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[Disseminated craniofacial chondrosarcoma].

Chondrosarcomas are malignant mesenchymal tumors of chondroid nature. Less than 5% of all chondrosarcomas are localized in the head and neck. The paper analyzes a case of extensive chondrosarcoma of the skill base, which involves the anterior and median cranial fossa, ethmoidal, sphenoidal, right maxillary sinuses, and intratemporal fossa. A basal approach that is a combination of two-flapped subfrontal and orbitozygomatous accesses was used to remove the tumor. In doing so, the tumor could be excised and extensive defects of the skull base could be effectively closed with the flaps of the periostium and musculus temporalis, yielding a good functional and cosmetic effect.

Adult↗

[Parapharyngeal chondrosarcoma and Ollier's disease: a case report and review of the literature].

OBJECTIVES: Ollier's disease is a constitutional pathology of unknown etiology. It is characterized by bone dysplasia generating numerous enchondromas. The malignant degeneration of this dysplasia is well known. The aim of this article is to study the diagnostic, therapeutic and prognostic characteristics of these lesions. MATERIAL AND METHODS: We report a case of parapharyngeal chondrosarcoma extended to the base of the skull in a patient with Ollier's disease. The treatment was a surgical removal by a cervicotransoral incision combined with a preauricular incision and with a mastoidectomy. It was therefore possible to control the skull base, the parapharyngeal space, the infratemporal fossa and the major neurovascular structures. The removal of the lesion was completed at the level of the clivus and sphenoid with optics (30 and 70 degrees ). We discuss this treatment and the follow up on the bases of literature data. RESULTS: The neoplastic degeneration of enchondromas is estimated between 25 to 50% of cases. The most frequent location is the pelvic bones. Chondrosarcomas are slow growing tumors and their metastatic potential is less significant as we note it in our case report. Their diagnostic is essentially based on histological criteria's and their treatment is surgical. CONCLUSION: Chondrosarcomas of the ENT area occurring with Ollier's disease is rare. Their prognostic is good if the surgical treatment is well done.

Adult↗

Isolation and characterization of proteoglycans from the swarm rat chondrosarcoma.

Proteoglycan monomer (D1) and aggregate (A1) preparations were isolated from 4 M guanidinium chloride extracts of the Swarm rat chondrosarcoma. When EDTA, 6-aminohexanoic acid, and benzamidine were present in the solutions, the D1 preparation contained a single component (SO = 23 S), and the A1 preparation contained 30% monomer (SO = 23 S) and 70 percent aggregate (SO = 111 S). In the absence of EDTA, 6-aminohexanoic acid, and benzamidine, the A1 preparations contained only small proteoglycan fragments, indicating that extensive enzymatic degradation had occurred. The composition of the proteoglycan monomer was different from that of proteoglycan monomer preparations from normal hyaline cartilages in that it did not contain keratan sulfate and chondroitin 6-sulfate; only chondroitin 4-sulfate was found. The A1 preparation from the chondrosarcoma contained only one link protein, which was like the smaller (molecular weight of 40,000) of the two link proteins present in A1 preparations from bovine nasal cartilage. When the A1 preparation from the chondrosarcoma was treated with chondroitinase ABC and trypsin and the digest was chromatographed on Sepharose 2B, a complex was isolated which contained the link protein and the segments of the protein core from the hyaluronic acid-binding region of the proteoglycan molecules.

Amino Acids↗

Mesenchymal chondrosarcoma: molecular characterization by a proteomic approach, with morphogenic and therapeutic implications.

