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

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

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

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

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

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

Chondrosarcomas are malignant tumors of cartilage that rarely involve the sinonasal region, and myxoid chondrosarcoma is a rare histologic variant of chondrosarcoma that usually occurs in the soft tissue of extremities. Although several case reports and results of small series of chondrosarcomas in the sinonasal region in children are available, myxoid type chondrosarcoma is extremely rare. We recently experienced a case of low grade myxoid chondrosarcoma involving the sinonasal cavity in a 10-year-old boy, and here we report its radiologic-pathologic findings. In this case, chondroid calcification on CT and septal and marginal enhancement on MRI suggested a chondrosarcoma. Whole body PET-CT demonstrated no definite metastatic lesion and a low peak standardized uptake value primary tumor. However, no definite distinguishing imaging features were observed that distinguished low grade myxoid chondrosarcoma from conventional chondrosarcoma.
Bone Neoplasms/*diagnosis/radiotherapy/surgery ; Child ; Chondrosarcoma/*diagnosis/radiotherapy/surgery ; Diagnosis, Differential ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Male ; Myxosarcoma/*diagnosis/radiotherapy/surgery ; Nasal Cavity/*pathology/*radiography/surgery ; Paranasal Sinuses/*pathology/*radiography/surgery ; Positron-Emission Tomography ; Rare Diseases ; Tomography, X-Ray Computed ; Whole Body Imaging