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Mesenchymal chondrosarcoma of the rib: report of a case.

Mesenchymal chondrosarcoma is a rare malignant cartilaginous tumor arising within the bone or soft tissue. An 18-year-old woman presented with a tumor on her left fourth rib. We performed a wide resection of the tumor and administered three cycles of postoperative adjuvant chemotherapy. Three years after the operation, the patient is alive without any evidence of either local recurrence or distant metastases. The findings of this case may thus support the usefulness of a radical resection and adjuvant chemotherapy for mesenchymal chondrosarcoma.

Adolescent↗

Chondrosarcoma in children subsequent to other malignant tumours in different locations.

During recent years the combination of surgery, radiotherapy, and multiple chemotherapy in the treatment of patients with cancer has resulted in increased survival periods and, in some cases, complete cures. In children, however, the subsequent development of other and apparently unrelated malignancies has been observed 20 times more commonly than in a control group. This paper reports the occurrence of chondrosarcoma of the femur in two children who had been treated successfully for malignant tumours of soft tissue. One of these tumours was a malignant fibrous histiocytoma in the muscles of the forearm; the other was a rhabdomyosarcoma of the cheek. The intervals between termination of treatment of these lesions and recognition of the chondrosarcoma were five years and 10 years, respectively. In addition to surgical and radiotherapeutic measures, both children had received high doses of chemotherapy.

Adolescent↗

Chondrosarcoma of the spine: an unusual radiographic presentation.

Chondrosarcoma is a primary malignant tumor arising rarely in the axial skeleton. It usually presents as a destructive lesion with diffuse mottled calcification. We report three patients with chondrosarcoma of the spine. Each patient was evaluated by conventional radiographic technique, computed tomography, and myelography. All of them show radiographically a purely destructive process of the body and in one case the body and lamina. This pattern remains to be documented in the radiologic literature.

Adult↗

The prognostic significance of different histomorphologic features in chondrosarcoma.

Cellularity, mitotic rate and nuclear size were determined objectively in 47 chondrosarcomas. Grading was also performed, according to the systems of O'Neal and Ackerman and of Evans. The survival rate and incidence of metastases 10 years after diagnosis were calculated. The prognostic significance of the objectively determined cytological parameters was investigated. The accuracy of determination of these cytological parameters by grading (Evans) was also analyzed. Nuclear size seemed to be the best prognostic parameter, while cellularity appeared to be the strongest determinant for grading. Gross differences of nuclear size can be assessed visually, while more subtle, although prognostically significant, differences can only be determined by objective means. The results of this study indicate that the mere determination of nuclear size by objective means may give significant prognostic information, which is equal to, or even better than, conventional grading of chondrosarcoma.

Adolescent↗

Case report 829: Intramuscular myxoid chondrosarcoma.

A 61-year-old man presented with a 6-month history of a painless mass in the right buttock. Although the MR appearance of myxoid neoplasms is variable, a myxoid neoplasm was suspected on the basis of the MR study. Percutaneous needle biopsy results yielded a diagnosis of intramuscular myxoma. Myxoid chondrosarcoma, however, was diagnosed at the time of excisional biopsy. One should be wary of a diagnosis of intramuscular myxoma based solely on the results of percutaneous needle biopsy. In addition, myxoid chondrosarcoma of the soft tissues can have a variable appearance on T1-weighted imaging.

Buttocks↗

Intraspinal mesenchymal chondrosarcoma in a three-year-old boy.

A case is presented of a 3-year-old boy with a mesenchymal chondrosarcoma extending from the 1st to the 5th lumbar vertebra. This is the youngest case of a mesenchymal chondrosarcoma located outside the skeleton or in the C.N.S. After assumed total excision with subsequent radiotherapy and chemotherapy, local tumor recurrence and (later) systemic metastases were detected. Standard therapy should include radical excision because of the high incidence of local recurrence and subsequent radiotherapy because of the expected high incidence of tumor cells in the CSF. The value of chemotherapy cannot be assessed, as it has been applied in only one other case found in the literature.