This study characterizes 3 cases of mesenchymal chondrosarcoma (MC) utilizing a proteomic approach that allows for the detection, visual quantification, cellular compartmentalization, and assessment of the functional state of certain proteins that may promote tumor growth and/or oppose apoptosis. Immunohistochemical procedures were performed to detect the following protein antigens: CD99, interleukin (IL)-1alpha, IL-6, transforming growth factor (TGF)-alpha, conventional (c) protein kinase C (cPKC)-alpha, cPKC-betaII, phosphorylated (p)-PKC-alpha/betaII, c-kit (CD117), platelet-derived growth factor receptor (PDGFR)-alpha, PDGFR-beta, epidermal growth factor receptor (EGFR), human epidermal growth factor receptor (HER)-2/neu, cathepsin D, angiotensin-converting enzyme (ACE), angiotensin II type 1 (AT1) receptor, p21ras, the alpha subunit of farnesyl and geranylgeranyl transferase (FTalpha/GGTalpha), phospho (p)-c-Jun N-terminal kinase (p-JNK), p-p38 mitogen-activated protein kinase (MAPK), cyclin D1, c-Jun, Ki-67, bcl-2, TGF-beta1 latency-associated peptide (LAP), TGF-betaRII, and cyclooxygenase (COX)-2. Immunoreactivities were scored from 0 to 3+ positivity using bright-field microscopy. The results showed that malignant mesenchymal chondroblasts exhibit stronger expressions of CD99, IL-1alpha, cPKC-alpha, p-PKC-alpha/betaII, PDGFR-alpha, p-JNK, Ki-67, and bcl-2 antigens than their more mature-appearing chondrocytic counterparts in MC. In conclusion, molecular profiling of mesenchymal chondrosarcoma using a proteomic approach characterized the mesenchymal chondroblasts as possessing pathways that incorporate PKC-alpha and PDGFR-alpha signaling and anti-apoptotic bcl-2 expression. Specific therapies to target the mesenchymal chondroblasts in mesenchymal chondrosarcoma might include interferon-alpha, rapamycin, ciprofloxacin, and STI571.

Adult↗

Extraskeletal mesenchymal chondrosarcoma.

Extraskeletal mesenchymal chondrosarcoma is a rare malignancy. It is characteriged by a bimorphic histologic pattern, with a mesenchymal tissue mixed with malignant hyaline cartilage. It also has high incidence of local recurrence and distant metastasis. All cases have been reported fatal associated with this tumor in spite of complete surgical excision. A 35-year-old female presented with a palpable mass about 5 x 3 x 2.5 cm3 in size over her left forearm and was reported to be an extraskeletal mesenchymal chondrosarcoma. Marginal resection followed by regional radiotherapy was done. No local recurrence or distant metastasis was found two years after surgery. Thorough physical examination, series radiographic studies, and multiple sites of tumor biopsies before radical resection of the tumor may decrease the misdiagnostic rate for extraskeletal mesenchymal chondrosarcoma. Appropriate tumor treatment, close patient follow-up and timely treatment for local recurrence or distant metastasis may increase the survival rate.

Adult↗

Squamocellular carcinoma and chondrosarcoma: a true pulmonary carcinosarcoma. Report of a case.

BACKGROUND: Carcinosarcoma is one of the less common tumors of the lung and is composed of a mixture of malignant epithelial and mesenchymal elements of the type ordinarily seen in malignancies of adults. The carcinomatous component is mostly epidermoid and sometimes adenomatoid or undifferentiated. The mesenchymal part is mostly a spindle cell sarcoma and sometimes a polymorphocellular sarcoma. Differentiation as osteosarcoma and chondrosarcoma is rare. CASE REPORT: This report describes the case of a patient with carcinosarcoma of the lung composed of epidermoid carcinoma and chondrosarcoma. A left hilar mass was incidentally diagnosed. The patient was submitted to surgical exploration and a left lower lobectomy with dissection of local lymph nodes was performed. At microscopy the tumor was composed of both epithelial and stromal malignant component. The epithelial component consisted of poorly-differentiated squamous cell carcinoma and the stromal component consisted of chondrosarcoma. He remains well 30 months later. CONCLUSION: The prognosis of patients with carcinosarcoma is not always unfavourable. Potentially curative surgical resections should always be attempted. Pathologists should be aware of a wrong diagnosis of undifferentiated small cell lung carcinoma which eliminates the patient from surgery.

Aged↗

Spinal meningeal mesenchymal chondrosarcoma. Report of a new case and review of the literature.

A case of T12-L2 intraspinal extradural meningeal mesenchymal chondrosarcoma is described in a patient presenting with a clinical history of low-back pain and weakness of the lower limbs. Magnetic resonance T1-weighted Gadolinium enhanced imaging, showed an intraspinal extradural mass, extending from T12 to L2, located anterolaterally into the spinal canal and pushing posteriorly the conus medullaris. The mass was completely removed and postoperative histological diagnosis was of mesenchymal chondrosarcoma. The patient was completely symptoms-free after surgery. Intraspinal meningeal mesenchymal chondrosarcoma and treatment options are reviewed.

