Malignant histiocytosis (MH) is characterized by an invasive, progressive proliferation of neoplastic histiocytes associated with jaundice, lymphadenopathy, refractory anemia, leukopenia, and often hepatic and splenic enlargement. As lymphoblastic leukemia and lymphoma are regarded as neoplasms of lymphoid cells, MH is thought to represent a malignant transformation of the macrophage and dendritic cells. A classification of malignant histiocytic disorders was oriented by cell lineage in the Histiocyte Society's 1987 version. So dendritic cell-related histiocytic sarcoma (localized or disseminated) and macrophage-related histiocytic sarcoma (localized or disseminated) are the recommended nosology. To establish the correct diagnosis, the major challenge seems to distinguish lymphoid from histiocytic cells. M-CSF receptor, lysozyme, Ki-M8, and S-100 protein, etc are useful markers for histiocytes and T-cell and B-cell lineage markers, such as CD3, CD20, and CD79, etc, for lymphocytes. We have experienced a patient with disseminated histiocytic sarcoma diagnosed by positive istiocytic markers, such as lysozyme, S-100 protein, and CD68.
Anemia, Refractory ; B-Lymphocytes ; Cell Lineage ; Classification ; Dendritic Cells ; Diagnosis ; Histiocytes ; Histiocytic Disorders, Malignant ; Histiocytic Sarcoma* ; Humans ; Jaundice ; Leukopenia ; Lymphatic Diseases ; Lymphocytes ; Lymphoma ; Macrophage Colony-Stimulating Factor ; Macrophages ; Muramidase ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; S100 Proteins ; T-Lymphocytes

Aged ; Antineoplastic Agents ; therapeutic use ; B7-2 Antigen ; analysis ; Diagnosis, Differential ; Follow-Up Studies ; Histiocytic Disorders, Malignant ; drug therapy ; metabolism ; pathology ; Humans ; Male ; Sarcoma ; chemistry ; drug therapy ; pathology ; Skin Neoplasms ; chemistry ; drug therapy ; pathology

PURPOSE: 92 kDa matrix metalloproteinase-9 (MMP-9) is believed to play an important role in degrading the matrix and basement membrane, contributing to the invasion and metastasis of malignant solid tumors. However, little is known about its involvement in a malignant fibrous histiocytoma. The aim of this study was to investigate the expression of MMP-9 and to correlate its expression with clinicopathologic parameters in human malignant fibrous histiocytomas. MATERIALS AND METHODS: Archival tumor tissues from 20 patients with a malignant fibrous histiocytoma were analyzed by immunohistochemistry for the expression of MMP-9. Clinical information was obtained through the computerized retrospective database from the tumorregistry. RESULTS: Seventeen of 20 (85%) tumors showed a positive reaction for MMP-9. MMP-9 activity was inversely correlated with patients' survival time (p=.011). There was no significant correlation between the activated MMP-9 expression and all the other clinicopathologic variables. CONCLUSION: Our data demonstrate that MMP-9 activation is likely to occur in human malignant fibrous histiocytomas. It is also noteworthy that the expression of MMP-9 may aid in predicting patients' poor prognosis.
Basement Membrane ; Histiocytic Disorders, Malignant ; Histiocytoma, Malignant Fibrous* ; Humans* ; Immunohistochemistry ; Matrix Metalloproteinase 9* ; Neoplasm Metastasis ; Prognosis* ; Retrospective Studies

BACKGROUND: The bone marrow biopsy sections of acute leukemia patients occasionally reveal a proliferation of large mononuclear cells that accompany the leukemic blasts, and this proliferation shows a starry sky pattern. We characterized these large mononuclear cells by performing immunohistochemistry with 12 different antibodies. The clinical characteristics were examined and then we determined their difference from hemophagocytic lymphohistiocytosis (HLH) and malignant histiocytic disorders. METHODS: Of the 200 acute leukemic bone marrow biopsy samples, 11 ALL and 10 AML cases showed large mononuclear cell proliferations. The panel of antibodies used for immunohistochemistry included those against the mononuclear phagocyte system, and immunohistochemistry was performed on the patients' initial specimens and the complete remission specimens. 10 normal specimens, 4 initial CML specimens and their complete hematologic response specimens were included as controls. RESULTS: The large mononuclear cells showed immunohistochemical results consistent with histiocytes. They were negative for the markers of dendritic cells the histiocytes and cytokines that are involved in the pathogenesis of HLH and vascular proliferation. Histiocyte proliferation was not observed in the complete remission specimens and in the initial and complete hematological response specimens of the CML patients and the normal bone marrow specimens. None of the cases fulfilled the criteria of HLH, and all 5 ALL cases, for which the immunophenotype results were available, showed a B cell phenotype. CONCLUSION: We characterized the large mononuclear cell proliferations as reactive histiocyte proliferations and we differentiated these from those of secondary HLH and malignant histiocytic disorders. A proportion of the large mononuclear cells showed negative results for all 12 antibodies and they showed characteristics that were suggestive of small fat cells. The pathophysiology and the prognostic effect of the reactive histiocyte proliferation accompanying acute leukemia require further study.
Adipocytes ; Antibodies ; Biopsy ; Bone Marrow ; Cytokines ; Dendritic Cells ; Histiocytes ; Histiocytic Disorders, Malignant ; Humans ; Immunohistochemistry ; Leukemia ; Lymphohistiocytosis, Hemophagocytic ; Mononuclear Phagocyte System ; Phenotype

