Work

Allogeneic Hematopoietic Stem Cell Transplantation for Management of Histiocytic Sarcoma: A Systemic Review

Year: 2025

Type: article

Abstract: Introduction There is no established standard of care for Histiocytic Sarcoma (HS), with management typically involving surgery and radiotherapy, chemotherapy, or targeted therapy in various combinations. While these regimens can sometimes be successful, a diagnosis of HS generally indicates a poor prognosis, especially in cases of systemic disease. Method Following PRISMA guidelines, a comprehensive literature search was performed on PubMed, Cochrane, and Embase using MeSH terms and keywords relevant to HS. Ninety-nine original studies (clinical trials, retrospective and prospective studies), case reports, and case series, including patients with a confirmed diagnosis of HS above 18 years of age, were included. Data was extracted, including age, gender, ethnicity, clinical presentation and stage, site, diagnostic criteria, immunohistochemistry (IHC) markers, treatment, follow-up, and outcomes. Results A total of 185 patients with HS were included, with nearly equal gender distribution, and median age of 55 years (range 18-82). The most commonly reported chemotherapy regimen was CHOP (cyclophosphamide, hydroxydaunorubicin/doxorubicin, oncovin/vincristine, and prednisone), followed by ICE (ifosfamide, carboplatin, and etoposide). Less frequently reported regimens included HIPEC (hyperthermic intraperitoneal chemotherapy), DHAP (dexamethasone and high-dose cytarabine), and AEVD (doxorubicin, etoposide, vinblastine, and dacarbazine). The median follow-up period was 12 months (range 0.5-144). Immunotherapy with agents such as nivolumab and pembrolizumab was used in cases with PD-L1 mutations, showing potential benefits. A total of 64 patients were treated with surgery (± radiotherapy). Most of these patients had unifocal disease, resulting in a favorable survival rate in approximately half of these cases. Ten patients underwent stem cell transplantation - five allogeneic and five consolidative autologous transplants. Three of the five patients in each transplant group achieved remission. We present a case of a 33-year-old male diagnosed with systemic HS, successfully treated at the University of Kansas Medical Center. Despite initial disease progression following treatment with ICE, CHOP, and CLAG-M (Cladribine, Ara-C, mitoxantrone, and G-CSF) chemotherapies and no targetable mutations, allogeneic haploidentical peripheral blood stem cell transplantation effectively curtailed tumor growth and reduced metastases, leading to a remission lasting more than a year after the transplant. Conclusion Histiocytic sarcoma has heterogeneous clinical behavior necessitating tailored therapeutic approaches and comprehensive management strategies to address its aggressive nature and potential for systemic involvement. Allogeneic hematopoietic stem cell transplantation can be considered in refractory cases.

Source: Transplantation and Cellular Therapy

DOI: 10.1016/j.jtct.2025.01.262