Adult↗

Chondrosarcoma of the larynx: a histo-radiologic analysis.

Chondrosarcoma of the larynx is a rare malignant cartilaginous neoplasm. To date, the serial sectioned laryngeal specimens of this disease have not been correlated with the pre-operative computed tomographic findings. A retrospective analysis of 833 serially sectioned laryngeal specimens found chondrosarcoma in six cases (0.72%), of which four cases had a pre-surgical computed tomography (CT). Radiologic axial sections were compared with microscopic sections in the same plane. Whole organ sections showed the tumor to have an expansile growth pattern, showing little impulse to infiltrate adjacent cartilage. CT scanning successfully predicted the diagnosis of a cartilage neoplasm prior to surgery, and accurately reflected the site and extent of laryngeal involvement. We conclude that CT is a reliable method of evaluating chondrosarcoma of the larynx, thereby predicting the feasibility of partial laryngeal resection.

Aged↗

Primary chondrosarcoma of the breast: a case report.

Mammary sarcomas are uncommon tumors. Of these pure chondrosarcoma without any other area of epithelial or mesenchymal differentiation feature as even rarer. This excludes tumors like malignant cystosarcoma phyllodes and metaplastic carcinoma where malignant cartilaginous areas may be present. Further primary chondrosarcoma arises from breast stroma and not from underlying bone or cartilage. Only five cases of pure and primary chondrosarcoma have been reported so far. The sixth case is reported here.

Breast Neoplasms↗

An unusual presentation of an anterior chest wall chondrosarcoma.

BACKGROUND: Occasionally, anterior chest wall chondrosarcoma presents in an unusual manner. AIM: We report a case of an anterior chest wall chondrosarcoma which was misdiagnosed as a breast tumour. METHOD: The hospital notes of the patient and the literature on the disease were reviewed. RESULT: A 28-year old female Nigerian presented with a progressive enlargement of the right breast. Chest radiograph revealed an opacity in the right hemi-thorax. At surgery, the mass was found to be a chondrosarcoma arising from the anterior chest wall with the breast mass compressed anteriorly. This was confirmed by histopathological examination. She was followed up as outpatient but declined to further treatment. CONCLUSION: An anterior chest wall tumour of the magnitude described, should always be investigated and evaluated adequately prior to surgery in order to avoid a diagnostic pitfall.

Adult↗

Enchondroma and chondrosarcoma of bone: clinical, radiologic, and histologic differentiation.

Intraosseous cartilage tumors are commonly encountered by orthopaedic surgeons during evaluation of a patient for undetermined pain; however, the possibility of missing a malignancy during examination is cause for concern. Surgery for enchondromas is not generally necessary but when clinical and radiographic features suggest a potential chondrosarcoma, prompt referral is necessary. Chondrosarcoma almost always occurs in adults. Patients usually present with pain and have characteristic findings on staging studies. Lucencies developing within the calcification, periosteal reactions, and a soft-tissue mass all are characteristic features of chondrosarcoma. Biopsies are not typically indicated because of the histologic similarity of benign and malignant cartilage lesions.

Biopsy↗

Chondrosarcoma of the hand: is a wide surgical resection necessary?

Chondrosarcomas of the hand are rare and generally treated with surgical resection. Thirteen patients with Grade 1 chondrosarcoma of the small bones of the hand were followed up for a mean of 99.8 months (range, 26-293 months). In eight patients (Group 1) curettage and reconstruction with cancellous bone was done and in five patients (Group 2) a wide resection was done. No patient experienced relapse in Group 2. In Group 1 one patient had a local relapse 18 months after intralesional resection. Using the Musculoskeletal Tumor Society score for evaluation, the clinical results showed an average of 98% and 95% of the normal function in Groups 1 and 2, respectively. None of the patients had evidence of systemic spread of the disease. With a relapse rate of 12.5% and no distant metastases after curettage, intralesional resection is the preferred method of treatment in Grade 1 chondrosarcoma of the hand, allowing the patient to avoid amputation and major loss of function.

