关键词: CYLD gene Cylindroma Scalp irradiation Trichoepithelioma Turban tumor

Mesh : Female Humans Aged Skin Neoplasms / pathology Neoplastic Syndromes, Hereditary / pathology Carcinoma, Adenoid Cystic / pathology surgery

来  源:   DOI:10.1007/s00066-023-02077-7

Abstract:
To describe the case of successful radiotherapeutic treatment of a woman suffering from Brooke-Spiegler syndrome who had multiple disfiguring cylindromas on the entire scalp and further tumors on the trunk.
After decades of treatment with conventional therapies including surgery and topically applied salicylic acid, the 73-year-old woman agreed to undergo radiotherapeutic treatment. She received 60 Gy to the scalp and 36 Gy to painful nodules in the lumbar spine region.
Over a follow-up period of 14 and 11 years, respectively, the scalp nodules almost completely regressed, while the lumbar nodules became painless and considerably smaller. Apart from alopecia, no late adverse effects of treatment remain.
This case should remind us of the potential role that radiotherapy could play in treating Brooke-Spiegler syndrome. The required dose for treatment of such extensive disease is still a matter of debate due to the scarcity of radiotherapeutic experience. This case demonstrates that for scalp tumors, 30 × 2 Gy can result in long-term tumor control, while other dose prescriptions may be adequate for tumors in other locations.
摘要:
目的:描述一名患有Brooke-Spiegler综合征的妇女成功接受放射治疗的情况,该妇女在整个头皮上有多个毁容性圆柱瘤,在躯干上有进一步的肿瘤。
方法:经过数十年的常规治疗,包括手术和局部应用水杨酸,这位73岁的妇女同意接受放射治疗。她的头皮接受了60Gy,腰椎区域的疼痛结节接受了36Gy。
结果:经过14年和11年的随访,分别,头皮结节几乎完全消退,而腰椎结节变得无痛且小得多。除了脱发,治疗没有后期不良反应。
结论:该病例应提醒我们放疗在治疗Brooke-Spiegler综合征中的潜在作用。由于缺乏放射治疗经验,治疗这种广泛疾病所需的剂量仍然存在争议。这个病例表明,对于头皮肿瘤,30×2Gy可导致肿瘤的长期控制,而其他剂量处方可能足以治疗其他部位的肿瘤。
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