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PubMed1999

Mesenchymal extraskeletal chondrosarcoma of the orbit. Report of a case and review of the literature.

BACKGROUND: Extraskeletal mesenchymal chondrosarcoma (MCS) is relatively uncommon. Orbital location is extremely rare: only 16 cases have been reported until now. We report a case of extraskeletal mesenchymal chondrosarcoma in a 27-year-old man and review the literature on its manifestations and management. CASE REPORT: This patient had a 2-year history of progressive proptosis of the right eye. Skull X-ray and CT scan showed intraorbital calcification and a large lesion in the upper right orbit. He was operated three times because of recurrence of the tumor. The last recurrence was observed to have extension to the intracranial region, detected on MRI and CT scan. This secondary extension of the tumor to the intracranial region has not been previously reported. Immunohistochemical analysis for S-100 protein showed focal positivity. CONCLUSION: Mesenchymal chondrosarcoma of the orbit is rare, and secondary extension to the intracranial region has not previously been reported.

Adult↗
PubMed2003

Penile metastasis of chondrosarcoma of the jaw.

Metastasis to the penis is an unusual event. Bladder and prostate tumors are the main sources of penile metastasis. Other sites include the rectosigmoid, kidney, and, less frequently, the pancreas, liver, nasopharynx, and lung. Other sources include malignant melanoma and Burkitt's lymphoma. The differential diagnosis includes idiopathic priapism, venereal or infectious disease, tuberculosis, Peyronie's disease, and primary penile tumor. Chondrosarcoma of the jaw is responsible for 10% of all chondrosarcomas that originate with craniofacial bones. Its behavior is usually characterized by local aggression; however, distant metastasis is uncommon. We report a case of chondrosarcoma of the jaw with penile metastasis. This is the first case described in published medical reports.

Adult↗
PubMed2003

Chondrosarcoma of small bones of the hand.

A clinicopathological review of 23 patients (mean age, 67 years; range, 42-85 years) with chondrosarcoma of the bones of the hand was done. The mean follow up was 8.5 years. Eleven patients presented with a progressive painless swelling, 26% having had symptoms for over 10 years. The proximal phalanx was the commonest site. Initial clinical misdiagnosis as ganglion, bursa, gout, rheumatoid arthritis and a cyst occurred in five patients. Radiologically most lesions showed bone expansion, cortical destruction and soft-tissue extension. The majority was of high histologic grade (Evan's grade 2 & 3) with extensive myxoid areas. Five out of eight patients who were originally treated by curettage or excision had local recurrences compared to none treated by ray resection or amputation of phalanx (P=0.002). None had metastases. The low risk of metastases despite the high histologic grade indicates that chondrosarcomas of the hand behave differently from chondrosarcomas elsewhere.

Adult↗
PubMed1987

Rhabdomyosarcomatous component in dedifferentiated chondrosarcoma.

The case of a 57-year-old male is described with an extended bone tumor in the proximal left femur. The tumor is histologically classified as dedifferentiated chondrosarcoma. Immunohistologic examination revealed a strong positive reaction for desmin mainly in the tumor giant cells in the dedifferentiated areas, whereas chondroblasts in the low grade chondrosarcoma areas reacted negatively with desmin. In accordance with some cross striation in polymorphic tumor cells this points to a rhabdomyosarcomatous component in the dedifferentiated portions of the tumors. Thus, in addition to fibrosarcomatous, osteosarcomatous and malignant fibrous histiocytoma-like areas, our case presents another sarcomatous constituent in dedifferentiated chondrosarcoma.

Bone Neoplasms↗
PubMed1998

Temporal lobe (TL) damage following surgery and high-dose photon and proton irradiation in 96 patients affected by chordomas and chondrosarcomas of the base of the skull.

