Cases reported "Chondrosarcoma"

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1/145. Neuro-ophthalmologic manifestations of Maffucci's syndrome and Ollier's disease.

    patients with Ollier's disease (multiple skeletal enchondromas) and Maffucci's syndrome (multiple enchondromas associated with subcutaneous hemangiomas) may develop skull base chondrosarcomas or low-grade astrocytomas as a delayed consequence of these disorders. We report three patients with Ollier's disease and Maffucci's syndrome who had diplopia as the initial manifestation of intracranial tumors. Since patients with Maffucci's syndrome and Ollier's disease are at risk for the delayed development of brain and systemic neoplasms, neuroophthalmologists must be aware of the need for long-term surveillance in patients affected by these conditions.
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2/145. Malignant phyllodes tumor with chondrosarcomatous differentiation: report of a case with cytological presentation.

    Malignant phyllodes tumor is a rare breast tumor with neoplastic epithelial and stromal components. The stromal component may show homologous and heterologous sarcomatous elements, including chondrosarcomatous and osteosarcomatous differentiation. Because these tumors may present with an almost exclusively sarcomatous component, it is important for the pathologist to include this entity in the diagnostic considerations of fine-needle aspirations of breast neoplasms showing sarcomatous differentiation. Following surgical excision, careful examination of the gross specimen and thorough sampling of the specimen is recommended before rendering a definitive histologic diagnosis. We describe the cytologic and histologic findings in a case of malignant phyllodes tumor with sarcomatous overgrowth showing predominantly chondrosarcomatous differentiation.
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3/145. temporal bone tumours in patients irradiated for nasopharyngeal neoplasm.

    radiation-associated tumours are rare complications of radiotherapy. This study seeks to highlight and discuss the clinically challenging problem of radiation-associated tumours (rats) in the temporal bones of seven patients previously irradiated for nasopharyngeal neoplasm. Seven patients (six males and one female) with radiation-associated temporal bone tumours are presented (five squamous cell carcinomas, one osteogenic sarcoma and one chondrosarcoma). The initial nasopharyngeal disease for which radiotherapy was indicated was nasopharyngeal carcinoma (six patients) and nasopharyngeal lymphoma (one patient). The latency period between radiotherapy and presentation of temporal bone tumours ranged from five years to 30 years with a mean of 12.9 years. All the patients underwent surgical tumour resection. Three patients had post-operative radiotherapy and one patient underwent pre- and post-operative chemotherapy. Two patients died from the disease within three months of treatment with one patient surviving 36 months at the time of writing. One patient died from an unrelated medical condition three months after surgery. With refinement in radiotherapy techniques and the resultant increase in patient survival, there may be more patients with radiation-associated tumours in the future. It remains imperative for clinicians to be vigilant when patients previously irradiated for nasopharyngeal carcinoma present with otological symptoms as the key to the successful management of this condition lies in the early detection and expedient treatment of this difficult disease.
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4/145. Mesenchymal chondrosarcoma associated with Goldenhar's syndrome.

    Goldenhar's syndrome is characterised by bony abnormalities of the face, jaw and vertebral column. We report the first case of the development of a primary malignant neoplasm (mesenchymal chondrosarcoma) initially misdiagnosed as a meningioma, occurring in association with this syndrome.
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5/145. Extraskeletal myxoid chondrosarcoma of the knee.

    Extraskeletal myxoid chondrosarcoma is an uncommon neoplasm, accounting for less than 2% of all soft tissue sarcomas. It affects adult males with a median age in the fifth decade at the time of diagnosis. The tumor usually arises in the deep soft tissues, especially in the lower extremities. patients present with a gradually enlarging mass that may or may not be associated with pain. This report describes a 25-year-old man who initially presented with a 4- to 5-year history of right knee pain and an enlarging mass in the right knee. Evaluation revealed a cartilaginous neoplasm with no evidence of metastatic disease. The tumor was widely excised and an allograft reconstruction was performed. The patient was closely followed with an eventual above the knee amputation for recurrent myxoid chondrosarcoma. At 34 months, retroperitoneal metastases were noted on abdominal CT. The patient underwent a left radical nephrectomy, renal vein thrombectomy and enucleation of the mass in the right kidney, distal pancreatectomy, and splenectomy. The patient received postoperative chemotherapy. Forty-eight months after initial diagnosis, the patient was found to have recurrent abdominal and retroperitoneal lesions. At 64 months, the patient died from complications of extraskeletal myxoid chondrosarcoma.
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6/145. Craniofacial resection of a nasoseptal chondrosarcoma: case report and review of the literature.

