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PubMed1989

Chondrosarcoma of the posterior fossa--case report.

The authors describe a case of well differentiated (grade I) chondrosarcoma in a 52-year-old male. The tumor originated in the posteromedial wall of the left petrous bone and extended into the posterior fossa and cervical spine. He showed impaired functions of the left VII to XII cranial nerves. Plain skull x-rays demonstrated erosion of the left petrous and occipital bones, with speckled calcifications. Computed tomography showed a huge mass of slightly high density with mushroom-like calcifications, which was poorly enhanced by contrast medium. Inversion recovery magnetic resonance imaging clearly defined the low-intensity lesion, while the long spin-echo image depicted the tumor as mixed-intensity. On electron microscopic examination, the tumor cells were rich in Golgi apparatus, endoplasmic reticulum, glycogen granules and mitochondria in the cytoplasm, and formed many vacuoles crossing the cell membrane. These vacuoles were suggested to play an important role in matrix formation. Thirty nine other reported cases of chondrosarcoma are reviewed, and the two reported posterior fossa chondrosarcomas are compared with that of ours.

Chondrosarcoma↗
PubMed1995

Primary spinal chondrosarcoma localized in the cervical spinal canal and intervertebral foramen--case report.

A 48-year-old male presented with a very rare case of spinal chondrosarcoma localized in the cervical spinal canal and intervertebral foramen, but without marked destruction of the vertebral column. Spinal chondrosarcoma is characterized by radiological evidence of destruction of the surrounding bone structure and mottled calcification. Magnetic resonance imaging was useful in the diagnosis and determination of the extent of this spinal chondrosarcoma. However, preoperative differential diagnosis of the tumor was not possible based on neuroimaging evidence.

Cervical Vertebrae↗
PubMed1997

Secondary chondrosarcoma in osteochondromas. Medullary extension in 15 of 45 cases.

We reviewed 45 secondary chondrosarcomas: 16 arising in solitary osteochondromas and 29 in 27 patients with multiple osteochondromas. Less than radical or wide primary surgery resulted in local recurrence in 8 of 14 lesions: 2 of 2 intralesional excisions, 5 of 11 marginal excisions, and in one case with radiotherapy alone. No local recurrence was found after a primary wide surgical procedure in 30 lesions and a radical procedure in 1 lesion after a mean of 8 (0.2-22) years' follow-up. 5 patients had died: 3 of pulmonary metastases (2 stage IB, 1 stage IIB), and 2 of local tumor invasion, both in the spine. Medullary invasion occurred in 15 of 45 lesions, this was often than reported in secondary chondrosarcoma. Medullary invasion was not always detected preoperatively, even when MR or CT examinations had been performed. Therefore, we recommend that a secondary chondrosarcoma should be removed with a wide surgical resection, including a part of the underlying bone, to keep the local recurrence risk low. There were no clinical or prognostic differences between tumors that had arisen from solitary or from multiple osteochondromas.

Adult↗
PubMed2001

Chordomas and chondrosarcomas of the skull base: results and complications of surgical management.

OBJECT: Chordomas and chondrosarcomas are rare and difficult to treat tumors for which the optimum treatment modality remains controversial. The aim of this study was to evaluate the surgery-related results and complications in a series of patients in whom radical resection was the treatment of choice. METHODS: The authors conducted a retrospective analysis of the surgery-related results and complications associated with chordoma and chondrosarcoma in 64 patients of whom 33 (52%) had previously undergone some form of treatment. Total or near-total excision was achieved in 56% and this rate increased to 68% in patients without prior treatment. The main complications were postoperative cerebrospinal fluid leakage, intraoperative arterial injury, and new-onset cranial nerve deficits. Arterial injury occurred only and perioperative death occurred more often in patients who had undergone previous treatment. CONCLUSIONS: Analysis of the results provides support for a policy of radical excision of chordomas and chondrosarcomas at the time of first presentation. A higher incidence of procedure-related complications is found in patients who have already undergone surgery and radiotherapy.

Adult↗
PubMed2001

A multidisciplinary team approach to skull base chondrosarcomas.

OBJECT: The authors review their experience with treating skull base chondrosarcomas, which are much rarer than skull base chordomas and differ from them in prognosis and treatment. METHODS: Seventeen patients (12 male and five female patients) with histologically verified chondrosarcomas were followed up prospectively over a 12-year period. The mean age at presentation was 35.9 years. Most patients presented with cranial nerve palsies. Seven had undergone surgery prior to referral to the authors' unit. All underwent maximum surgical cytoreduction by the most direct surgical approach; only the two patients harboring the mesenchymal variant underwent radiotherapy. CONCLUSIONS: One patient died of a pulmonary embolus; the patients harboring mesenchymal chondrosarcomas died at 20 and 36 months, respectively, after treatment. Of the remaining patients, 93% were alive 5 years postsurgery and had a projected 10-year survival rate of 84% (mean survival time 9.3 years). These data emphasize the very slow progression of this tumor compared with skull base chordoma.

