Clear-cell chondrosarcoma, a recently specified entity, is a low-grade malignant tumor and has characteristic clinical, roentgenographic and pathologic findings which separate it from conventional chondrosarcoma and other benign tumors. Therefore, correct diagnosis is important from the viewpoint of both prognosis and therapeutic approach. We report a case of typical recurrent clear-cell chordirosarcoma. Typical round cells with clear cytoplasm, large nuclei, and small nucleoli were wellnoted. The clear cytoplasm was faintly positive in PAS staining. Electronmicroscopic study showed that these cells were of chondroid origin, showing indented nuclei, large dilated endoplasmic reticulum cisternae, bundles of actin-like filaments and a few glycogen particles.
Chondrosarcoma/*pathology/ultrastructure ; Female ; Femoral Neoplasms/*pathology/ultrastructure ; Humans ; Microscopy, Electron ; Middle Aged ; Neoplasm Recurrence, Local/*pathology/ultrastructure

OBJECTIVETo study the clinicopathologic features, histologic diagnosis and differential diagnosis of sinonasal-type of hemangiopericytoma (SNTHPC).

METHODSThe clinical, radiographic and pathologic findings of 6 cases of SNTHPC were analyzed. Immunohistochemistry and electron microscopy were performed on selected examples.

RESULTSAmongst the 6 patients studied, 4 were males and 2 were females. The age of patients ranged from 56 to 71 years (mean = 60.5 years old). The commonest clinical presentation was nasal obstruction and/or epistaxis. Other symptoms could include increased nasal secretion, eyeball pain, decreased visual acuity, increased tear secretion and headache. The tumor involved nasal cavity and/or paranasal sinuses. Gross examination showed polypoid tumor masses, brownish fleshy tissue or whitish tumor tissue fragments. Histologically, the tumor showed a mixture of diffuse, fascicular, storiform, reticulated and whorled growth patterns. The tumor cells were spindle-shaped and possessed clear to eosinophilic cytoplasm. Mitotic figures were rarely seen. The intervening vasculature was characteristically thin-walled, with focal hyalinization changes and rarely the staghorn pattern. Immunohistochemical study showed that the tumor cells expressed vimentin (6/6), smooth muscle actin (5/6) and CD34 (3/6). Electron microscopy demonstrated the presence of intracytoplasmic myofilaments. The tumor cells were linked together by primitive cell junctions. In general, the histologic diagnosis of SNTHPC was difficult, and only 1 case had the correct initial pathologic diagnosis made. Follow-up data were available in 5 patients and 2 of them had local recurrences.

CONCLUSIONSSNTHPC is a low to intermediate grade soft tissue tumor with pericytes differentiation. Correct diagnosis relies on detailed pathologic assessment and application of ancillary investigations.

Actins ; metabolism ; Aged ; Antigens, CD34 ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Hemangiopericytoma ; metabolism ; pathology ; ultrastructure ; Humans ; Immunohistochemistry ; Male ; Microscopy, Electron ; Middle Aged ; Nasal Cavity ; pathology ; Neoplasm Recurrence, Local ; Paranasal Sinus Neoplasms ; metabolism ; pathology ; ultrastructure ; Paranasal Sinuses ; pathology ; Vimentin ; metabolism

Antigens, CD34 ; metabolism ; Carcinoma, Renal Cell ; metabolism ; pathology ; Central Nervous System Neoplasms ; metabolism ; pathology ; surgery ; ultrastructure ; Diagnosis, Differential ; Ependymoma ; metabolism ; pathology ; Hemangioblastoma ; metabolism ; pathology ; surgery ; ultrastructure ; Humans ; Meningeal Neoplasms ; metabolism ; pathology ; Meningioma ; metabolism ; pathology ; Mutation ; Neoplasm Recurrence, Local ; Prognosis ; Receptor, Epidermal Growth Factor ; metabolism ; Vimentin ; metabolism ; von Hippel-Lindau Disease ; genetics ; metabolism ; pathology ; surgery

OBJECTIVETo study the clinicopathologic features, immunophenotype and ultrastructural features of sinonasal inflammatory myofibroblastic tumors (IMT).

METHODSThe clinical and histologic features of 5 cases of sinonasal IMT were reviewed. Immunohistochemical study for vimentin, MSA, SMA, calponin, h-caldesmon, desmin, ALK, fibronectin, CK, S-100 and Ki-67 was carried out. Ultrastructural examination was also performed in two of the cases.