OBJECTIVETo study the clinicopathologic characteristics and diagnostic criteria of interdigitating dendritic cell sarcoma/tumor (IDCS/T).

METHODSThe clinical features, histologic findings and results of immunohistochemical study in six cases of IDCS/T were analyzed, with review of literature.

RESULTSThe age of patients ranged from 20 to 68 years. The sites of involvement included lymph node, tonsil and soft tissue. Histologically, the tumor cells were arranged in sheets, fascicles or whorls and intimately admixed with abundant lymphocytes and plasma cells. They were oval to spindly in shape and contained pale eosinophilic cytoplasm, oval nuclei and distinct nucleoli.Immunohistochemical study showed that the tumor cells were positive for S-100 protein and CD68.

CONCLUSIONSIDCS/T is a rare malignant tumor with poor prognosis. It carries distinctive histologic pattern and immunophenotype. The entity needs to be distinguished from follicular dendritic cell sarcoma/tumor, anaplastic large cell lymphoma and other spindle cell sarcomas in occurring soft tissue.

Adult ; Aged ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Dendritic Cell Sarcoma, Follicular ; metabolism ; pathology ; Dendritic Cell Sarcoma, Interdigitating ; metabolism ; pathology ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Histiocytoma, Malignant Fibrous ; metabolism ; pathology ; Histiocytosis, Langerhans-Cell ; metabolism ; pathology ; Humans ; Lymph Nodes ; pathology ; Lymphoma, Large-Cell, Anaplastic ; metabolism ; pathology ; Male ; Middle Aged ; Neck ; S100 Proteins ; metabolism ; Sarcoma ; pathology ; Soft Tissue Neoplasms ; metabolism ; pathology ; Thigh ; Tonsillar Neoplasms ; metabolism ; pathology ; Vimentin ; metabolism ; Young Adult

Dendritic Cell Sarcoma, Follicular ; pathology ; Dendritic Cell Sarcoma, Interdigitating ; pathology ; Female ; Humans ; Middle Aged ; Stomach Neoplasms ; pathology

OBJECTIVETo study the pathologic features, diagnosis and differential diagnosis of interdigitating dendritic cell sarcoma (IDCS).

METHODSThe clinical findings, morphologic features and immunophenotype of 3 cases of IDCS were investigated.

RESULTSGross examination showed that IDCS had a greyish-white to greyish-yellow cut surface. The site of occurrence included lung, spleen (with lymph node metastasis) and lymph node. Histologically, the tumor cells were arranged in nests, fascicles and whorls, with intimate admixture of many lymphocytes and plasma cells. They were oval to spindle in shape and contained pale eosinophilic cytoplasm, oval and sometimes grooved nuclei, small distinct nucleoli and ill-defined cell borders. Immunohistochemical study showed that the tumor cells expressed S-100 protein.

CONCLUSIONSIDCS is a rare type of histiocytic and dendritic cell malignancy with distinctive morphologic findings. It needs to be distinguished from follicular dendritic cell sarcoma, inflammatory pseudotumor, Langerhans' cell histiocytosis, malignant melanoma, undifferentiated carcinoma and anaplastic large cell lymphoma. Immunohistochemical staining for S-100 protein is helpful in confirming the diagnosis.

Adolescent ; Carcinoma ; pathology ; Dendritic Cell Sarcoma, Follicular ; pathology ; Dendritic Cell Sarcoma, Interdigitating ; diagnosis ; pathology ; Dendritic Cells ; pathology ; Diagnosis, Differential ; Female ; Humans ; Lymph Nodes ; pathology ; Lymphatic Metastasis ; pathology ; Male ; Middle Aged ; S100 Proteins ; analysis ; immunology ; Young Adult

