Aged ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Carcinoma, Signet Ring Cell ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Gastrectomy ; methods ; Histiocytic Sarcoma ; metabolism ; pathology ; surgery ; Humans ; Lymphatic Metastasis ; Male ; Phosphoglucomutase ; metabolism ; Stomach Neoplasms ; metabolism ; pathology ; surgery

Aged ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Carcinoma, Large Cell ; metabolism ; pathology ; Diagnosis, Differential ; Histiocytic Sarcoma ; metabolism ; pathology ; surgery ; Hodgkin Disease ; metabolism ; pathology ; Humans ; Lymphoma, Large B-Cell, Diffuse ; metabolism ; pathology ; Lymphoma, Large-Cell, Anaplastic ; metabolism ; pathology ; Male ; Melanoma ; metabolism ; pathology ; Receptors, Cell Surface ; metabolism ; Stomach Neoplasms ; metabolism ; pathology ; surgery

OBJECTIVETo study the clinicopathologic features and differential diagnosis of extranodal Rosai-Dorfman disease (RDD) of the upper respiratory tract.

METHODSThe clinical, pathologic and immunohistochemical features of 10 cases of RDD were evaluated.

RESULTSAmong the 10 cases studied, there were 3 males and 7 females. The age of patients ranged from 20 to 61 years old (mean 38 years). The lesion arose in the nasal cavity (7 cases), nasopharynx (2 cases) or hard palate to trachea (1 case). Most of the patients presented with nasal obstruction, rhinorrhagia or tumor mass in the nasal/nasopharyngeal regions. CT scan often showed the presence of soft tissue lesion without bone destruction. Histologically, extranodal RDD was characterized by light-staining bands alternating with dark-staining bands. The light-staining bands were formed by aggregates of large round or polygonal histiocytes with emperipoiesis. The dark-staining bands were formed by abundant lymphoplasmacytic infiltrates. Immunohistochemical study showed that the histiocytes strongly expressed S-100 protein and partially expressed CD68. Six patients had no recurrence after surgical resection.

CONCLUSIONSExtranodal RDD of the upper respiratory tract is a rare disorder of histiocytic proliferation, which usually involves the nasal cavity and paranasal sinuses. RDD can easily mimic rhinoscleroma, mainly due to the overlapping morphologic appearance. Immunohistochemical study is helpful in the differential diagnosis.

Adult ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Histiocytic Sarcoma ; metabolism ; pathology ; Histiocytosis, Langerhans-Cell ; metabolism ; pathology ; Histiocytosis, Sinus ; diagnostic imaging ; metabolism ; pathology ; surgery ; Humans ; Male ; Middle Aged ; Nasal Cavity ; pathology ; Nasopharyngeal Diseases ; diagnostic imaging ; metabolism ; pathology ; surgery ; Neoplasms, Muscle Tissue ; metabolism ; pathology ; Nose Diseases ; diagnostic imaging ; metabolism ; pathology ; surgery ; S100 Proteins ; metabolism ; Tomography, X-Ray Computed ; Young Adult

OBJECTIVETo investigate the clinicopathologic features, immunophenotyping, differential diagnoses and prognosis of histiocytic sarcoma (HS).

METHODSThe clinical and pathologic findings of 4 cases of HS were reviewed. The samples were used for paraffin section, HE stain, immunohistochemistry stain by EnVision method, electron microscope observation. Follow-up information was available in all patients.

RESULTSThe age of patients, 2 males and 2 females, ranged from 22 to 65 years old (median, 43.25 years). The sites of involvement included lymph node (2 cases), skin or soft tissue (1 case) and colon (1 case). The tumor cells were widespread infiltration, diffused distribution, no adhesion to each other. Tumor cells were middling and large, round, orbicular-ovate, polygon, epithelium appearance, plentiful cytoplasm and acidophilia, cystose. Nucelus was round, orbicular-ovate, dissymmetry. Nuclear chromatin was vacuole appearance, basophilia nucleolus, caryocinesia and pathological mitotic figure. Three of the cases showed conjugate nuclei, increased pleomorphism with multinucleated tumor giant cell formation. Focal cytoplasmic with foamy appearance was identified in 2 cases. One case demonstrated foci of spindly sarcomatoid appearance. Hemophagocytosis was identified in 2 cases. The tumor cells of 4 cases were often accompanied by various numbers of inflammatory cells. Immunohistochemical study showed that all cases were diffusely positive for α-1-ACT, CD68, CDl63 and lysozyme. Three of 4 cases also expressed CD45, CD45RO. The electron microscope results of 4 cases showed that the tumor cells were plentiful cytoplasm and a few cytolysosome in the cytoplasm, and no birbeck cytorrhyctes, cell-cell junction and digitation. Amongst the 4 patients with follow-up information available, three died of the disease 6-13 months after diagnosis. One patient, whose lesion was localized at the skin and soft tissue, survived at the present time.