Adolescent↗
PubMed1989

Dedifferentiated chondrosarcoma of bone.

Dedifferentiated chondrosarcoma is a highly malignant variant of chondrosarcoma. Approximately 11% of chondrosarcomas can be expected to dedifferentiate into more anaplastic lesions. Radiographic evidence of a benign-appearing cartilage lesion adjacent to a lytic, highly aggressive lesion suggests dedifferentiation. Despite radical surgical treatment, the prognosis is unfavorable, with a 5 year survival rate of 10%.

Aged↗
PubMed2000

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.

Amputation, Surgical↗
PubMed1999

[Costal chondrosarcoma. 4 cases].

BACKGROUND: Tumors of the rib cage are uncommon and malignant in 29% of the cases. Chondrosarcoma predominates, accounting for 40% of all cases of malignant costal tumors. CASE REPORTS: Four patients (3 women, 1 man, mean age 28.2 years) were hospitalized for costal chondrosarcoma. Pain and tumefaction dominated the clinical presentation. Calcifications suggested the diagnosis in 3 cases. Curative surgery was performed in all cases. Postoperative radiotherapy was unable to improve prognosis in 2 patients. DISCUSSION: Chondrosarcoma of the ribs is characterized by a strong potential for invasive extension. Diagnosis is suspected on the basis of imaging findings and confirmed at pathology. Surgery is required. Chemotherapy and radiotherapy do not improve prognosis significantly.

Adolescent↗
PubMed2000

Intracranial extraskeletal mesenchymal chondrosarcoma: case report.

OBJECTIVE AND IMPORTANCE: We present a patient with a dural-based intracranial extraskeletal mesenchymal chondrosarcoma, which was initially thought to be an atypical meningioma. This rare tumor should be considered in the differential diagnosis of young adults with an aggressive-appearing dural-based lesion. CLINICAL PRESENTATION: A 21-year-old woman reported a 3-week history of severe headaches and intermittent nausea and vomiting. Neurological examination revealed right optic nerve swelling. Magnetic resonance imaging demonstrated a large, intensely enhancing extra-axial mass, which appeared to originate from the right side of the falx cerebri with significant mass effect. A presumptive diagnosis of meningioma was made. INTERVENTION: The patient underwent preoperative embolization of the lesion and then underwent a bicoronal craniotomy, gross total resection of the tumor, removal of invaded calvarial bone, and cranioplasty. Pathological examination revealed an extraskeletal mesenchymal chondrosarcoma. Because of the potential for recurrence, the patient received subsequent radiotherapy. She remains free of recurrence 18 months after surgery. CONCLUSION: Intracranial mesenchymal chondrosarcoma is a rare neoplasm that can mimic a meningioma radiographically. We present the first patient in whom this lesion has been documented with computed tomography, cardiography, magnetic resonance imaging, and pathological findings. We emphasize the importance of gross total resection and close follow-up for this potentially aggressive tumor, and we present a review of the literature.

Adult↗
PubMedDate not supplied

[Dorsal medullary compression due to a primary vertebral chondrosarcoma].

INTRODUCTION: Chondrosarcomas are the second commonest type of bony tumors, although they are not commonly found to be vertebral. They may originate in healthy bone or develop in a cartilaginous lesion with sarcomatous degeneration. CLINICAL CASE: A 62 year old woman complained of pain in the back and weakness of her legs. Following magnetic resonance studies a tumour at the level of the D6-D7 vertebrae was diagnosed. This was operated on and an extradural tumour, a grade 11 chondrosarcoma was removed. A local recurrence was removed two years later and three years afterwards she is still asymptomatic. CONCLUSIONS: A spinal chondrosarcoma is a tumour which usually presents with local pain and signs of spinal involvement. On magnetic resonance it is iso-intense on T1 and shows up with contrast. The treatment of choice is radical surgery since it does not respond well to oncological treatment.

Bone Neoplasms↗
PubMed2000

Primary chondrosarcoma of the head and neck in pediatric patients: a clinicopathologic study of 14 cases with a review of the literature.

