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.
aDepartment of Haematology
bDepartment of Pathology
cDepartment of Nephrology, Zhejiang Provincial People's Hospital, People's Hospital Of Hangzhou Medical College, Hangzhou, China.
Correspondence: Wen-na Liang, Department of Nephrology, Zhejiang Provincial People's Hospital, People's Hospital Of Hangzhou Medical College, Hangzhou 310000, China (e-mail: firstname.lastname@example.org).
Abbreviations: 2-CDA = 2-chlorodeoxyadenosine, ALK = anaplastic lymphoma kinase, CHOP = cyclophosphamide, vindesine, dexamethasone, E-CHOP = etoposide, cyclophosphamide, vindesine, dexamethasone, ESHAP = etoposide, carboplatin, cytarabine, methylprednisolone, FDG–PET = fluorine-18 fluorodeoxyglucose positron emission tomography, LCH = langerhans cell histiocytosis, LCS = langerhans cell sarcoma, MUM-1 = melanoma associated antigen-1.
Informed written consent was obtained from the patient for publication of this case report and accompanying images.
This article was supported by Funds of Zhejiang Provincial Natural Science Foundation of China (No. LY19H160037), Science Technology Department of Zhejiang Province (No. 2017KY228).
The authors have no conflicts of interest to disclose.
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Received October 3, 2018
Received in revised form January 11, 2019
Accepted January 23, 2019