Adolescent↗

Radiation-induced chondrosarcoma of the maxilla 7-year after combined chemoradiation for tonsillar lymphoma.

Radiation-induced sarcoma is a rare complication of radiation therapy. We report a case of radiation-induced chondrosarcoma of the maxilla. An 80-year-old Persian woman developed radiation-induced chondrosarcoma of the left maxilla 7 years after combined chemotherapy and external beam radiation therapy for the Ann Arbor stage IE malignant lymphoma of the right tonsil. She underwent suboptimal tumour resection and died due to extensive locoregional disease 8 months later. An English language literature search of Medline using the terms chondrosarcoma, radiation-induced sarcoma and maxilla revealed only one earlier reported case. We describe the clinical and pathological features of this case and review the literature on radiation-induced sarcomas.

Aged↗

Cytogenetics of swarm rat chondrosarcoma.

The Swarm rat chondrosarcoma is a tumor tissue line derived from a tumor that arose spontaneously in a Sprague-Dawley rat. The original tissue has given rise to several tissue lines and cell lines that have been prepared in different laboratories. It has been observed that these lines differed in their growth rates and biochemical characteristics. We have characterized our Swarm rat chondrosarcoma tissue and cell lines currently in use in terms of their cytogenetic profiles and their tumorigenic properties in vivo. We found a wide variety of chromosomal abnormalities among cell lines, including translocations, deletions and polyploidy. There were also significant differences in their growth properties in vivo, giving rise to tumors of a few milligrams in the case of Ng cells, to 35 grams in the tissue line JWS. The cytogenetic complexity of the Swarm rat chondrosarcoma between and among different lines makes it very suitable to address questions about the changes that occur as a result of karyotypic abnormalities and to provide links between cytogenetic abnormalities and the dynamic oncogenic machinery.

Animals↗

[Chondrosarcoma of the chest wall].

We report 2 patients with chondrosarcoma of the chest wall. They were a 67-year-old woman (case 1) with an anterior chest wall tumor and a 68-year-old woman (case 2) with a painful rib tumor. Their computed tomography (CT) both revealed calcified tumors. Case 1 underwent a wide resection by partial sternectomy, with free surgical margins. Case 2 underwent tumor resection with the posterior part of the 3rd rib, with positive surgical margin in the vertebral site, and received adjuvant radiotherapy. Both patients were pathologically diagnosed as having grade II chondrosarcoma. In their postoperative courses, they are free from recurrence. Wide resection is likely to be the key to successful management of chest wall chondrosarcoma.

Aged↗

High hydrostatic pressure-induced cell death in human chondrocytes and chondrosarcoma cells.

BACKGROUND: In orthopedic surgery, sterilization of bone used for reconstruction of osteoarticular defects caused by malignant tumors is carried out in different ways. At present, to devitalize tumor-bearing osteochondral segments, extracorporal irradiation or autoclaving is mainly used, although both methods have substantial disadvantages, e.g. loss of biomechanical and/or biological integrity of the bone and destabilization of the articular surface. In this regard, high hydrostatic pressure (HHP) treatment of bone is a new, advancing technology, now being used in preclinical testing to inactivate tumor cells. To find out if this technique is also suited for extracorporal inactivation of chondrocytes and chondral tumor cells, the effect of HHP on cell viability and morphology of human chondrocytes / chondrosarcoma cells was investigated in the present study. MATERIALS AND METHODS: SW1353 chondrosarcoma cells and chondrocytes were subjected to HHP in the range of 50 to 350 MPa (10 min, 37 degrees C) and, subsequently, cell viability and cell morphology assessed. RESULTS: After exposure at 350 MPa, all HHP-treated chondral cells showed explicit morphological changes, evident by membrane ruffling and bleb formation; chondrosarcoma cells treated this way were irreversibly damaged and not alive. CONCLUSION: We anticipate that, in orthopedic surgery, HHP eventually can serve as a novel, promising technical approach for cell inactivation (including tumor cells) and allow subsequent reimplantation of the osteoarticular autograft.

Aged↗