BACKGROUND: Primary chondrosarcoma of the head and neck in the pediatric age group is rare. The literature contains several single cases and small series; however, to the authors' knowledge, there has been no previous comprehensive larger study to evaluate the clinicopathologic aspects of these tumors. METHODS: Fourteen cases of chondrosarcoma of the head and neck from patients age 18 years or younger, diagnosed between 1970 and 1997, were retrieved from the Otorhinolaryngic-Head & Neck Tumor Registry of the Armed Forces Institute of Pathology. No secondary sarcomas (radiation-induced or arising in association with Maffucci syndrome or Ollier disease) were included. Clinical, radiographic, and histologic features were reviewed and patient follow-up obtained. RESULTS: The patients included 6 girls and 8 boys ages 3-18 years (mean, 11.8 years). Patient symptoms (nasal stuffiness or discharge, sinusitis, headaches, or a mass lesion) were related to tumor location and were present for an average of 7.2 months. No genetic abnormalities were documented. The tumors most frequently involved the maxillary sinus (n=4), followed by the mandible (n=3), nasal cavity (n=2), and neck (n=2), with 1 each of the nasopharynx, orbit, and base of the skull. The tumors ranged in size from 2.0 to 15.0 cm (mean, 3.1 cm). All tumors were invasive and malignant as determined by radiology and/or histology. The tumors were Grade 1 (n=9), Grade 2 (n=1), or Grade 3 (mesenchymal, n=2; dedifferentiated n=2). All patients were treated by surgery, followed by radiation (n=5) and/or chemotherapy (n=2). Follow-up was available for 11 patients; all were alive (at a mean of 14.8 years), with only a single patient demonstrating evidence of residual/ recurrent tumor (at 16.6 years). CONCLUSIONS: Primary head and neck chondrosarcoma in the pediatric population is typically low grade and occurs in the maxillary sinus or mandible. Despite the invasive and high grade nature of some of these tumors, there is an excellent long term prognosis for patients in this age group with tumors in these locations.

Adolescent↗
PubMed1976

[Mediastinal chondrosarcoma (author's transl)].

A 41 years old female patient was operated on account of mediastinal tumour. A chondrosarcoma of 10 X 8 X 8 cm was removed. The patient remained symptomfree and had no complaints over 4 years after the operation, but after this relapse and pulmonary metastasis developed. In connection with the demonstrated case the referring literature of mediastinal chondrosarcomas was surveyed and it could be established that this is the twentieth published case about mediastinal chondrosarcoma.

Adult↗
PubMed2000

Overexpression of cathepsin B and urokinase plasminogen activator is associated with increased risk of recurrence and metastasis in patients with chondrosarcoma.

BACKGROUND: Deregulation of the cellular protease network has been shown to be responsible for aggressive clinical behavior in several common human malignancies. In the current study, the authors evaluated the expression patterns of proteases in patients with chondrosarcoma of bone and correlated these patterns with clinical outcome. METHODS: The expression levels of urokinase plasminogen activator; matrix metalloproteinase types-1, -2, and -9; and cathepsins B and L were determined immunohistochemically in 114 cases of chondrosarcomas of bone and were correlated with their clinicopathologic parameters as well as with long term follow-up data. RESULTS: Overexpression of cathepsin B was associated with a high rate of local recurrence (P = 0.006) and a decreased recurrence free survival (P = 0.005). Overexpression of urokinase plasminogen activator was associated with an increased rate of metastasis (P = 0. 013), a decreased metastasis free survival (P = 0.016), and a decreased 5-year overall survival rate (P = 0.048). The univariate Cox model showed that tumor extension into soft tissue, high histologic grade, and overexpression of cathepsin B were predictors of adverse outcome. Multivariate analysis showed only overexpression of cathepsin B and tumor extension into soft tissue to be independent predictors of local recurrence. CONCLUSIONS: Overexpression of cathepsin B and urokinase plasminogen activator can be used to identify those patients with chondrosarcoma of bone who have an increased risk of local recurrence and distant metastases.

Adult↗
PubMedDate not supplied

Chondrosarcoma of the anterior mandible. A case report.

Chondrosarcomas of the maxillofacial region are extremely rare tumors, accounting for approximately 1% of chondrosarcomas of the entire body. This case represents the 12th case of mandibular symphyseal chondrosarcoma reported in the literature. It presented as a painful swelling of the alveolus, associated with a mobile mandibular incisor. The patient remains free from recurrence six years after a marginal resection.

Adult↗
PubMed1999

[Extraskeletal myxoid chondrosarcoma with neuroendocrine differentiation].

