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

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

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

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

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