CONCLUSIONHS was a scarce malignant tumor with mature histiocyte morphology and immunophenotype character. The diagnosis should be based on tissue morphology, immunohistochemistry and electron microscope observation to exclude other disorders.

Adult ; Aged ; Diagnosis, Differential ; Female ; Histiocytes ; pathology ; Histiocytic Sarcoma ; diagnosis ; pathology ; Humans ; Immunohistochemistry ; Immunophenotyping ; Male ; Microscopy, Electron ; Middle Aged ; Young Adult

Histiocytic sarcoma is a rare malignant neoplasm that originates from a histiocytic hematopoietic lineage characterized by histiocytic differentiation and its corresponding immunophenotypic features. Patients with histiocytic sarcoma usually have a poor prognosis due to its aggressive clinical behavior. Here we report a rare case of extranodal histiocytic sarcoma of the stomach which was confirmed through immunohistochemical staining. A 71-yearold man was presented with epigastric pain. Gastroscopy, abdominal CT, and EUS revealed a mass located on the posterior wall of upper body and fundus of the stomach. Grossly, grayish white solid masses were seen extending down to the submucosal layer. Microscopically, the tumor cells had eosinophilic cytoplasm, abundant vacuole, and mitosis. Immunohistochemical staining revealed that the tumor cells were positive for LCA, CD68, and lysozyme. Early detection and accurate diagnosis of this rare neoplasm is important because it can make a great difference in prognostic outcomes. To make an accurate and definitive diagnosis, immunohistochemical staining is essential in the confimation of histiocytic orign.
Adenocarcinoma/diagnosis/pathology/ultrasonography ; Aged ; Antigens, CD/metabolism ; Antigens, CD45/metabolism ; Antigens, Differentiation, Myelomonocytic/metabolism ; Diagnosis, Differential ; Gastroscopy ; Histiocytic Sarcoma/*diagnosis/pathology/ultrasonography ; Humans ; Male ; Muramidase/metabolism ; Stomach Neoplasms/*diagnosis/pathology/ultrasonography ; Tomography, X-Ray Computed

OBJECTIVETo study the morphologic features, immunophenotypes, differential diagnoses and prognosis of histiocytic sarcoma (HS).

METHODSThe clinical and pathologic findings of 6 cases of HS were reviewed. Immunohistochemical assay (Elivision staining) was also performed. Follow-up information was available in 4 patients.

RESULTSThere were altogether 3 males and 3 females. The age of patients ranged from 12 to 81 years old (median = 54.6 years). The sites of involvement included lymph node (number = 2 cases) and skin or soft tissue (number = 4 cases). The tumor was composed of sheets of large epithelioid cells with abundant eosinophilic cytoplasm, oval to irregular nuclei, vesicular chromatin and large nucleoli. Binucleated form was not uncommon. Two of the cases showed increased pleomorphism with multinucleated tumor giant cell formation. Focal cytoplasmic with foamy appearance was identified in 3 cases. One case demonstrated foci of spindly sarcomatoid appearance. Hemophagocytosis was identified in 2 cases. Mitotic figures were readily identified. The tumor cells were often accompanied by various numbers of inflammatory cells. Immunohistochemical study showed that all cases were diffusely positive for leukocyte common antigen, CD4, CD68 and CD163. Four of the 5 cases studied also expressed lysozyme. Amongst the 4 patients with follow-up information available, 3 died of the disease at 6 to 11 months interval after diagnosis. One patient, whose lesion was localized at the skin and soft tissue, survived for 3 years, with no evidence of tumor recurrence.

