11/894. chondrosarcoma in a patient with McCune-Albright syndrome. Report of a case. A case of McCune-Albright syndrome with acromegaly and chrondrosarcoma is reported. The potential role of chronic growth hormone overproduction in the occurrence of malignant transformation and the possible value of bisphosphonates in the treatment of bone fibrous dysplasias are discussed. ( info) |
12/894. Orbital chondrosarcoma developing in a patient with Paget disease. PURPOSE: To describe the radiologic, histopathologic, and cytogenetic features of an orbital chondrosarcoma developing in a patient with Paget disease. methods: A 64-year-old woman presented with rapidly progressive proptosis of her right eye. Computed tomographic scans, histopathologic examination, and cytogenetic analysis were performed. RESULTS: Computed tomographic scans disclosed osseous changes of the temporal and frontal bones, with areas of high density consistent with Paget disease. A soft-tissue tumor in the right lateral orbital wall was consistent with Paget sarcoma. On histology, a chondrosarcoma was diagnosed, which was confirmed by fluorescent in situ hybridization. CONCLUSION: This is a unique case of orbital chondrosarcoma developing in a patient with Paget disease. ( info) |
13/894. Recurrent chondrosarcoma of the cranial base: a durable response to ifosfamide-doxorubicin chemotherapy. The case of a 46-year old woman with recurrent chondrosarcoma of the cranial base, refractory to neurosurgical intervention and external radiotherapy is reported. She received five cycles of systemic chemotherapy utilizing ifosfamide and doxorubicin which resulted in a durable clinical and radiographic response lasting 52 months. A review of the management options for recurrent chondrosarcoma of the cranial base is also presented. ( info) |
14/894. chondrosarcoma of the proximal phalanx. An 87-year-old male presented with a painless, large mass on the dorsum of the left foot. He reported that the mass had first appeared 10 years ago and now had become so large that he could no longer tie his shoe. The mass originated from the proximal aspect of the second digit, encompassing the second web space and distal one third of the second and third metatarsals. Surgical excision of the mass was performed and pathologic diagnosis of the specimen confirmed a grade 1 chondrosarcoma. As expected with a lower grade chondrosarcoma, the patient did not have metastasis and fully recovered. While the occurrence of chondrosarcoma is not uncommon, it rarely affects the foot. This appears to be the third case of chondrosarcoma appearing in the proximal phalanx second digit of the foot. ( info) |
15/894. Clear cell chondrosarcoma of the pelvis in a skeletally immature patient. We report on a case of clear cell chondrosarcoma (CCCS) of the left iliac bone in a 12-year-old skeletally immature boy. Radiographic examination revealed an aggressive osteolytic lesion with areas of mineralization. Fluid-fluid levels were seen on T2-weighted MR images. Laboratory data showed slight elevation of serum alkaline phosphatase. The biopsy specimen showed histological features of CCCS with some resemblance to osteosarcoma, such as prominent irregular osteoid formation among clear tumor cells. Surgical treatment was accomplished without pre- or post-operative chemotherapy. Because of the patient's age, elevated serum alkaline phosphatase, and histopathology with prominent osteoid production, this case could be confused with osteosarcoma. Although CCCS is an extremely rare bone tumor in children, it is important to be aware that it may arise in a skeletally immature patient. CCCS, unlike osteosarcoma, is not treated with neo-adjuvant chemotherapy. ( info) |
16/894. Conservative surgery for chondrosarcoma of the first metacarpal bone. A rare case of a chondrosarcoma of the first metacarpal bone is presented. The lesion was radiographically interpreted initially as an enchondroma and treated conventionally by curettage and cancellous autologous bone grafting. After final histology, a low-grade chondrosarcoma was reported. A resection of the entire first metacarpal bone was performed, followed by reconstruction using an autologous corticocancellous bone graft and plate fixation, creating arthrodeses of the adjacent joints. Although isolated enchondromas are considered to have no malignant potential, histological examination is essential to rule out malignancy. A preoperative biopsy should be recommended in lesions suspected to be chondromas. Chondrosarcomas are rarely located in bones of the hand, where they are usually treated by amputation. With the case presented we wish to advocate that cases of low-grade, intraosseous chondrosarcoma (stage IA) can be treated by conservative surgery, especially when it is located in the thumb. ( info) |
17/894. 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. ( info) |
18/894. 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. ( info) |
19/894. 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. ( info) |
20/894. High-grade extraskeletal myxoid chondrosarcoma: a high-grade epithelioid malignancy. AIMS: Extraskeletal myxoid chondrosarcoma is typically a low-to-intermediate grade sarcoma that is associated with a prolonged clinical course. High-grade forms are rare and not well characterized. In this series we report the clinicopathological, immunohistochemical and ultrastructural findings in four cases of high-grade extraskeletal myxoid chondrosarcoma. methods AND RESULTS: The patients were three men and one woman (ages 34-73 years) with tumours located in the thigh (two cases), paraspinal soft tissue and perineum. Three patients had metastases, one at 12 weeks, one at 10 months, and one at presentation of recurrent tumour. In the latter case the original tumour was low grade and became high grade when it recurred 3.5 years later. All three patients died of disease. One patient was lost to follow-up. The most striking histological feature in all four tumours was the presence of numerous large epithelioid cells. These cells were arranged in cords within myxoid matrix and in sheets devoid of matrix. Two tumours had areas of conventional extraskeletal myxoid chondrosarcoma intermixed with the high-grade areas. One tumour showed transition to high-grade spindle cell sarcoma. One tumour had cells with rhabdoid features. Immunohistochemically, two tumours focally expressed S100 protein, and one focally expressed EMA. All were negative with cytokeratin, desmin, smooth muscle actin, HMB45, CD31 and CD34. Ultrastructural features in three cases were compatible with chondrosarcoma; one tumour had aggregates of microtubules within rough endoplasmic reticulum, a characteristic feature of this tumour. CONCLUSIONS: High-grade extraskeletal myxoid chondrosaroma is a rare and aggressive soft tissue sarcoma, and should be included in the differential diagnosis of other epithelioid malignancies. ( info) |