PURPOSE: To determine the temporal lobe (TL) damage rate in 96 patients treated with high-dose proton and photon irradiation for chordomas and chondrosarcomas of the base of the skull. METHODS AND MATERIALS: The records of 96 consecutive patients treated at Massachusetts General Hospital (MGH) and Harvard Cyclotron Laboratory (HCL) between June 1984 and 1993, for chordomas and chondrosarcomas of the base of the skull were reviewed. All the patients had undergone some degree of resection of the tumor prior to radiation therapy. Seventy-five patients were classified as "primary tumors" and 21 as recurrent or regrowing tumors after one or more surgical procedures. All the patients were randomized to receive 66.6 or 72 cobalt Gray equivalent (CGE) on a prospective dose-searching study by proton and photon irradiation (Radiation Therapy Oncology Group #85-26) with conventional fractionation (1.8 CGE/day, 5 fractions/week). All treatments were planned using the three-dimensional (3D) planning system developed at the Massachusetts General Hospital, and the dose was delivered using opposed lateral fields for the photon component and a noncoplanar isocentric technique for the proton component. Clinical symptoms of TL damage were classified into 4 grades. Computerized tomography (CT) and magnetic resonance imaging (MRI) scans were evaluated for white matter changes. Abnormalities associated with persistent or recurrent tumor were distinguished from radiation-induced changes. TLs were delineated on the original scans of the 10 patients with damage and those of a group of 33 patients with no clinical or MRI evidence of injury. Dose distributions were calculated and dose-volume histograms were obtained for these patients. RESULTS: Of the patients, 10 developed TL damage, with bilateral injury in 2 and unilateral injury in 8. The cumulative TL damage incidence at 2 and 5 years was 7.6 and 13.2%, respectively. The MRI areas suggestive of TL damage were always separated from the tumor bed. Symptoms were severe to moderate in 8 patients. Several baseline factors, tumor- or host-related, were analyzed to evaluate their predictivity for TL damage: age, gender, tumor site, histology, type of presentation, type and number of surgical procedures, primary tumor volume, prescribed dose, normal tissue involvement, and volume of TL receiving doses ranging between 10 and 50 CGE or more. Only gender, in a univariate analysis (log rank) was a significant predictor of damage (0.0155), with male patients being at significantly higher risk of TL injury. In a stepwise Cox regression that included gender as a variable, no other baseline variable improved the prediction of damage. CONCLUSIONS: The 2- and 5-year cumulative TL damage rates were 7.6 and 13.2%, respectively. Despite the different TL damage rates related to age, tumor volume, number of surgical procedures prior to radiation therapy, and prescribed doses to the tumor, only gender was a significant predictor of damage (p = 0.0155) using a univariate (log rank) test. Chordomas and chondrosarcomas of the base of the skull may represent an interesting model to evaluate the TL damage rates because of their extradural origin, displacing the white matter instead of infiltrating it as gliomas do, because of their longer local recurrence-free survival other than gliomas and other brain tumors and because of the high doses of irradiation delivered to the target volume to obtain local control.

Adolescent↗
PubMed2002

Cancer/testis antigen CSAGE is concurrently expressed with MAGE in chondrosarcoma.

Differential display-polymerase chain reaction was used to compare gene expression between human chondrosarcoma cell lines and normal cartilage. A new gene, CSAGE, has been cloned and belongs to a gene family that includes the taxol resistance associated gene (TRAG)-3. CSAGE, like TRAG-3, does not confer resistance to taxol when transfected in vitro. Both genes have alternatively spliced variants. CSAGE and TRAG-3 are expressed in chondrosarcoma, melanoma, and cartilage and testis, but not in other normal tissues. TRAG-3 has been reported to be a cancer/testis antigen. Our results suggest that CSAGE belongs to the growing list of cancer/testis antigens as well. In all of the CSAGE positive samples, the melanoma antigen gene family was also expressed. This is the first report on the expression of cancer/testis antigens in chondrosarcoma.

Alternative Splicing↗
PubMed2002

Low-grade chondrosarcomas: a difficult target for radionuclide imaging. Case report and review of the literature.

Bone scan with Tc-99m (technetium) diphosphonate is sensitive, but non-specific for musculoskeletal tumors. Tl-201 (thallium), Tc-99m-sestamibi, Tc-99m-tetrofosmin, and F-18-fluorodeoxyglucose (F-18-FDG) can visualize tumors more specifically and are therefore useful in orthopedic oncology. However, cartilaginous tumors are characterized by histological and biological features, which potentially impair specific radionuclide imaging. A case of a patient with a low-grade primary chondrosarcoma of the femur and a false negative Tl-201 scan is presented. Tc-99m-based tumor-localizing compounds (sestamibi, tetrofosmin), as well as metabolic and receptor-imaging radiopharmaceuticals have also been reported to fail in low-grade chondrosarcomas imaging. Low cellularity, mitochondrial specialization and the presence of an efflux membrane pump may contribute to poor imaging. A negative Tl-201 or Tc-99m-sestamibi scan should be interpreted with caution, when the possibility of a chondrosarcoma is not negligible.

Aged↗
PubMed2002

Tenascin-C expression and distribution in cultured human chondrocytes and chondrosarcoma cells.