Cauda Equina↗

Effects of certain antiarthritic agents on the synthesis of type II collagen and glycosaminoglycans in rat chondrosarcoma cultures.

Cartilage destruction is a characteristic feature of osteoarthritis. Treatment with certain nonsteroidal anti-inflammatory drugs could exacerbate cartilage destruction by impairing the synthesis of cartilage matrix proteins, type II collagen and proteoglycan. In order to monitor the changes occurring in cartilage collagen synthesis, we developed a type II collagen specific ELISA. The effects of antiarthritic agents on type II collagen and glycosaminoglycan synthesis were examined in rat chondrosarcoma cultures. Drugs were added to the monolayer cultures and 4 days later the total type II collagen, as determined by the type II collagen ELISA, and glycosaminoglycan content, as measured by dimethyl-methylene blue dye binding assay, was measured. All drugs except tiaprofenic acid decreased type II collagen synthesis by at least 40% at 100 micrograms/ml. Tiaprofenic acid at 1 microgram/ml increased type II collagen content by 54% of the controls. Glycosaminoglycan synthesis was decreased by acetylsalicylic acid, diclofenac and tiaprofenac acid, at 50 micrograms/ml or above. Indomethacin, naproxen and dexamethasone had no effect. Interestingly, tenidap stimulated the glycosaminoglycan synthesis by 32% at 100 micrograms/ml. We show that the combination of chondrosarcoma cultures, type II collagen specific ELISA and dimethylmethylene blue dye binding assay serves as a useful model for screening the effects of agents capable of modulating type II collagen and glycosaminoglycan synthesis.

Analysis of Variance↗

Chondrosarcomas of the head and neck.

Chondrosarcomas of the head and neck are uncommon. Over a 35-year period (1950-1985) at our institution, 557 patients had an established diagnosis of chondrosarcoma. In 28 (5%) of these patients, the tumor was located in the head and neck region. A retrospective analysis was performed in an attempt to accurately define clinical characteristics, management, and outcome in these 28 patients. The patient population consisted of 15 males and 13 females whose ages ranged from 10 to 72 years, with a median of 38 years. Ten (36%) patients were untreated and 18 (64%) previously treated patients were referred for further management. The primary sites were maxilla (11), cervical vertebrae (7), mandible (3), skull (2), sphenoid and ethmoid sinuses (2), frontal sinus (1), nasal septum (1), and orbit (1). Lesions arising in the larynx were excluded from this study. The most common presenting symptom was a painless mass. Surgery was the definitive treatment in all patients. However, adjuvant radiation therapy or chemotherapy was utilized for residual disease in almost half of the cases (13 patients). Follow-up ranged from 5 to 35 years. Twelve (43%) of the 28 patients were known to be alive and free of disease more than 5 years after surgery. The most common cause of death was uncontrollable local disease (88%). However, only 3 of the 10 previously untreated patients developed local recurrence. For all patients, the median interval from time of first recurrence until death was 25 months (range 6 to 70 months). Positive margins in 8 of 12 patients resulted in local recurrence and eventual death.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Soft tissue chondrosarcoma of the back--a case report.

A case of extraosseous chondrosarcoma arising from the soft tissues of the back is presented. The tumour was completely excised with a wide margin of normal tissues. Extraosseous chondrosarcoma arising from the soft tissues of the back has apparently never been reported.

Back↗

Primary cardiac chondrosarcoma with large cell pulmonary carcinoma.

A 55-year-old man was transferred to our hospital for removal of cardiac and pulmonary tumors. Transesophageal echocardiography demonstrated a large echogenic mass in the left ventricle. The mass was attached to the posterior wall of the left ventricle and the mitral valve. Chest computed tomography showed a solitary, well-defind nodular lesion in the right upper lung. We performed concomitant resection of cardiac and pulmonary tumors through a midline sternotomy. The pathological diagnosis was cardiac chondrosarcoma with pulmonary large cell carcinoma. Postoperatively pelvic computed tomography, bone and gallium scintigrams did not identify any other active lesion, hence the cardiac tumor was considered to be of cardiac origin. He is alive 20 months after the operation and findings from the cardiac and pulmonary examination are unremarkable. Primary cardiac chondrosarcoma is extremely rare, and to our knowledge, only 13 cases have been recorded. We summarize herein these 14 cases.

