Successful treatment of histiocytic sarcoma with cladribine and high-dose cytosine arabinoside in a child

Abstract

Histiocytic sarcoma, a rare hematopoietic neoplasm with evidence of histiocytic differentiation, is often refractory to conventional chemotherapy and radiotherapy, and its prognosis is generally dismal. The optimal management of this malignancy has not been established. We report a case of 8-year-old girl with histiocytic sarcoma involving the left femur. The tumor rapidly responded to a combination of cladribine and high-dose cytosine arabinoside, an aggressive salvage regimen for refractory Langerhans cell histiocytosis, and became impalpable during the first cycle. The patient has remained in complete remission more than 7 years from diagnosis.

Histiocytic sarcoma in a child—successful management and long‐term survival with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy

“…Diagnosis is made by core, incisional, or excisional biopsy which reveals an entity with large and irregularly pleomorphic bi‐ or tri‐nucleated cells with lobulated nuclei and abundant eosinophilic cytoplasm. By definition, there is expression of cytoplasmic histiocytic markers (CD163 and/or CD68) with lysozyme positivity and absence of B‐ and T‐cell markers. Markers for macrophages (CD31) are focally positive, but are negative for Langerhans cells (CD1a, langerin/CD207), follicular dendritic cells (CD21, CD23, CD35, CAN.42), epithelial cells (pancytokeratin, epithelial membrane antigen [EMA]), melanocytes (melanA, HMB45), and myeloid cells (CD13, CD33, myeloperoxidase) .…”

“…By definition, there is expression of cytoplasmic histiocytic markers (CD163 and/or CD68) with lysozyme positivity and absence of B‐ and T‐cell markers. Markers for macrophages (CD31) are focally positive, but are negative for Langerhans cells (CD1a, langerin/CD207), follicular dendritic cells (CD21, CD23, CD35, CAN.42), epithelial cells (pancytokeratin, epithelial membrane antigen [EMA]), melanocytes (melanA, HMB45), and myeloid cells (CD13, CD33, myeloperoxidase) . CD52 is variably expressed and the target of alemtuzumab .…”

“…There is no standardized therapy for HS. Systemic treatments have included regimes for both NHL and Hodgkin lymphoma including cyclophosphamide, doxorubicin, vincristine, and prednisone; cyclophosphamide, doxorubicin, vincristine, prednisone, and etoposide; carmustine, etoposide, cytarabine, and melphalan; doxorubicin, bleomycin, vinblastine, and dacarbazine; and ifosfamide, carboplatin, and etoposide, and Langerhans cell histiocytosis including prednisone, 6‐mercaptopurine, vinblastine, methotrexate, and etoposide, or cladribine and cytosine‐arabinoside . One group tried vincristine, doxorubicin and cyclophosphamide alternating with ifosfamide and etoposide (VDC‐IE) usually used in Ewing sarcoma and another, single agent temozolamide for cerebral HS with no evidence of recurrence after 23 months .…”

“…Both surgery and radiotherapy have been used successfully as local control measures. Provided the disease is completely resected, outcomes appear to be favorable with recurrence free survivals of up to 2 years at the time of reporting .…”

“…HS most commonly arises in the lymph nodes; however, it may occur anywhere in the body and has been reported in the respiratory tract, gastrointestinal tract, 2 spleen, liver, pancreas, skin, 9 bone and bone marrow, 10 15 or cladribine and cytosine-arabinoside. 12 One group tried vincristine, doxorubicin and cyclophosphamide alternating with ifosfamide and etoposide (VDC-IE) usually used in Ewing sarcoma 14 and another, single agent temozolamide for cerebral HS with no evidence of recurrence after 23 months. 11 Induction is usually followed by consolidation with combinations of cyclophosphamide, cytarabine, vincristine, PEG-asparaginase, and 6-mercaptopurine, 16 or cyclophosphamide and actinomycin-D, or idarubicin and cladribine.…”