CONCLUSIONSAccurate diagnosis of the HS is based on the combination of morphologic examination and immunohistochemical assay. HS often presents with clinically advanced disease and pursues an aggressive clinical course, with a poor response to therapy. However, a subset of cases presenting with clinically localized lesion may carry a relatively favorable long-term outcome.

Adult ; Aged ; Aged, 80 and over ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Carcinoma, Renal Cell ; metabolism ; pathology ; Child ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Histiocytic Sarcoma ; drug therapy ; metabolism ; pathology ; surgery ; Humans ; Lymphoma, Large B-Cell, Diffuse ; metabolism ; pathology ; Lymphoma, Large-Cell, Anaplastic ; metabolism ; pathology ; Male ; Melanoma ; metabolism ; pathology ; Muramidase ; metabolism ; Prognosis ; Receptors, Cell Surface ; metabolism ; Skin Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Soft Tissue Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Young Adult

Actins ; metabolism ; Adult ; Diagnosis, Differential ; Granzymes ; metabolism ; Histiocytic Sarcoma ; metabolism ; pathology ; Humans ; Ki-1 Antigen ; metabolism ; Lymph Nodes ; metabolism ; pathology ; Lymphoma, Large-Cell, Anaplastic ; metabolism ; pathology ; Male ; Neoplasms, Muscle Tissue ; metabolism ; pathology ; Protein-Tyrosine Kinases ; metabolism ; Receptor Protein-Tyrosine Kinases

Histiocytic sarcoma (HS) is a very rare neoplasm that often shows an aggressive clinical course and systemic symptoms, such as fever, weight loss, adenopathy, hepatosplenomegaly and pancytopenia. It may present as localized or disseminated disease. We describe here a 63-yr-old male who manifested systemic symptoms, including fever, weight loss and generalized weakness. Abdominal and chest computed tomography failed to show specific findings, but there was suspicion of multiple bony changes at the lumbar spine. Fusion whole body positron emission tomography, bone scan and lumbar spine magnetic resonance imaging showed multiple bone lesions, suggesting a malignancy involving the bone marrow (BM). Several BM and bone biopsies were inconclusive for diagnosis. Necropsy showed replacement of the BM by a diffuse proliferation of neoplastic cells with markedly increased cellularity (95%). The neoplastic cells were positive for lysozyme and CD68, but negative for T- and B-cell lineage markers, and megakaryocytic, epithelial, muscular and melanocytic markers. Morphologic findings also distinguished it from other dendritic cell neoplasms.
Antigens, CD/metabolism ; Antigens, Differentiation, Myelomonocytic/metabolism ; Bone Marrow/metabolism/pathology ; Bone Marrow Neoplasms/*diagnosis/pathology ; Diagnosis, Differential ; Histiocytic Sarcoma/*diagnosis/pathology ; Humans ; Magnetic Resonance Imaging ; Male ; Muramidase/metabolism ; Positron-Emission Tomography ; Tomography, X-Ray Computed

Histiocytic sarcoma is a malignant proliferation of cells showing morphologic and immunophenotypic features similar to those of mature tissue histiocytes and is known for its rapid progression and poor prognosis. We describe a case of histiocytic sarcoma diagnosed by bone marrow biopsy. A 64-yr-old male was admitted for fever and weight loss that persisted for 8 months. The patient died undiagnosed on the 7th hospitalization day. A bone marrow biopsy performed just before the patient's death revealed diffuse proliferation of large pleomorphic neoplastic cells with large, round to oval nuclei, vesicular chromatin, and abundant foamy cytoplasm. These cells were positive for histiocytic markers, CD68, lysozyme, CD21, and S-100 protein, but negative for B-cell, T/NK-cell, and epithelial cell markers, thus confirming the presence of histiocytic sarcoma.
Antigens, CD/metabolism ; Antigens, CD31/metabolism ; Antigens, Differentiation, Myelomonocytic/metabolism ; Bone Marrow/*pathology ; Fever/diagnosis ; Histiocytic Sarcoma/*diagnosis/pathology/radiography ; Humans ; Male ; Middle Aged ; Muramidase/metabolism ; S100 Proteins/metabolism ; Tomography, X-Ray Computed

Histiocytic Sarcoma ; diagnosis ; pathology ; therapy ; Humans ; Infant, Newborn ; Male