Abstract

Pulmonary histiocytic sarcoma is a rare, but highly malignant disease. Its low incidence imposes significant difficulties on physicians confronted with affected patients. The reported patient is a 24-year old male with histiocytic sarcoma of the lung. Left lower lobectomy was performed. Histologically, tumor cells had no features of carcinoma. Several entities were raised as differentials: large B or T cell lymphomas, metastatic melanoma, sarcoma, undifferentiated carcinoma, NK cell lymphoma. Immunohistochemically, melanoma, carcinoma, undifferentiated epithelioid sarcoma and also different types of lymphoma were excluded. Therefore, tumors of the histiocytic and dendritic cell lineage had to be considered. Tumors of the dendritic cell lineage were also immunohistochemically excluded, leaving histiocytic sarcoma by exclusion. The patient was followed for six years and six months, with no signs of recurrence of the tumor. Histiocytic sarcomas are tumors of uncertain behavior, with some progressing quickly, and others having a much slower course. As these tumors are rare, there is not much information, although a low number of mitosis might point to a less aggressive course. In the present patient with an unifocal disease, surgical excision was sufficient, without adjuvant radiotherapy and chemotherapy.

Highlights

  • Histiocytic sarcoma (HS) is a rare hematological malignant neoplasm composed of cells showing morphologic and immunophenotypic evidence of histiocytic differentiation [1]

  • Reported cases of HS located in the central nervous system (CNS) and bone marrow [3,4,5] are regarded as aggressive hematopoietic neoplasm with a strong potential for systemic spread and a poor response to therapy [6]

  • Open lung biopsy showed nodular densities measuring up to 3 cm in diameter. These nodules showed an infiltration by tumor cells, which did not form epithelial structures, had large polymorphic nuclei with vesicular chromatin, accentuated nuclear membrane staining, and medium-sized nucleoli, usually centrally located within the nucleus (Figure 3)

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Summary

Introduction

Histiocytic sarcoma (HS) is a rare hematological malignant neoplasm composed of cells showing morphologic and immunophenotypic evidence of histiocytic differentiation [1]. Reported cases of HS located in the central nervous system (CNS) and bone marrow [3,4,5] are regarded as aggressive hematopoietic neoplasm with a strong potential for systemic spread and a poor response to therapy [6]. Erythrocyte sedimentation rate (ESR) was 110 mm/h (reference interval: 2-13 mm/h), a complete blood count showed a leukocyte (WBC) count of 8.9 x109/L (reference interval: 4.0-11.0 x109/L), with normal percentage of particular classes of leukocytes. Serum biochemistry revealed an elevated alkaline phosphatase (ALP) level of 248 U/L (reference interval: 60-142 U/L), and gamma glutamyl transferase (GGT) level of 95 U/L (reference interval: 11-55 U/L). Tumor markers [CA (cancer antigen) 19-9, CA (cancer antigen) 125, CEA (carcinoembryonic antigen), PSA (prostate specific antigen), AFP (alpha-1-phetoprotein), NSE (neuron-specific enolase), CYFRA (cytokeratin 19 fragments) 21-1, B2M (beta-2 microglobulin)] in the serum were normal

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