We describe a case of extraskeletal myxoid chondrosarcoma with neuroendocrine differentiation. The tumor occurred in subcutaneous tissue of the right popliteal region in a 50-year-old man. It measured 5 cm in diameter, was well circumscribed, lobular and gelatinous, and lacked any necrosis or hemorrhage. Histologically, the tumor structure was a typical of extraskeletal myxoid chondrosarcoma. The lesion was lobulated and contained small to medium-sized chondroblast-like cells with ovoid hyperchromatic nuclei and without prominent nucleoli. The cells created cords and nests and showed focally a perivascular rosette-like arrangement. A few of the tumor cells were spindle shaped. The myxoid matrix was stained with alcian blue and this reaction was resistant to prior treatment with hyaluronidase. PAS-positive glycogen was found in the cytoplasm of some tumor cells. Immunohistochemically, the tumor cells were diffusely positive for neuron specific enolase, monoclonal synaptophysin and vimentin. Following antibodies gave negative results: desmin, actins, S-100 protein, pancytokeratin, epithelial membrane antigen, chromogranin A, neurofilament protein, myelinic basic protein, glial fibrillary acidic protein. The patient is well four years after the wide excision of tumor and radiotherapy. Neuroendocrine differentiation in extraskeletal myxoid chondrosarcoma was described at first by Chhieng et al. in 1998 (1). Our observation confirms this interesting finding.

Chondrosarcoma↗
PubMedDate not supplied

Prognostic relevance of urokinase type plasminogen activator, its receptor and inhibitors in chondrosarcoma.

The plasminogen activation system plays an important role in enhancing pericellular proteolysis of tumor invasion/metastasis and in autocrine/paracrine tumor growth stimulation. To investigate the prognostic significance of the plasminogen activation system in human chondrosarcoma, the immunohistochemical expression of urokinase-type plasminogen activator (uPA), urokinase-type plasminogen activator receptor (uPAR), plasminogen activator inhibitor, 1 (PAI-1) and 2 (PAI-2) were analyzed in 28 patients with chondrosarcoma. In multivariate survival analysis, histological grade (p = 0.0008) and location (p = 0.02) were independent risk factors for local relapse. For metastasis-free survival, uPA index (p = 0.006) and PAI-2 index (p = 0.04) were independent prognostic factors. PAI-2 index (p = 0.02), uPAR index (p = 0.02) and histological grade (p = 0.03) predicted total survival. These results demonstrated the usefulness of uPA, uPAR and PAI-2 expression as biological prognostic indicator and the importance of the plasminogen activation system in tumor progression and metastasis in chondrosarcoma.

Adolescent↗
PubMed2001

Localization of (99m)Tc HMDP in an extraskeletal myxoid chondrosarcoma: a case report.

Extraskeletal myxoid chondrosarcoma of the lower extremity is rare, and slowly progressive. The authors of this article present the case of a man with progressive enlargement of the right thigh that underwent bone scintigraphy. The bone images showed a diffuse, moderate increase in uptake in the swollen right thigh. Despite chemotherapy, the patient died 28 mo later. At autopsy, it was confirmed that he had extraskeletal myxoid chondrosarcoma of the right thigh, which had metastasized to the upper arms, left scapula, lungs, pleurae, and right lower quadrant of the abdomen. The myxoid chondroid matrix, a major feature of the extraskeletal myxoid chondrosarcoma, is thought to account for the localization of the bone-imaging agent.

Aged↗
PubMed2001

[A case of chondrosarcoma originating from the rib and presenting as a posterior mediastinal tumor-like shadow].

A 66-year-old man was admitted to our hospital because of induction therapy of interferon for chronic hepatitis due to hepatitis C virus. On routine investigation, a mass-like lesion was detected at the level of the hilum of the left lung on the chest plain radiograph. On the thoracic CT, the heterogeneous tumor, including calcification, was 6 cm in diameter in the posterior mediastinal area, and the rib and vertebra had been damaged by it. The tumor extended to the descending aorta and the left main bronchus. A small nodule in the left S1 + 2 was noted on another slice section of this thoracic CT. The possible diagnoses, based on these radiological findings, for this tumor were primary lung cancer, posterior mediastinal tumor and malignant tumor originating from the chest wall. The diagnosis of chondrosarcoma was made based on the histological findings of the specimen obtained from the tumor. This tumor could not be resected in this case, because it had extensively damaged the rib and vertebra, and the nodular lesion in the left S1 + 2 was regarded as a metastasis from it. Although reports of chondrosarcoma originating from a rib and presenting as a posterior mediastinal tumor-like shadow are rare, chondrosarcoma should be considered as a possible diagnosis for such tumors-posterior mediastinal masses with characteristic findings such as a large tumor in contact with the chest wall, and containing calcification, with destruction of bone and dissection of the rib as shown in our CT findings.

Aged↗