Abstract
We report a patient with primary histiocytic sarcoma of the spleen associated with prominent hemophagocytosis. Although thrombocytopenia, probably due to hemophagocytosis, was refractory to corticosteroid therapy, the transfusion of platelets, and splenic irradiation, partial splenic embolization was effective and facilitated splenectomy for a diagnosis. The majority of the spleen showed necrosis, but viable neoplastic cells with pleomorphic nuclei and abundant cytoplasm, showing occasional erythrocytes or leukocytes, were still discernible. The neoplastic cells expressed CD68, lysozyme, and S-100 protein, and were negative for lymphoid, myeloid, and epithelial cell markers. CD163, a monocyte/macrophage-specific molecule, was positive in only some of them. Despite multiagent chemotherapy, the patient died of the disease, showing a rapidly progressive clinical course. Although the preoperative diagnosis of primary splenic histiocytic sarcoma is difficult, it has been confirmed in patients with splenomegaly of unknown etiology that clinicolaboratory features suggestive of hemophagocytosis may be important clues suggestive to the disease. CD163 expression by neoplastic cells could be confirmed only after careful observation, because the molecule may only be seen in some of the neoplastic cells.
References
Weiss LM, Grogan TM, Mueller-Hermelink H-K, et al. Histiocytic sarcoma. In: Jeffe ES, Harris NL, Stein H, Vardiman JW, editors. World Health Organaization classification of tumors: pathology and genetics of tumors of haematopoietic and lymphoid tissues. Lyon, France: International Agency for Research on Cancer Press; 2001. pp. 278–9.
Pileri SA, Grogan TM, Harris NL, et al. Tumours of hisiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases. Histopathology. 2002;41:1–29.
Vos AJ, Abbondanzono SL, Barekman CL, Andriko JW, Miettien M, Aguilera NS. Histiocytic sarcoma: a study of five cases including the histiocyte marker CD163. Mod Pathol. 2005;18:693–704.
Copie-Bergman C, Wotherspoon AC, Norton AJ, Diss TC, Isaacson PG. True histiocytic lymphoma: a morphologic immnohistochemical and molecular genetic study of 13 cases. Am J Surg Pathol. 1998;22:1386–92.
Hornick JL, Jaffe ES, Fletch CDM. Extranodal histiocytic sarcoma. Clinicopathologic analysis of 14 cases of a rare epithelioid malignancy. Am J Surg Pathol. 2004;28:1133–44.
Lauritzen AF, Delsol G, Hansen NE, et al. Histiocytic sarcoma and monoblastic leukemias: a clinical, histologic, and immunopheotypical study. Am J Clin Pathol. 1994;102:45–54.
Audouin J, Vercelli-Retta J, Le Touneau A, et al. Primary histiocytic sarcoma of the spleen associated with erythrophagocytic histiocytosis. Pathol Res Pract. 2003;199:107–12.
Franchino C, Reich C, Distenfeld A, et al. A clinicopathologically distinctive primary splenic histiocytic neoplasm: demonstration of its histiocytic derivation by immunophenotypic and molecular genetic analysis. Am J Surg Pathol. 1988;12:398–404.
Kimura H, Nasu K, Sakai C, et al. Histiocytic sarcoma of the spleen associated with hypoalbuminemia hypo γ-globulinemia and thrombocytopenia as a possibly unique clinical entity: report of three cases. Leuk Lymphoma. 1998;31:217–24.
Caulier M, Darloy F, Rose C. Splenic irradiation for chronic autoimmune thrombocytopenic purpura in patients with contra-indications to splenectomy. Br J Haematol. 1995:91:208–11.
Mozes MF, Spigos DG, Pollak R. Partial splenic embolization, an alternative to splenectomy: results of a prospective randomized study. Surgery. 1984;96:694–702.
Lau SK, Chu PG, Weiss LM. CD163: a specific marker of macrophages in paraffin-embedded tissue samples. Am J Clin Pathol. 2004;122:794–801.
Author information
Authors and Affiliations
Corresponding author
About this article
Cite this article
Oka, K., Nakamine, H., Maeda, K. et al. Primary histiocytic sarcoma of the spleen associated with hemophagocytosis. Int J Hematol 87, 405–409 (2008). https://doi.org/10.1007/s12185-008-0059-6
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12185-008-0059-6