Carcinoma, Large Cell↗

[Skull base chondrosarcoma. An interdisciplinary challenge].

BACKGROUND: Chondrosarcoma is a rare differential diagnosis of malignant tumours of the skull base. The prognosis was rated as unfavourable in articles for many years. It has, however, improved considerably in recent years. The objective of this study was to evaluate and current, new optimised treatment strategies. PATIENTS AND METHODS: We retrospectively analysed the case histories and course of four patients whom we treated for chondrosarcoma of the skull base over the past 5 years at the Freiburg Skull Base Centre. RESULTS: Because of initially mild symptoms, the patients first came for examination at an advanced stage of the tumour. All patients underwent surgery, whereby an R0-resection was barely or only questionably present. Three patients underwent radiation therapy postoperatively. All patients are currently tumour free. CONCLUSIONS: Surgical treatment with curative intent is basically the therapy of choice. Due to the usually large size of the tumour and its close relationship to relevant structures, complete resection is, however, not always possible despite advances in surgical procedures. Taking the possibility of modern adjuvant radiotherapeutic procedures into account, an incomplete, function-preserving resection is preferred to a radical and mutilating resection.

Adult↗

[Myxoma and myxoid chondrosarcoma of the nasal septum: two case reports].

Myxoid neoplasms are histopathologically divided into true myxomas and other tumors of myxomatous character. In the region of the nasal septum, this tumor entity is extremely rare. In the following two case reports, the unusual findings of a myxoma and a myxoid chondrosarcoma of the nasal septum are presented and discussed. At first visit, both patients reported a slowly progressing, bilateral nasal obstruction. In nasal endoscopy, both showed a smoothly surfaced, spheroid, soft tissue mass in the dorsal nasal septum. On CT-scans, a displacing growth was described for the first patient; in the second patient, a bony arrosion of the floor of the sphenoid sinus was suspected. Both lesions were surgically completely removed. The histopathological diagnosis was myxoma in the first and myxoid chondrosarcoma in the second patient. An early, locally recurrent tumor in the second patient could also be surgically removed. Both patients have been in full remission for more than a year. In spite of the similar symptoms and clinical findings, histopathologically different myxoid neoplasms of the nasal septum show marked variations in growth and recurrence.

Aged, 80 and over↗

[Subtotal cricoid resection as primary therapy for a highly differentiated chondrosarcoma].

Chondrosarcomas of the larynx are rare malignant tumors usually diagnosed with significant delay due to their nonspecific symptoms. We report a 50-year-old male presenting with progressive dyspnea. Indirect laryngoscopy revealed a subglottic stenosis. The cricoid cartilage was shown on CT to be massively damaged. Histologic differentiation between chondroma and highly differentiated chondrosarcoma was very difficult. Therefore, an organ-preserving treatment concept using partial cricoid resection and staged endoscopic arytenoidectomy was chosen. Total laryngectomy and permanent tracheostomy could be avoided. Due to the risk of recurrence, early follow-up with endoscopy and CT is mandatory.

Chondrosarcoma↗

[Mesenchymal chondrosarcomas of the facial skull].

A 13-year-old girl suffered from a mesenchymal chondrosarcoma of the left maxilla. The therapeutic options and the prognosis for this disease are described with respect to the currently known 72 cases in the literature. Mesenchymal chondrosarcomas are rare tumors of the bone and soft tissue. The first clinical symptom is a painless swelling of the facial skull. They occur largely in the 2nd and 3rd decades of life, preferentially in males. Radiological criteria for the identification of this type of tumor include focal ossification areas which are accompanied by non-calcified regions. Complete surgical removal of the tumor is the therapy of choice. Pre- and postoperative chemotherapy can have a beneficial effect. The final outcome of the disease is difficult to evaluate since late complications (e.g., reoccurrence and/or metastases) appear even after 20 years and only a small number of cases have been reported. At present, the 5-year survival rate is reported to be 54-82% and the 10-year rate 28-56%.

