTMF are described, with prognosis compared to published data of non-transformed MF/SS. Eligibility required clinicopathological diagnosis of LCTMF (1/1/1990-31/10/2021), managed at Peter MacCallum Cancer Centre. Eighty-three patients were eligible. Median follow-up was 8.0 years. At the time of LCTMF, 36% had early-stage MF (IA-IIA), 76% had cutaneous-only LCTMF. The most common first-line treatments were localised radiotherapy (48%) and multiagent chemotherapy (23%). Median overall survival (OS) from LCTMF diagnosis was 3.5 years (95% [confidence interval] CI: 2.2-8.2). Three prognostic groups of LCTMF were identified: unifocal cutaneous only, multifocal cutaneous only and extracutaneous (median OS: 4.6, 2.5 and 1.1 years, respectively; p = 0.005). Unfavourable prognostic factors were advanced age and extracutaneous LCTMF. In conclusion, treatment pathways for patients with LCTMF were varied, and prognosis was poor, despite >1/3 having early-stage MF. However, differences in prognosis were suggested, with unifocal cutaneous LCTMF associated with greater OS. Given prognostic differences from MF/SS, consideration to include LCTMF in staging systems is warranted.
蕈样肉芽肿大细胞转化（LCTMF）罕见，组织学特征明显，临床病程侵袭性强；然而，在蕈样肉芽肿和塞扎里综合征（MF/SS）的分类系统和分期系统中，它都未被视为一个独立的实体。本文描述了LCTMF患者的治疗模式和生存结果，并将其预后与非转化型MF/SS的已发表数据进行了比较。入选标准为在彼得·麦卡勒姆癌症中心接受治疗的LCTMF患者的临床病理诊断（1990年1月1日至2021年10月31日）。83例患者符合条件。中位随访时间为8.0年。在LCTMF发生时，36%的患者处于MF早期（IA-IIA期），76%的患者仅为皮肤型LCTMF。最常见的一线治疗方法是局部放疗（48%）和多药联合化疗（23%）。从LCTMF诊断开始的中位总生存期（OS）为3.5年（95%[置信区间]CI：2.2-8.2）。确定了LCTMF的三个预后组：仅单灶皮肤型、仅多灶皮肤型和皮肤外型（中位OS分别为4.6年、2.5年和1.1年；p=0.005）。不良预后因素为高龄和皮肤外LCTMF。总之，LCTMF患者的治疗途径各不相同，预后较差，尽管超过1/3的患者处于MF早期。然而，提示预后存在差异，单灶皮肤型LCTMF的总生存期更长。鉴于与MF/SS的预后差异，有必要考虑将LCTMF纳入分期系统。
