Department of Internal Medicine and Immunology, Erasmus MC University Medical Center Rotterdam, PO Box 2040, 3000 CA, Rotterdam, The Netherlands.
Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands.
Radiat Oncol. 2022 Aug 1;17(1):137. doi: 10.1186/s13014-022-02108-0.
Langerhans cell histiocytosis (LCH) is a rare haematological neoplasm characterized by the accumulation of CD1a, CD207/Langerin histiocytes within inflammatory lesions. LCH can involve any organ, but osteolytic bone lesions are most often encountered. Unifocal bone lesions may regress spontaneously after a thick needle biopsy has been taken.
In this case report, we describe the initial presentation of a single BRAF mutated osteolytic LCH lesion in the left proximal humerus of a 46-year-old previously healthy woman. Despite multiple surgical interventions, she unexpectedly experienced progressive disease manifestation with significant soft tissue extension to the surrounding musculature, subcutis and epidermis. Because the disease manifestation remained loco-regional, radiotherapy (RT) (total dose of 20 Gy in 10 fractions) was initiated.
The patient achieved a complete remission without any side effects. This case highlights that RT is a rational and relative mild local treatment option for patients with aggressive LCH affecting the bone and surrounding soft tissue.
朗格汉斯细胞组织细胞增生症（LCH）是一种罕见的血液系统肿瘤，其特征是在炎症病变中积累 CD1a、CD207/Langerin 组织细胞。LCH 可累及任何器官，但溶骨性骨病变最常发生。在进行粗针活检后，单发骨病变可能会自发消退。
在本病例报告中，我们描述了一位 46 岁健康女性左侧肱骨近端单发 BRAF 突变型溶骨性 LCH 病变的初始表现。尽管进行了多次手术干预，但她仍出现了意外的进行性疾病表现，伴有明显的软组织向周围肌肉、皮下组织和表皮延伸。由于疾病表现仍局限于局部，因此开始进行放疗（RT）（总剂量 20Gy，10 次分割）。
患者无任何副作用达到完全缓解。本病例强调，对于累及骨骼和周围软组织的侵袭性 LCH 患者，RT 是一种合理且相对温和的局部治疗选择。
