Malignant Histiocytoses/Disseminated Histiocytic Sarcoma With Hemophagocytic Syndrome in a Patient With Mediastinal Germ Cell Tumor

Abstract

We describe a case of a 24-year-old man with a large anterior mediastinal mass showing a nonseminomatous germ cell tumor then subsequently developed hemophagocytic syndrome involving the bone marrow and liver. During the course of chemotherapy, he developed profound thrombocytopenia, eccymoses, and bleeding. He had moderate splenomegaly and splenectomy was performed to restore adequate hematologic reserve to permit further chemotherapy. The spleen showed marked erythrophagocytosis and markedly atypical histiocytes consistent with malignant histiocytoses. Atypical histiocytes stained positive for CD68, CD163, CD4, CD45 (LCA), and S-100. Cytogenetics studies were negative for i12p. The patient was refractory to therapy and ultimately died 5 months after diagnosis.

Fine needle aspiration of Histiocytic sarcoma

“…Histiocytic sarcoma needs to be distinguished from other neoplasms like interdigitating dendritic cell tumor and DLBCL. [567] In our case, the cells were strongly positive for CD68 and focally positive for S100, CD4 and CD45Ro. IHC for CD3, CD20, CD33, CD30 and vimentin was negative.…”

“…[13] A few cases have been described with prior mediastinal germ cell tumor and malignant teratomas. [35] The architecture of the normal organ is effaced with polymorphic tumor cells with the cells being large with increased nuclear cytoplasmic ratio, large nucleus and irregular nuclear outlines. [136] The tumor cells are positive for CD68, CD163, lysozyme, CD11c and CD14 and are negative for myeloperoxidase, CD33 and CD34.…”

Histiocytic sarcoma with two immunohistopathologically distinct populations

“…Recently, CD163 has been recognized as a new macrophage-related differentiation marker, which is more specific than the conventional histiocyte-related molecules [2,3,5]. HS is a rare neoplasm and, by our search, CD163 staining was evaluated in 28 cases as summarized in Table 1 [3,[5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20]. There are several reports of HS patients arising from other neoplasms, such as acute lymphoblastic leukemia, chronic lymphocytic leukemia, malignant lymphoma, and germ cell tumor [13][14][15][16][20][21][22].…”

Cytologic identification of erythrophagocytic neoplasms in dogs

“…In people, erythrophagocytosis by neoplastic cells has been reported in several tumor types, including leukemias, plasma cell tumors, histiocytic sarcoma, angiosarcoma, and hemangiosarcoma . In some patients, erythrophagocytosis by tumor cells exacerbates anemia, which can be correlated with a grave prognosis .…”