Yi Wu, Chen Wan-Yuan, Yang Tian-Xin, Lan Jian-Ping, Liang Wen-Na
Department of Haematology.
Department of Pathology.
Medicine (Baltimore). 2019 Mar;98(10):e14531. doi: 10.1097/MD.0000000000014531.
Langerhans cell sarcoma (LCS) is a rare, high-grade neoplasm characterized by overtly malignant cytologic features and a poor prognosis. Herein, we present a rare case of langerhans cell histiocytosis (LCH) that later transformed into langerhans cell sarcoma 11 months after the benign mass was excised from soft tissue in the right groin.
A 41-year-old patient who presented with a mass in the right groin for 3 years earlier after being bitten by ants.
The patient was diagnosed with langerhans cell sarcoma arising from antecedent langerhans cell histiocytosis.
The patient underwent with 6 cycles of a modified etoposide, cyclophosphamide, vindesine, dexamethasone (E-CHOP) regimen.
The patient is currently receiving follow-up care.
LCH transformed into LCS is a rare case. E-CHOP as an effective first-line therapy to treat LCS cases, but, the mechanism is unclear. Due to their rarity, further data on clinical outcomes are necessary to establish the optimal treatment strategy for LCS.
朗格汉斯细胞肉瘤(LCS)是一种罕见的高级别肿瘤,具有明显的恶性细胞学特征且预后较差。在此,我们报告一例罕见的朗格汉斯细胞组织细胞增多症(LCH)病例,该病例在右侧腹股沟软组织的良性肿块切除11个月后转变为朗格汉斯细胞肉瘤。
一名41岁患者,3年前被蚂蚁叮咬后右侧腹股沟出现肿块。
该患者被诊断为源于先前朗格汉斯细胞组织细胞增多症的朗格汉斯细胞肉瘤。
患者接受了6个周期的改良依托泊苷、环磷酰胺、长春地辛、地塞米松(E-CHOP)方案治疗。
患者目前正在接受随访。
LCH转变为LCS是罕见病例。E-CHOP作为治疗LCS病例的有效一线疗法,但其机制尚不清楚。由于其罕见性,需要更多关于临床结果的数据来确定LCS的最佳治疗策略。