Tenascin-C (TNC) is an oligomeric glycoprotein of the extracellular matrix with several distinct isoforms variably expressed during embryogenesis, tumorogenesis, angiogenesis and wound healing. In the normal human adult, TNC is found in large concentrations in articular cartilage, suggesting tissue-specific function. The purpose of this study was to determine the specific in vitro TNC splicing patterns of articular chondrocytes and a human chondrosarcoma cell line. Cells were cultured in a three-dimensional bead system and TNC splice variant expression and distribution were examined with the use of Western blotting techniques, semi-quantitative reverse-transcription polymerase chain reaction and immunohistochemistry. At both the transcriptional and post-translational levels, the chondrocytes were found to express significantly higher levels of the smaller 220 kDa isoform (P < 0.01), which was predominantly incorporated into the matrix. The splicing pattern of the malignant cells was characterized by a higher proportion of the larger 320 kDa isoform which was extruded into the media. In vivo studies are necessary to verify the expression of the large TNC isoform in chondrosarcoma and the production and integration of the smaller isoform in normal chondroid matrix. In addition, elucidation of the biologic functions of the two major TNC isoforms may lead to the development of novel diagnostic and therapeutic approaches to chondrosarcoma.

Blotting, Western↗
PubMed2003

The C-terminal domain of focal adhesion kinase reduces the tumor cell invasiveness in chondrosarcoma cell lines.

Human chondrosarcoma is a malignancy that has no effective systemic therapy, making the interruption of the metastatic cascade critical to enhance patient survival. The processes of local invasion and metastases share similar mechanisms at a cellular level. Focal adhesion kinase (FAK) has been implicated in local invasion of malignant tumor cells. In the current manuscript we examine the effect of FAK inhibition on cell attachment to extracellular matrix (ECM) and in vitro invasion. Bovine articular chondrocytes and two human chondrosarcoma cell lines were utilized to examine FAK activity in tumor cell invasiveness. Endogenous FAK activity was inhibited by adenoviral transfection with the C-terminal domain of FAK. This inhibition resulted from decreased FAK phosphorylation, while FAK expression remained unchanged. Inhibition of FAK phosphorylation and hence its activity lead to decreased cell adhesion to Type II collagen and decreased cell invasiveness. These effects were not due to changes in integrin expression, indicating that the inhibition was the result of disruption of outside: in signaling. There are three important aspects to these results. The first is that interruption of transmembrane signaling can affect cell attachment. The second is that in chondrosarcoma, cell differentiation correlates with FAK expression and metastatic potential. Thirdly, that cell invasiveness correlates with FAK activity and implies a mechanistic role for this molecular complex in local invasion and metastasis.

Animals↗
PubMed1998

Chondrosarcoma of the aorta: a rare source of bowel and lower extremity emboli.

Malignant aortic tumors occur infrequently. At least 70 cases of primary aortic malignancies have been reported in the literature. Within this group, chondrosarcoma is exceedingly rare, with only 1 case having been reported. An aortic chondrosarcoma developed in our patient and embolized to the small bowel and lower extremities. Although initially thought to arise from the abdominal aorta, this tumor, in fact, originated from the thoracic aorta. This case illustrates the need for complete aortic imaging when unexplained recurrent embolization occurs. In general, the survival rates with chondrosarcoma are diminished, but this patient survived 69 months after he was initially seen.

Aged↗
PubMedDate not supplied

Extraskeletal mesenchymal chondrosarcoma: case report.

This is a case report of an extraskeletal mesenchymal chondrosarcoma (ESMC) that originally occurred in the retroperitoneum of a 24-year-old female and later metastasized to the left proximal humerus. Mesenchymal chondrosarcomas are very rare in comparison to conventional chondrosarcomas and even more so when arising from an extraskeletal location. In this report, we discuss the major characteristics of ESMC and offer a review of the current knowledge regarding this rare disease entity.

Adult↗
PubMed2000

Prognostic significance of nuclear DNA content in chondrosarcoma.

Chondrosarcoma is the second most frequent primary malignant tumor of bone. Many of these tumors represent histopathologic borderline cases. In this study, DNA ploidy status, 2c deviation index (2cDI), and DNA malignancy grade (DNA-MG; based on the variation of nuclear DNA content of tumor cells around the normal DNA [2c] peak) were examined for their diagnostic and prognostic value in comparison with conventional histopathologic grading. Twenty-two paraffin-embedded samples were available for histopathologic investigation and for quantitative cytophotometric DNA determination of Feulgen-stained nuclei. Clinicopathologic parameters and prognosis were analyzed over a maximum follow-up period of 252 months. Nineteen of 22 (86%) chondrosarcomas showed aneuploid DNA content. 2cDI (r =.58, P <.01) and DNA-MG (r =.58; P <.01) correlate with the histopathologic grading. Significant correlation between the 2cDI (P <.01) and DNA-MG (P <.025) and the overall survival was found. Ploidy did not influence the overall survival rate. In metastasis-free patients, the 2cDI and DNA-MG gave better prognostic information than conventional histopathologic grading. When patients developed metastasis, however, histopathologic grading was the prognostic parameter of choice. Cytometric DNA measurement provide additional objective information regarding the diagnosis and prognosis of chondrosarcomas, even more than that obtained by conventional histopathologic grading, and may be helpful in planning the treatment.

