Complete remission of disseminated Langerhans cell sarcoma after stem cell transplantation
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With fewer than 100 cases described in the English literature so far, Langerhans cell sarcoma represents an orphan malignant disease deriving from histiocytic cells. Clinical course is extremely aggressive and associated with poor survival rates, especially in disseminated condition. Herein, we describe the case of a young patient with fulminant development of metastatic Langerhans cell sarcoma, who achieved persistent remission after polychemotherapy and subsequent high-dose chemotherapy with autologous stem cell transplantation.
KeywordsAutologous stem cell transplantation Chemotherapy
Considering the young patient age, good pre-existing general condition and disseminated illness, we decided for subsequent polychemotherapy and stem cell transplantation. In September 2017, the patient received a preliminary phase with vinblastine (3 mg/m2 on day 1) and dexamethasone (40 mg on days 1–3), followed by primary chemotherapy with six cycles of cyclophosphamide (750 mg/m2 on day 1), doxorubicin (50 mg/m2 on day 1), vincristine (1.4 mg/m2 on day 1), and prednisolone (40 mg/m2 on days 1–5) (CHOP). The first cycle already led to a good partial remission (Fig. 1b and 2b); oxygen demand was reduced to two liters per minute. After reaching complete remission and successful stem cell apheresis, high-dosage therapy with carmustine (300 mg/m2 on day 1), cytarabine (400 mg/m2 on days 2–5), etoposide (200 mg/m2 on days 1–5), and melphalane (120 mg/m2 on day 6) with subsequent autologous stem cell transplantation was administered in May 2018. At the present time, one year later, the patient is in excellent general condition without oxygen demand and complete remission is still persisting (Fig. 1c and 2c).
Critical discussion and review of the literature
Langerhans cell sarcoma (LCS) is a rare malignant disease deriving from antigen-presenting cells within the histiocytic system . Given its extreme scarcity with less than 100 cases reported in the English literature so far, there is a lack of evidence regarding the most appropriate treatment regimen .
In contrast to Langerhans cell histiocytosis (LCH), LCS is characterized by an aggressive clinical course and overtly malignant cytologic features . The most frequent sites of presentation are lymph nodes (75%), followed by the skin (50%), lung, liver, and spleen . Langerhans cells typically show longitudinally grooved nuclei and Birbeck granules and are confirmed by positive immunohistochemical staining for CD1a, CD207 (Langerin) and S‑100 protein . Overall survival in patients with LCS is reported for 58% at 1 year and 28% at 5 years. With increase of tumor burden survival rates even deteriorate significantly. Until now, no case achieving 5‑year survival in disseminated disease has been described .
Treatment options. Data adopted from 
Proportion of treated patients (%)
Stem cell transplantation
Langerhans cell sarcoma is an extremely rare disease associated with poor clinical outcome. Due to a lack of data, standard treatment regimens have not been established yet. With this case report, we want to emphasize the role of high-dose chemotherapy and autologous stem cell transplantation as a curative treatment option for patients with disseminated disease. In addition, the case supports the necessity for a rapid clarification of diagnosis in such situations.
Open access funding provided by Paracelsus Medical University.
Conflict of interest
V. Schlintl, K. Schlick, G. Russ, S. Gampenrieder, C. Monzo Fuentes, T. Kos, F. Renneberg, L. Rettenbacher, J. Gradl, K. Sotlar, A. Hoffmann and R. Greil declare that they have no competing interests.
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