Histiocytic Sarcoma Arising From a Long Bone: Report of Two Cases

Abstract

Histiocytic sarcoma is a rare, but aggressive malignant neoplasm of monocyte/macrophage lineage with a wide age distribution. Bone involvement is exceedingly rarer compared to the lymph node, skin, and soft tissue, and no long bone involvement has been reported in the English literature. We here report 2 cases of histiocytic sarcoma involving the long bone: one from the femur of a 77-year-old female, status post the placement of an intramedullary nail for subtrochanteric hip fracture; the other from the radius of a 3-year-old female with no significant medical history. Radiologic imaging showed highly destructive lesions in both cases with soft-tissue extension. Microscopy in both cases showed sheets of polygonal mononuclear cells with abundant eosinophilic cytoplasm, prominent nucleoli, and frequent mitosis. Hemophagocytosis were also identified. Immunohistochemistry showed that the lesional cells were strongly diffusely positive for CD68 and CD163. The first patient deteriorated rapidly, despite the aggressive treatment of amputation and chemotherapy. However, the second patient is disease free 36 months post the treatment of amputation only. We conclude that the long bone could be the primary site of histiocytic sarcoma. Its prognosis could be very variable and it is difficult to predict its behavior based on morphological evaluation only.

A Systemic Review of Primary Malignant Long Bone Tumors in Children and Adolescents

“…However, in contrast to OS it might respond to certain degree. X-ray shows signs of bone destruction with lesion that can be lytic, mixed, or sclerotic (2,18,28). Again, this paper stresses the need for a high index of suspicion and confirmation of diagnosis with a biopsy before proceeding definitive excision.…”

Histiocytic Sarcoma with an Unusual Clinical Manifestation Imitating Malignant Vascular Tumor: A Case Report

“…Hornick et al investigated 14 cases of extranodal HS, in which the patients with poor outcome had the largest primary tumors [ 16 ]. The age of patients can be independent prognostic factor, with younger patients expected to have better disease outcomes [ 12 , 17 ]. In general, surgery, chemotherapy, and radiotherapy are therapeutic modalities of choice.…”

“…Chemotherapy regimens are more controversial and limited [ 18 ]. HS with systemic involvement has very poor prognosis despite aggressive chemotherapy [ 4 , 17 , 19 ]. Thalidomide, along with autologous or allogeneic stem cell transplant, has been recently shown to be of benefit for several patients with HS [ 20 , 21 ].…”