Adult↗
PubMed2005

Emerging pathways in the development of chondrosarcoma of bone and implications for targeted treatment.

Chondrosarcoma is a malignant cartilage-forming tumour of bone, of which distinct clinicopathological subtypes are known. Conventional chondrosarcoma is notorious for its locally aggressive behaviour as well as for its resistance to chemotherapy and radiotherapy; so far surgery is the only effective therapeutic option. During the past 10 years, substantial new insights have been gained about molecular cell biology, molecular cytogenetics, and immunopathology, leading to better understanding of chondrosarcoma development at the molecular level, which will ultimately lead to better clinical understanding and possibly to the development of targeted treatment.

Bone Neoplasms↗
PubMed2004

Extraskeletal intracranial mesenchymal chondrosarcoma: case report and literature review.

We present the case of a 13-year-old girl with a huge intracranial mesenchymal chondrosarcoma. She had suffered from severe headache, diplopia, intermittent nausea and vomiting for 1 month. Neurologic examination revealed bilateral blurred optic disc margins and abducens paresis. Magnetic resonance imaging demonstrated a giant, heterogeneous, intensely enhancing mass of 7 x 8 x 6 cm, occupying the bilateral frontal and left high parietal regions and based on the anterior cerebral falx. A presumptive diagnosis of aggressive meningioma was made. The patient underwent bicoronal craniotomy and gross total resection of the tumor. Pathologic examination revealed an extraskeletal mesenchymal chondrosarcoma. She was under regular follow-up and remained free of recurrence after surgery. In addition to the current case, we review previously reported cases of extraskeletal intracranial mesenchymal chondrosarcoma and discuss treatment strategies and outcomes.

Adolescent↗
PubMed1985

Metachranous chondrosarcoma and malignant melanoma of the nose.

A case is presented of chondrosarcoma of the right lateral nasal wall treated surgically in which a malignant melanoma arose eight months later. Review of the literature has revealed no other report of a second primary within the nose or paranasal sinuses following a chondrosarcoma. Neither chondrosarcoma nor malignant melanoma of the nose is radiosensitive and the treatment for these tumours is wide excision with confirmation of clear margins of resection by frozen section.

Aged↗
PubMed1995

Chondrosarcoma of the larynx.

A case of a low grade chondrosarcoma of the cricoid cartilage which had been diagnosed initially as a chondroma is presented. The tumour recurred twice after limited surgical resections. Total laryngectomy was inevitable due to near total involvement of the cricoid cartilage and subsequent histological examination revealed a low grade chondrosarcoma. We have discussed in brief, the diagnosis and treatment of chondrosarcomas of the larynx and support the view of conservative surgical management for low grade tumours as they are slow growing and metastases are infrequent. A total laryngectomy may be reserved for salvage or primarily when more than half of the cricoid cartilage needs to be resected. Histological grading reveals the biological behaviour of the tumour and CT scans help in planning the surgery. A regular follow-up is necessary for early detection of recurrences and metastases.

Chondrosarcoma↗
PubMed1999

Myxoid chondrosarcoma of the external auditory meatus.

Extraskeletal myxoid chondrosarcoma presenting in the head and neck is extremely rare. Histological diagnosis is difficult and requires close co-operation between clinician, radiologist and pathologist. The tumour has a good prognosis in comparison to myxoid chondrosarcoma of the bone but surgical resection may be difficult due to its gelatinous nature. We present a case of extraskeletal myxoid chondrosarcoma originating in the external auditory meatus of a 42-year-old man. This is the first report of this tumour in this site.

Adult↗
PubMed2004

Intracranial chondrosarcoma: a case report and review of the literature.

OBJECTIVE AND IMPORTANCE: Chondrosarcomas rarely occur intracranially away from the skull base. We present a case of a supratentorial dural-based, low-grade classic chondrosarcoma and emphasize salient imaging findings that assist in its diagnosis. CLINICAL PRESENTATION: A 23-year-old female presented with new onset seizures. Multiple radiographic imaging studies identified a large left frontal mass with radiological findings that suggested the correct diagnosis. INTERVENTION: The patient underwent a left frontal craniotomy and gross total resection of the lesion and associated dura. CONCLUSION: Intracranial chondrosarcomas away from the skull based are extremely rare malignancies. As with other extra-axial masses, they often grow to a relatively large size before generating symptoms. Careful analysis of CT, MR, and angiographic imaging characteristics should suggest the correct diagnosis.

Adult↗