The term myxosarcoma is currently not used in standard classification for soft tissue tumors, but restricted to cardiac tumors. Primary cardiac myxosarcoma is a very rare disease and is difficult to differentiate from myxoma clinically and pathologically. We report a case of left atrial myxosarcoma with widespread systemic metastasis in a 21-yr-old male. The patient presented with sudden onset of intermittent dyspnea and orthopnea. Echocardiography showed a mobile, pedunculated tumor, 7.5x5x2 cm in size, at left atrium. Histologically, the excised tumor showed an amorphous finely fibrillar and mucinous stroma, in which irregular cords and clusters of lepidic cells and large stellate cells with plump vesicular nuclei resembled the usual type of cardiac myxoma were noted. And it showed focally cellular area with great nuclear pleomorphism and frequent mitoses. The patient received combination chemotherapy, peripheral blood stem cell collection transplantation and operations for systemic metastases in the brain, skeletal muscle and lung. He is alive at present 37 months after initial diagnosis and has no more new metastatic lesion.
Adult ; Case Report ; Heart Neoplasms/therapy ; Heart Neoplasms/pathology* ; Human ; Male ; Myxosarcoma/therapy ; Myxosarcoma/secondary* ; Myxosarcoma/pathology*

The authors present a case of primary intracranial extraosseous myxoid chondrosarcoma without any attachment to the cranium or the meninges. The clinical and radiological findings of the primary intraparenchymal tumor are described with a review of the literature concerning cranial and intracranial myxoid chondrosarcoma.
Adolescent ; Adult ; Bone Neoplasms/diagnosis* ; Bone Neoplasms/pathology ; Bone Neoplasms/surgery ; Child ; Chondrosarcoma/diagnosis* ; Chondrosarcoma/pathology ; Chondrosarcoma/surgery ; Female ; Human ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Myxosarcoma/diagnosis* ; Myxosarcoma/pathology ; Myxosarcoma/surgery ; Tumor Markers, Biological

Adult ; Diagnosis, Differential ; Heart Neoplasms ; metabolism ; pathology ; surgery ; Humans ; Immunohistochemistry ; Liposarcoma, Myxoid ; metabolism ; pathology ; surgery ; Male ; Myxoma ; metabolism ; pathology ; Myxosarcoma ; metabolism ; pathology ; Pericardium ; S100 Proteins ; metabolism ; Vimentin ; metabolism

Adenocarcinoma, Mucinous ; pathology ; Breast ; pathology ; Breast Diseases ; pathology ; Breast Neoplasms ; pathology ; Carcinoma, Papillary ; pathology ; Carcinoma, Signet Ring Cell ; pathology ; Cystadenocarcinoma, Mucinous ; pathology ; Diagnosis, Differential ; Female ; Fibroadenoma ; pathology ; Fibrosarcoma ; pathology ; Humans ; Mucocele ; pathology ; Myxosarcoma ; pathology

A case of primary malignant fibrous histiocytoma of the breast is reported. The patient was a 48-yr-old woman with a huge tumor involving almost the entire left breast. The central portion of her left breast was already rotted by extensive necrosis and inflammation. She was treated by radical mastectomy and axillary lymphadenectomy to level I. Pathologic examination supported by an immunohistochemical staining confirmed the tumor as malignant fibrous histiocytoma of giant cell type. Axillary lymph nodes were free from tumor metastasis. She had not taken any postoperative adjuvant therapy. The metastasis to lungs was found 2 months after the operation, and she died within 6 months.
Breast/pathology ; Breast Neoplasms/*diagnosis/*pathology ; Female ; Giant Cells/*pathology ; Histiocytoma, Fibrous/*diagnosis/*pathology ; Human ; Immunohistochemistry ; Lung Neoplasms/mortality/secondary ; Middle Aged ; Myxosarcoma/diagnosis/pathology ; Necrosis ; Neoplasm Metastasis ; Sarcoma/diagnosis/pathology

Cyclin E ; metabolism ; Dermatofibrosarcoma ; pathology ; Diagnosis, Differential ; Fibrosarcoma ; classification ; metabolism ; pathology ; Humans ; Ki-67 Antigen ; metabolism ; Liposarcoma ; pathology ; Myxoma ; pathology ; Myxosarcoma ; metabolism ; pathology ; Proliferating Cell Nuclear Antigen ; metabolism ; Proto-Oncogene Proteins p21(ras) ; metabolism

Adult ; Diagnosis, Differential ; Foot Diseases ; metabolism ; pathology ; surgery ; Humans ; Immunohistochemistry ; Male ; Myxosarcoma ; metabolism ; pathology ; surgery ; Neoplasms, Muscle Tissue ; pathology ; Soft Tissue Neoplasms ; metabolism ; pathology ; surgery ; Tenosynovitis ; pathology ; Vimentin ; metabolism

Aged, 80 and over ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Diagnosis, Differential ; Fasciitis ; pathology ; Fibroblasts ; pathology ; Fibrosarcoma ; metabolism ; pathology ; surgery ; Forearm ; Histiocytoma, Malignant Fibrous ; pathology ; Humans ; Male ; Myxosarcoma ; metabolism ; pathology ; surgery ; Soft Tissue Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism ; alpha 1-Antichymotrypsin ; metabolism

OBJECTIVETo study the clinicopathological features, immnophenotype and differential diagnosis of low-grade fibromyxoid sarcoma (LGFMS).

