Etter Emily, Bosse Benjamin, Wang Yi Peng, Hiniker Susan, Oh Justin
University of Nevada Reno School of Medicine Reno Nevada USA.
Department of Radiation Oncology Stanford University School of Medicine Stanford California USA.
Clin Case Rep. 2023 Jun 19;11(6):e7506. doi: 10.1002/ccr3.7506. eCollection 2023 Jun.
Langerhans cell histiocytosis (LCH) is a rare inflammatory myeloid neoplasm arising from the proliferation of pathologic Langerhans cells. LCH has a spectrum of presentations predominantly affecting male pediatric patients. As LCH is a relatively uncommon diagnosis, there is no standard of care for treatment of the disease and treatment is based largely on clinical judgment, lesion characteristics, and symptoms at presentation. Here we present a case of unifocal, isolated orbital LCH in a 19-year-old young man treated initially with surgical resection. Follow-up imaging 2 months later demonstrated significant regrowth of the mass and no other sites of disease. The recurrent orbital disease was treated with stereotactic radiosurgery (SRS) to 7 Gy in one fraction. Near complete resolution of the mass was achieved with no recurrence after 1.5 years of follow-up. SRS for treatment of orbital LCH is a novel treatment not previously described in the literature which may provide benefit in select cases.
朗格汉斯细胞组织细胞增多症(LCH)是一种罕见的炎症性髓系肿瘤,由病理性朗格汉斯细胞增殖引起。LCH有一系列表现,主要影响男性儿科患者。由于LCH是一种相对不常见的诊断,目前尚无该疾病的标准治疗方案,治疗主要基于临床判断、病变特征和就诊时的症状。在此,我们报告一例19岁青年男性的单灶性、孤立性眼眶LCH病例,最初接受手术切除治疗。2个月后的随访影像学检查显示肿块显著复发,且无其他疾病部位。复发性眼眶疾病采用立体定向放射外科(SRS)单次给予7 Gy治疗。随访1.5年后,肿块几乎完全消退,无复发。SRS治疗眼眶LCH是一种文献中未曾描述过的新治疗方法,可能在某些病例中具有益处。