Langerhans cell histiocytosis (LCH) is a pleomorphic disease entity characterized by local or disseminated atypical Langerhans cells (LCs) found most commonly in bone, lungs, mucocutaneous structures and endocrine organs. Among different sites, unifocal disease confined to a lymph node (LN) is rare. We report a case of LCH confined to a LN in a 38-year-old male who presented with a 2 cm-sized cervical mass. The fine needle aspiration (FNA) smears of cervical LN showed high cellularity having isolated LCs with contorted nuclei and nuclear grooves mixed in multinucleated giant cells, small lymphocytes and eosinophils. Charcot-Leyden crystals were also seen, as were a few dendritic-like cells and intranuclear inclusions. Confirmation of LCH was made by histopathologic studies, positive reactions for S-100 protein and CD1a immunohistochemical staining and by the demonstration of Birbeck granules on electron microscopy. The differentials to be considered include dermatopathic lymphadenitis, sinus histiocytosis with massive lymphadenopathy, Hodgkin's lymphoma and malignant histiocytosis. The characteristic cytomorphologic pattern of LCH in a LN FNA smear plays an important role in suggesting the diagnosis of LCH.
Adult ; Biopsy, Fine-Needle* ; Diagnosis ; Eosinophils ; Giant Cells ; Histiocytic Sarcoma ; Histiocytosis, Langerhans-Cell* ; Histiocytosis, Sinus ; Hodgkin Disease ; Humans ; Intranuclear Inclusion Bodies ; Langerhans Cells ; Lung ; Lymph Nodes* ; Lymphadenitis ; Lymphocytes ; Male ; Microscopy, Electron ; S100 Proteins

Abdominal Neoplasms ; drug therapy ; metabolism ; pathology ; secondary ; surgery ; Dendritic Cell Sarcoma, Follicular ; drug therapy ; metabolism ; pathology ; surgery ; Dendritic Cell Sarcoma, Interdigitating ; metabolism ; pathology ; Diagnosis, Differential ; Female ; Gastrointestinal Stromal Tumors ; metabolism ; pathology ; Histiocytoma, Malignant Fibrous ; metabolism ; pathology ; Humans ; Middle Aged ; Neoplasm Recurrence, Local ; Omentum ; Peritoneal Neoplasms ; secondary ; Receptor, Epidermal Growth Factor ; metabolism ; Receptors, Complement 3b ; metabolism ; Receptors, Complement 3d ; metabolism

OBJECTIVETo study the clinicopathologic features of follicular dendritic cell sarcoma (FDCS) and its differential diagnosis.

METHODSTen cases of FDCS were studied by light microscopy, immunohistochemistry and in-situ hybridization. The clinical features and follow-up information were analyzed.

RESULTSAmongst the 10 cases of FDCS studied, the male-to-female ratio was 1:1. The mean age of the patients was 42 years. Six of them were located in cervical and peritoneal lymph nodes and four in extranodal sites (including tonsil, pelvic cavity, tail of pancreas and spleen). Histologically, the tumor cells had whorled, storiform or diffuse growth patterns. They were spindle in shape and contained syncytial eosinophilic cytoplasm, with round or oval nuclei, vesicular chromatin, distinct nucleoli and a variable number of mitotic figures. Multinucleated tumor giant cells and intranuclear pseudoinclusions were occasionally seen. There was a sprinkling of small lymphocytes and neutrophils within the tumor as well as in the perivascular region. Immunohistochemical study showed that the tumor cells were diffusely or focally positive for CD21, CD23, CD35 and D2-40, but negative for LCA, CD20, CD3, CD1a, HMB45 and CK. Some of them showed EMA, CD68 and S-100 reactivity. In-situ hybridization for Epstein-Barr virus-encoded RNA (EBER) showed positive signals in only one case (which was diagnosed as inflammatory pseudotumor-like FDCS). Of the 7 patients with follow-up information available (duration: 2 months to 39 months; mean: 14 months), 2 cases with paraneoplastic pemphigus died of pulmonary infection at 5 and 7 months respectively. The remaining 5 patients were alive and disease-free after surgical excision (+/- chemotherapy and radiotherapy).

CONCLUSIONSFDCS is a rare low to intermediate-grade malignant tumor. Appropriate application of FDC markers, such as CD21, CD35 and D2-40, would be helpful for arriving at a correct diagnosis. Most cases are associated with good prognosis after surgical treatment, with or without chemotherapy and radiotherapy. Patients with paraneoplastic pemphigus carry a less favorable prognosis.

Adult ; Antibodies, Monoclonal, Murine-Derived ; metabolism ; Dendritic Cell Sarcoma, Follicular ; complications ; metabolism ; pathology ; surgery ; Dendritic Cell Sarcoma, Interdigitating ; pathology ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Lymph Node Excision ; Lymph Nodes ; pathology ; surgery ; Male ; Meningioma ; pathology ; Middle Aged ; Nasopharyngeal Neoplasms ; pathology ; Paraneoplastic Syndromes ; complications ; Pemphigus ; complications ; Receptors, Complement 3b ; metabolism ; Receptors, Complement 3d ; metabolism ; Receptors, IgE ; metabolism ; Tonsillar Neoplasms ; metabolism ; pathology ; surgery ; Young Adult