RESULTSThe patients age ranged from 28 to 62 years (mean = 43 years). The male-to-female ratio was 2:3. The clinical presentation included nasal obstruction, nasal discharge, nasal bleeding, facial pain, facial swelling, toothache and tear overflow. All of the 5 patients suffered from disease relapses; and 4 of them had recurrences for more than 5 times. One patient had lymph node metastasis and 3 patients died of the disease. Histologically, the tumor cells were arranged in interlacing fascicles and sometimes haphazard in fashion. They were spindly in shape, cytoplasm eosinophilic with mild nuclear atypia and a low mitotic activity. The intervening stroma was myxoid in appearance accompanied by lymphocyte and plasma cell infiltration, abundant blood vessels and focal collagenized areas. In 3 of the recurrent cases, the tumor cells displayed increased nuclear atypia and mitotic activity (average about 5 to 6 per 10 high-power fields), accompanied by patchy necrosis, less inflammatory cell infiltration and focal sarcomatous changes. Immunohistochemical study showed that the tumor cells were diffusely positive for vimentin. SMA, MSA, calponin and fibronectin were variably expressed. Desmin was weakly positive in 1 case. The staining for h-caldesmon, ALK, S-100 and CK was negative. The Ki-67 proliferation index increased with tumor recurrences. Electron microscopy revealed abundant rough endoplasmic reticulum and dense body formation in the cytoplasm. There were an increased amount of collagen fibers in the stroma.

CONCLUSIONSIMT rarely occurs in nasal cavity and paranasal sinuses. The tumor is prone to local invasion and recurrences, with subsequent progression to frank malignancy and distant metastasis, resulting in high mortality and poor prognosis. Complete surgical resection remains the main modality of treatment.

Actins ; metabolism ; Adult ; Calcium-Binding Proteins ; metabolism ; Diagnosis, Differential ; Female ; Fibrosarcoma ; pathology ; Humans ; Ki-67 Antigen ; metabolism ; Lymphatic Metastasis ; Male ; Microfilament Proteins ; metabolism ; Middle Aged ; Neoplasm Recurrence, Local ; Neoplasms, Muscle Tissue ; metabolism ; pathology ; surgery ; ultrastructure ; Neurofibromatoses ; pathology ; Paranasal Sinus Neoplasms ; metabolism ; pathology ; surgery ; ultrastructure ; Vimentin ; metabolism

We report a case of granulocytic sarcoma presented as a recurrent breast tumor in a 42-year-old woman with no history of leukemia. The case was initially diagnosed as malignant lymphoma on a previous biopsy specimen and she refused chemotherapy. At the time of recurrence of the breast tumor, the patient showed full-blown features of leukemia. This case of rare tumor suggests that differential diagnosis should be considered when malignant lymphoma of the breast is detected.
Adult ; Breast Neoplasms/surgery ; Breast Neoplasms/pathology ; Breast Neoplasms/diagnosis+ACo- ; Carcinoma, Lobular/diagnosis ; Case Report ; Cell Nucleus/ultrastructure ; Diagnosis, Differential ; Diagnostic Errors+ACo- ; Female ; Human ; Leukemia, Myelocytic, Acute/pathology ; Leukemia, Myelocytic, Acute/diagnosis+ACo- ; Lymphoma, Large-Cell, Diffuse/diagnosis+ACo- ; Neoplasm Recurrence, Local/pathology

We report a case of granulocytic sarcoma presented as a recurrent breast tumor in a 42-year-old woman with no history of leukemia. The case was initially diagnosed as malignant lymphoma on a previous biopsy specimen and she refused chemotherapy. At the time of recurrence of the breast tumor, the patient showed full-blown features of leukemia. This case of rare tumor suggests that differential diagnosis should be considered when malignant lymphoma of the breast is detected.
Adult ; Breast Neoplasms/surgery ; Breast Neoplasms/pathology ; Breast Neoplasms/diagnosis+ACo- ; Carcinoma, Lobular/diagnosis ; Case Report ; Cell Nucleus/ultrastructure ; Diagnosis, Differential ; Diagnostic Errors+ACo- ; Female ; Human ; Leukemia, Myelocytic, Acute/pathology ; Leukemia, Myelocytic, Acute/diagnosis+ACo- ; Lymphoma, Large-Cell, Diffuse/diagnosis+ACo- ; Neoplasm Recurrence, Local/pathology