Kim Esther J, Aria Alexander B, Wilmas Kelly, Lewis Daniel J, Torres-Cabala Carlos A, Nunez Cesar, Dabaja Bouthaina S, Duvic Madeleine
School of Medicine, Baylor College of Medicine, Houston, Texas.
Department of Dermatology, The University of Texas MD Anderson Cancer Center, Houston, Texas.
Pediatr Dermatol. 2019 Jan;36(1):e23-e26. doi: 10.1111/pde.13728. Epub 2018 Dec 11.
Primary cutaneous CD4+ small- to medium-sized pleomorphic T-cell lymphoproliferative disorder (PCSM-LPD) is a rare and low-grade form of cutaneous T-cell proliferation with the average age of diagnosis of 54 years. Because of its rarity, the etiology or exact clinicopathology of PCSM-LPD remains unclear, with < 10 pediatric cases reported. A 13-year-old boy presented to our clinic with a raised tumor with PCSM-LPD histology and was successfully treated with ultra-low-dose radiation therapy. While no standard of care has been established for pediatric PCSM-LPD, this report represents an example of achieving remission in a pediatric tumor with minimal potential for therapy-related long-term toxicity.
原发性皮肤CD4⁺中小多形性T细胞淋巴增殖性疾病（PCSM-LPD）是一种罕见的低度皮肤T细胞增殖形式，平均诊断年龄为54岁。由于其罕见性，PCSM-LPD的病因或确切临床病理学仍不清楚，报道的儿科病例<10例。一名13岁男孩因组织学表现为PCSM-LPD的隆起肿瘤前来我院就诊，并成功接受了超低剂量放射治疗。虽然尚未为儿科PCSM-LPD确立标准治疗方案，但本报告展示了一例儿科肿瘤实现缓解且治疗相关长期毒性可能性最小的病例。