METHODSThe clinical, radiological and pathological features of 9 cases of LGFMS were reviewed.

RESULTSThe patients consisted of six males and three females with ages ranging from 11 to 65 years (mean 31.4 years). Clinically, most cases presented as slowly growing painless masses located in the extremities, trunk and neck. Two cases had a history of a rapid recent enlargement. Three cases presented with recurrent diseases after incomplete resection. Ultrasound showed heterogeneous signal. Precontrast CT and T1-weighted MRI showed a nodular mass of low to isodensity, while contrast-enhanced CT and T2-weighted MRI demonstrated contrasting zonal areas of hypo/hyperintensity. The tumors measured 3 to 16 cm (mean 7.7 cm), with a fibrous to myxoid appearance on cut surface. Histologically, they were composed of alternating collagenous and myxoid areas. A transition between the two areas could be identified. Giant rosette-like structures were observed in 3 cases. The center of the giant rosettes was composed of eosinophilic collagen, which showed centrifugal in arrangement. There was also gradual transition between the giant rosettes and the fibromyxoid component. On high magnification, the tumor was composed of ovoid to spindle-shaped cells with hyperchromatic nuclei. Nuclear atypia was inconspicuous. The tumor cells were arranged mostly in interlacing fascicles or whorls. Vessels were not prominent and necrosis was absent. Immunohistochemically, the tumor cells showed unique staining for vimentin, consistent with a fibroblastic differentiation.

CONCLUSIONSLGFMS is a distinctive low grade fibroblastic sarcoma of young adults. Recognizing the characteristics of the rare entity may help to avoid misdiagnosis. Wide local excision is recommended to avoid local recurrences.

Adolescent ; Adult ; Aged ; Child ; Diagnosis, Differential ; Extremities ; pathology ; Female ; Fibrosarcoma ; diagnosis ; metabolism ; pathology ; surgery ; Head and Neck Neoplasms ; diagnosis ; metabolism ; pathology ; surgery ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Myxosarcoma ; pathology ; Soft Tissue Neoplasms ; diagnosis ; metabolism ; pathology ; surgery ; Vimentin ; metabolism ; Young Adult

OBJECTIVETo study the clinicopathologic features, immunophenotypes and differential diagnosis of myxoinflammatory fibroblastic sarcoma (MIFS).

METHODSThe clinical and pathologic features of 6 cases of MIFS were analyzed. Immunohistochemical study was performed using the standard EnVision method.

RESULTSThere were altogether 2 adult males and 4 adult females (median age = 47 years and mean age = 50 years). Three cases were located in the lower extremities, 2 in the upper limbs and 1 in the axillary region. Common presentation included slowly growing mass or swelling in the extremities, accompanied by mild pain or tenderness. Grossly, the tumor appeared multinodular and ranged from 2.5 cm to 4.6 cm in diameter (mean = 3.4 cm). Microscopically, there was a dense inflammatory infiltrate merging with hyaline and myxoid zones in various proportions. Spindle-shaped tumor cells were seen admixed with large atypical cells which distributed singly or in small clusters, amongst an inflammatory, hyaline or a myxoid background. These atypical cells had large nuclei and prominent nucleoli, resembling virocytes, Reed-Sternberg cells or ganglion cells. Mitotic figures were rarely identified. Extracellular mucin associated with scattered monovacuolated or multivacuolated lipoblast-like cells was noted. Immunohistochemically, these bizarre cells were consistently positive for vimentin, but negative for a panel of antibodies including LCA, CD15, CD30, CD34, CD68, S-100, HMB45, AE1/AE3, smooth muscle actin and desmin. Follow-up result was available in 4 cases; and 2 of them showed local recurrence after an incomplete excision. There was no evidence of distant metastasis.

CONCLUSIONSMISF is a low-grade sarcoma of fibroblastic differentiation. Awareness of the clinical and pathologic characteristics is helpful in arriving at the correct diagnosis and distinction from benign inflammatory fibromyxoid lesions.

Adult ; Aged ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Extremities ; Female ; Fibroblasts ; pathology ; Fibrosarcoma ; metabolism ; pathology ; surgery ; Follow-Up Studies ; Humans ; Inflammation ; pathology ; Male ; Middle Aged ; Myxosarcoma ; metabolism ; pathology ; surgery ; Neoplasm Recurrence, Local ; Soft Tissue Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism