Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Dedifferentiation, a change in the histologic character and clinical behavior of a tumor to a more immature and aggressive one, occurs in approximately 11% of all chondrosarcomas. The original lesion is usually a low-grade chondrosarcoma. Clear cell chondrosarcoma is a rare cartilaginous tumor of low-grade malignancy with a preference for the ends of long bones. It is usually curable by resection. Recurrence commonly follows inadequate surgery, and metastases to lung, brain, and bones can develop. However, dedifferentiation has not yet been described in association with clear cell chondrosarcoma. Three patients are described who were initially diagnosed as having clear cell chondrosarcoma of the femur. Two were treated with en bloc resection for a clear cell chondrosarcoma. One of these had an undifferentiated sarcoma in a local recurrence after 6 years. In the second, metastasis of the clear cell chondrosarcoma developed 5(1/2) years after surgery; autopsy revealed undifferentiated sarcoma in the lung, heart, and lumbar spine. The third patient had dedifferentiated clear cell chondrosarcoma at the time of resection following the biopsy diagnosis of clear cell chondrosarcoma. All three died with metastatic disease. These three patients represent three different manifestations of dedifferentiation-at initial diagnosis, at recurrence, and at metastasis. To our knowledge, this is the first description of dedifferentiation occurring in clear cell chondrosarcoma.
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PMID:Dedifferentiated clear cell chondrosarcoma. 1093 48

Clear cell chondrosarcoma is a rare tumor that can be easily confused with benign tumors. The aim of this report is to present a case that is rare, initially diagnosed as aneurysmal bone cyst and then chondroblastoma, and has an interesting extension pattern. A 41-year-old male patient was treated for an apparently benign cystic lesion of the right proximal femur by intralesional excision and bone grafting. The pathological diagnosis was aneurysmal bone cyst. Two years later, the patient presented with a pathological fracture at the same site and a total hip arthroplasty was performed. The pathological specimen was diagnosed as chondroblastoma. Three years later, clinical and radiological examination of the patient revealed a large mass located on the intrapelvic side of the acetabulum. There was no evidence of distant metastases. Both tumors were resected with a wide margin on the femoral side and a marginal margin on the intrapelvic side. The extremity was reconstructed with a resection-type total hip prosthesis. Again, the pathological diagnosis was chondroblastoma. The patient developed a deep infection that was treated by antibiotic therapy and surgical debridement. One year later, there was recurrence of the femoral and intrapelvic masses and right hemipelvectomy was performed; the specimen was reported as clear cell chondrosarcoma. Since then, the patient has been leading an active life, and there is no evidence of local recurrence or distant metastasis. Clinically and pathologically, clear cell chondrosarcoma may be confused with benign bone tumors. This caused a delay in the final diagnosis of this patient and he received inadequate surgical treatment, leading to a hemipelvectomy. We also found that the intrapelvic mass seemed to have developed independently on the intrapelvic side of the acetabulum. We were unable to find an exact explanation for this finding and postulated that tumor cells might have been seeded into the inner wall of the acetabulum during acetabular preparation of the total hip prosthesis.
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PMID:Clear cell chondrosarcoma of the proximal femur with intrapelvic extension. 1555 92

Necrosis of the femoral head represents a special form of aseptic bone necrosis because it develops in the high load-bearing region of the hip joint. The incidence of the disease is not precisely known. Characteristic macroscopic findings include articular cartilage detachment in the load-bearing zone immediately below the subchondral osseous lamella, the bordering necrosis of the marrow space and bone cells, incursion of the spongiosa, and the gradual loss of roundness of the femoral head. The cause of the disease is a disorder in circulation supplying the proximal femur. The disease frequently occurs in disorders of lipometabolism, after steroid medication, in sickle cell anemia and chronic alcoholism, and after fractures of the femoral neck. Histopathological classification should distinguish between arthropathy with secondary femoral head necrosis and primary forms. Differential diagnosis based on histological findings should distinguish between primary coxarthrosis with secondary femoral head necrosis, metastases, and a few bone tumors in the epiphyses. Clear cell chondrosarcoma is particularly important in the context.
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PMID:[Pathohistology of femoral head necrosis]. 1745 Mar 48

Clear cell chondrosarcoma is a rare bone neoplasm with low-grade clinical course and the potential to metastasize to the skeleton and lungs. The aim of this report is to present a case that is extremely rare, but in accordance with the literature where the clear cell chondrosarcoma reportedly has a tendency for late metastases. In our patient the primary surgery was intralesional, since it was mistakenly interpreted as a benign tumour in the early 80s. The local recurrence and lung metastases occurred, however, 29 years after the initial treatment. The local recurrence was resected with wide margins, no additional surgery or oncological treatments were given, and two and half years postoperatively patient is doing well and there is no progression in the disease. In conclusion, it is important to have a long follow-up to the clear cell chondrosarcoma patients even for decades or lifelong, because the malignancy tends to metastasize or recur after an extended period. The course of metastasized disease may be unusually slow, so relatively aggressive treatment in metastasized and recurring cases is justified.
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PMID:An unusual case of clear cell chondrosarcoma with very late recurrence and lung metastases, 29 years after primary surgery. 2514 49

Clear cell chondrosarcoma is a rare chondrosarcoma variant often involving the long bone epiphyses of young to middle aged adults. We report herein a case involving the left femoral head in a 25-year-old female with a 3-month history of worsening left hip pain. Radiographs revealed a complex, multifocal and lytic lesion centered in the left proximal femoral epiphysis with involvement of the femoral neck. Computed tomography-guided fine needle aspiration biopsy with concomitant core needle biopsy was performed, and a diagnosis of clear cell chondrosarcoma was rendered. Cytologic smears revealed aggregates of matrix material accompanied by a population of mostly uniform spindled to epithelioid and histiocytoid cells, rarely accompanied by osteoclast-type giant cells. The patient underwent surgical resection with -total hip replacement, and subsequent pathologic examination confirmed the initial needle biopsy diagnosis. There has been no evidence of local recurrence or distant metastases with 3-years follow-up. To our knowledge, this is the first reported example of a primary clear cell chondrosarcoma initially evaluated by fine needle aspiration biopsy.
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PMID:Fine needle aspiration biopsy diagnosis of primary clear cell chondrosarcoma: A case report. 2892 93

Clear cell chondrosarcoma is an extremely rare malignant neoplasm. The rarity and slow-growing nature of this tumour often lead to prolonged symptoms and also initial misdiagnosis with benign lesions such as chondroblastoma. It can also be confused with avascular necrosis of the femoral head when the lesion is located in the femoral head, as was in the case we report. The patient was kept on observation and conservative treatment for almost 9 years before the correct diagnosis and appropriate treatment. Wide local resection with negative margins forms the mainstay of treatment since intralesional procedures predispose to high local recurrence rate. A prolonged follow-up is recommended since late local recurrences and metastases are common.
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PMID:Clear cell chondrosarcoma of proximal femur: a rare entity with diagnostic dilemma. 3312 29