ArticleThe American journal of case reports2026
Histiocytic Sarcoma With Striking Clinical Improvement.
Article in The American journal of case reports, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
BACKGROUND Histiocytic sarcoma (HS) is a rare and aggressive hematopoietic malignancy characterized by histiocytic cell proliferation. Its rarity and nonspecific presentation create significant diagnostic and therapeutic challenges, particularly in HIV-positive patients. CASE REPORT An HIV-positive man in his late 20s from a rural area and low-income background, referred from a district hospital, presented with asymptomatic nodular and crateriform lesions on the face, thorax, and back, characterized by central keratosis, crust, and erythematous-violaceous infiltrated edges. Histopathological and immunohistochemical analysis confirmed the diagnosis of histiocytic sarcoma, with tumor cells positive for CD68. Considering the patient's multisystem involvement, 6 to 8 cycles of CHOP chemotherapy were recommended. However, the patient declined systemic therapy and opted for a watch-and-wait approach. No invasive procedures such as bone marrow biopsy were performed, as the patient exhibited no constitutional symptoms or cytopenias. Clinical follow-up over 6 months revealed significant improvement in tumor size and overall condition. This favorable outcome may have been partially influenced by immune reconstitution associated with ongoing antiretroviral therapy (ART), although a direct causal relationship cannot be established. Management of histiocytic sarcoma remains controversial due to the lack of established guidelines and variability in natural history. For this patient, a non-aggressive, observational approach yielded favorable outcomes, challenging the conventional use of systemic chemotherapy in all cases of multisystem involvement. Regular clinical follow-up has been integral to monitoring disease progression. CONCLUSIONS This report underscores the diagnostic and therapeutic challenges associated with histiocytic sarcoma and highlights the importance of a multidisciplinary team approach involving infectious disease specialists and hematology-oncology experts in achieving optimal clinical outcomes. Despite declining systemic treatment, the patient had a favorable clinical outcome.
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