A Rare Case of Aggressive Histiocytic Sarcoma in a 9- years old male Child: Case report and Literature review

Abstract

Abstract Histiocytic Sarcoma(HS) is an extremely rare non-Langerhans neoplasm primarily composed of malignant cells with morphologically and immunohistochemically showing features of mature tissue histiocytes. It can affect any part of the body with unknown etiology and is an aggressive disease with a worse prognosis and management often requires multimodal treatment. Here, we report a 9 year old boy who was diagnosed with histiocytic sarcoma after he presented with left-side chest pain, dry cough, and significant weight loss of four months duration. Contrast-enhanced chest CT scan shows a heterogeneous contrast-enhancing solid extra parenchymal mass lesion that appears to be attached to the posteromedial basal pleura with adjacent lung subsegmental collapse and a minimal amount of ipsilateral pleural effusion. There was no evidence of systemic arterial supply. The patient underwent left posterolateral thoracotomy and mass excision. The biopsy and immunohistochemical examination confirmed the diagnosis of Histiocytic Sarcoma. The mass recurred after a month, and he started systemic chemotherapy with a CHOP regimen. Despite the multimodal treatment, he had a progressive disease with multiorgan involvement and was put on palliative care.