Quimioterapia: acesso aberto
Acesso livre

Abstrato

Langerhans Cell Sarcoma: Treatment with Dose-adjusted EPOCH

Abhisek Swaika, Neha Sood, David M Menke, Asit K Jha and Sikander Ailawadhi

Background: Langerhans cell sarcoma (LCS) is a rare malignancy with no specific defined treatment strategy.Chemotherapy is the preferred option in cases with systemic involvement, though no consensus exists on thepreferred regimen. Case: A sixty-six year old male presented with advanced-stage LCS involving the bone marrow (BM) and spleen. Immunohistochemical analysis of the BM-sample confirmed the diagnosis of LCS. The patient was treated withchemotherapy utilizing dose-adjusted Etoposide, Prednisone, Vincristine, Cyclophosphamide and Doxorubicin (DAEPOCH). The patient achieved complete remission and was able to tolerate the entire course (6 cycles) of chemotherapy with continued response at 10 months. Conclusion: Due to the rarity of the disease no standard treatment modality exists; most successful treatment options described are anecdotal reports. Due to the paucity of such data and inability to include enough patients on clinical trials, various combination chemotherapy options have been used for best clinical response. The unattractive response rate to CHOP (Cyclophosphamide, Doxorubicin, Vincristine and Prednisone) in previously described case series and the benefit of Etoposide in treating Langerhans cell histiocytosis and primitive sarcoma regimens along with an acceptable adverse effect profile, had us chose DA-EPOCH for our patient. We demonstrate that DAEPOCH is a tolerable and an efficacious treatment regimen for LCS.