    BACKGROUND: chondrosarcoma of the nasal septum is a rare, malignant neoplasm. A case is presented that illustrates the evaluation and treatment of this malignancy, reviews the relevant literature, discusses surgical approaches, and assesses adjuvant, nonsurgical therapy. methods: A craniofacial approach using an extended, bifrontal craniotomy and lateral rhinotomy with medial maxillectomy resulted in gross total resection at surgery. Because permanent sections of bone margins after decalcification were positive, conformal external beam radiation was used. RESULTS: The patient made a complete recovery, returned to work, and is disease-free 26 months after treatment. CONCLUSIONS: Surgical therapy using an anterior craniofacial resection is the preferred approach. External beam radiation therapy is potentially indicated for the following: positive or close surgical margins on permanent histopathology, extensive tumor with known residual at operation, or local recurrence not amenable to resection. Because of late local recurrence, lifelong follow-up is required.
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7/145. Chondroid chordoma: fine-needle aspiration cytology with histopathological, immunohistochemical, and ultrastructural study of two cases.

    Chondroid chordoma is a controversial and confusing entity that was originally described by Heffelfinger et al. (Cancer 1973; 32:410-420) as a biphasic malignant neoplasm possessing elements of both chordoma and cartilaginous tissue. Fine-needle aspiration (FNA) cytology of chondroid chordoma has not been described. The aim of our investigation was to characterize the chondroid area of chondroid chordoma and to compare the FNA features with those of well-differentiated chondrosarcoma. Clival and cervical spine chondroid chordomas were studied with light microscopy, immunohistochemistry, and electron microscopy. Chondroid chordomas demonstrated an epithelial nature by immunohistochemistry and ultrastructural studies. The FNA smears showed low cellularity, with loosely arranged or dispersed round cells in a myxoid background. Although the smears were similar to those of well-differentiated chondrosarcomas, they showed a positive reaction for epithelial markers. These findings reveal that chondroid chordoma is a variant of chordoma which possesses a hyaline matrix. Immunohistochemical demonstration of epithelial markers is useful to distinguish it from chondrosarcoma. Diagn. Cytopathol. 1999; 21:335-339.
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8/145. Malignant myxoid endobronchial tumour: a report of two cases with a unique histological pattern.

    AIMS: To present two cases of malignant endobronchial myxoid tumours with a highly distinctive sarcomatoid pattern not previously described at this site, and discuss their histogenesis in relation to previously documented endobronchial neoplasms. methods AND RESULTS: Both tumours presented in young adult females and were purely sarcomatoid with interweaving cords of small uniform, rounded or slightly elongated cells lying within a myxoid stroma. The stroma was alcian blue positive, but sensitive to hyaluronidase in both cases. The tumour cells contained a small volume of periodic acid-Schiff-positive eosinophilic cytoplasm and stained positively for vimentin only, but there also was a prominent background population of CD68-positive dendritic cells. Ultrastructural studies showed that the tumour cells contained an excess of rough endoplasmic reticulum, with some of the cisternae appearing dilated, and scalloping of the cell surfaces, although no intracisternal tubules were identified. CONCLUSIONS: Although the histological pattern was most reminiscent of extraskeletal myxoid chondrosarcoma, the sensitivity of the stroma to pretreatment with hyaluronidase precluded the diagnosis. However, there were similarities with the sarcomatoid component of malignant salivary gland-type mixed tumours of the lung and this tumour possibly represents a variant of a bronchial gland tumour. Despite this uncertainty over origin, this pattern should be recognized as part of the differential diagnosis of myxoid tumours in the lung, as an apparently indolent type of malignant endobronchial neoplasm.
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9/145. Parachordoma of the tibia: report of a rare case.

    We report a case of recurrent parachordoma of the left anterior tibial region in a 64-year-old male patient. The tumor was a periosteal tender mass, and, histologically, displayed vague nodules of spindle to rounded eosinophilic cells embedded in a myxoid matrix. Large vacuolated (physalphorouslike) cells were noted as in sacrococcygeal chordoma. This tumor should be differentiated from myxoid chondrosarcoma, myxoid liposarcoma, chondromyxoid fibroma, and metastatic chordoma. The presence of physaliphorous cells in the tumor with positive immunoreactions caused by cytokeratin rules out the diagnosis of another myxoid tumor. The differential diagnosis from metastatic chordoma is basically made by clinicians. Even though parachordoma is usually regarded as a benign soft tissue neoplasm, two recurrences occurred in our case. Since the reported cases, including ours, have diverse clinical courses, it is essential to follow-up the patient carefully.
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10/145. chondrosarcoma of the foot.

    Chondrosarcomas have been seen clinically as aggressive tumors, with radiographic and histologic findings consistent with malignancy; however, they may have an insidious clinical course, with relatively benign radiographic and histopathologic findings. Rarely, a chondrosarcoma may present as a primary lesion of the foot. It is important to recognize these lesions as malignancies. The authors provide an overview of these neoplasms and describe a case of a low-grade chondrosarcoma in a 64-year-old woman.
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