Adolescent↗

[Neoplastic chondroneogenesis as a characteristic of mesenchymal chondrosarcomas].

Mesenchymal chondrosarcomas are rare skeletal malignancies, which are typically characterized by tumor compartmentation. One tumor area is formed by small, undifferentiated neoplastic cells, whereas the second compartment is composed of cartilaginous areas. In this study, the application of in situ detection techniques enabled us to characterize the different tumor compartments according to their cellular differentiation patterns. The use of characteristic marker genes identified all steps of chondrogenesis within the different tumor compartments. Undifferentiated tumor cells in the small-cell areas were negative for vimentin and the cytoprotein S-100, whereas other tumor cells expressed collagen type IIA and vimentin indicating a chondroprogenitor cellular phenotype in these small-cell areas. Fully differentiated chondrocytic cells expressing collagen type II were found in the chondroid areas. The focal expression of type X collagen indicated hypertrophic differentiation of the neoplastic chondrocytes. The results characterize mesenchymal chondrosarcomas as skeletal malignancies that arise from undifferentiated chondroprogenitor cells and have the potential to undergo all steps of chondrocytic differentiation.

Biomarkers, Tumor↗

Chondrosarcoma of the larynx: treatment with radiotherapy.

The case of a 50-year-old man with chondrosarcoma of the larynx treated with radiotherapy is reported. The patient presented with hoarseness and dyspnea. He underwent computed tomography (CT), which demonstrated a soft tissue mass of the larynx. Direct laryngoscopy with biopsy established the diagnosis of chondrosarcoma. Although experience with radiotherapy in these cases has been lacking in the literature, it was considered and eventually used, as radical surgery would result in severe cosmetic and functional impairment. Radiation therapy alone resulted in long-term remission of the tumour for more than 3 years. The patient has been followed up using CT and direct laryngoscopy for early detection of recurrence or metastases.

Chondrosarcoma↗

Chondrosarcoma of the nasal septum.

The nasal septum is a particularly rare site of origin of chondrosarcoma. Cranial base invasion may be at hand, with such lesions making complete tumor removal difficult. MRI techniques allow precise definition of tumor extent. In the described case, CT and Dynamic MR imaging were performed in a case of chondrosarcoma of the nasal septum. Imaging clearly illustrated size and extent of the mass with central regions of internal calcification. Dynamic MRI was additionally performed, which helped to define the presumed origin of the lesion from the nasal septum.

Adult↗

Dedifferentiated chondrosarcoma of the appendicular skeleton: MRI-pathological correlation.

OBJECTIVE: To correlate the T2-weighted and STIR MRI appearances of dedifferentiated appendicular chondrosarcoma with gross and microscopic pathology. DESIGN AND PATIENTS: Nine patients with a histologically confirmed diagnosis of dedifferentiated appendicular chondrosarcoma were identified from the Bone Tumour Registry. All patients underwent MRI, including T2-weighted and/or STIR sequences in at least one plane, prior to limb salvage surgery. Areas of reduced signal intensity (SI) compared with hyperintense chondral tumour on the T2-weighted or STIR images were correlated with the resection specimen, to determine the relationship of such out areas of reduced SI with regions of dedifferentiation. RESULTS AND CONCLUSIONS: Patients presented over a period of 7 years. There were five men and four women with mean age 68.2 years and age range 51-78 years. Tumours arose in the femur (6 cases), humerus (2 cases) and tibia (1 case). Three MRI patterns were identified: (1) type 1, a lesion with two distinct signal characteristics-hyperintense chondral and reduced SI dedifferentiated tumour (n=6); type 2, mainly reduced SI lesion-dedifferentiated tumour, with areas of signal void corresponding to matrix calcification (n=2); type 3, a heterogeneous lesion with no radiological evidence of underlying chondral tumour (n=1). T2-weighted or STIR MR sequences can identify areas of dedifferentiation, which should be the preferential site of pre-operative biopsy.

Aged↗