Impact of surgery, radiation and systemic therapy on the outcomes of patients with dendritic cell and histiocytic sarcomas

Abstract

Background Neoplasms of histiocytic and dendritic cell origin, including follicular dendritic cell sarcoma (FDCS), histiocytic sarcoma (HS) and interdigitating dendritic cell sarcoma (IDCS), are extremely rare, and data on their natural history and treatment outcomes are sparse. We evaluated the impact of surgery, radiation and systemic therapies on overall survival (OS). Methods We conducted a retrospective chart review of patients with FDCS, IDCS and HS treated at Memorial Sloan Kettering Cancer Center between 1995 and 2014. Results We identified 31, 15 and 7 patients with FDCS, HS and IDCS, respectively. Median age was 48.7, 42.3 and 58.8 years for FDCS, HS and IDCS, respectively. Only a slight disparity in gender distribution existed for FDCS and HS; however, IDCS predominantly affected males (6:1). The most common sites of presentation were abdomen and pelvis (42%), extremities (33%) and head and neck (57%) for FDCS, HS and IDCS, respectively. At diagnosis, 74%, 40% and 86% of patients presented with localised disease in FDCS, HS and IDCS, respectively. Patients with localised disease had significantly improved OS than those with metastatic disease in FDCS (P = 0.04) and IDCS (P = 0.014) but not in HS (P = 0.95). In FDCS and HS, adjuvant or neo-adjuvant therapy was not associated with improved OS compared with observation. In IDCS, surgery alone provided a 5-year overall survival rate of 71%. Conclusions Adjuvant or neo-adjuvant treatment in FDCS and HS did not affect OS. Patients with IDCS had an excellent outcome with surgery. In the metastatic setting, chemotherapy and small molecule inhibitors may provide benefit.

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

“…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 . Induction is usually followed by consolidation with combinations of cyclophosphamide, cytarabine, vincristine, PEG‐asparaginase, and 6‐mercaptopurine, or cyclophosphamide and actinomycin‐D, or idarubicin and cladribine .…”

Response to MEK inhibition with trametinib and tyrosine kinase inhibition with imatinib in multifocal histiocytic sarcoma

“…Despite absence of prospective clinical data, non-Hodgkin lymphoma treatment approaches are often used for multifocal disease, but results are rather disappointing. 4 Few data exist for autologous and even less for allogeneic stem cell transplantation in the adult population, but some patients have shown favorable outcomes. 2,5,6 Thalidomide and alemtuzumab have been reported as potential therapies in single case reports, both in the pediatric or transplant setting.…”

Trametinib in Histiocytic Sarcoma with an Activating MAP2K1 (MEK1) Mutation

“…Since disease-specific therapies are lacking, systemic treatments include regimens for non-Hodgkin’s lymphoma or sarcoma. 3,4 Patients with histiocytic sarcoma and BRAF V600E mutations have been reported to have a response to vemurafenib. We describe a patient who had histiocytic sarcoma with an activating mutation in MAP2K1 who had a complete clinical response and a response on imaging to trametinib, a MAPK kinase (MEK) 1 and 2 inhibitor…”