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PubMed2000

Mesenchymal Chondrosarcoma: A Small Cell Neoplasm with Polyphenotypic Differentiation.

We analyzed the clinicoradiographic, microscopic, and immunophenotypic features of 21 tumors from 13 patients with mesenchymal chondrosarcoma (11 primary and 10 metastatic) and addressed the issue of their potential polyphenotypic differentiation. The immunophenotypic profile of the tumors was analyzed by studying the expressions of the MIC2 gene protein (p30/32(MIC2)), S-100 protein, desmin, myoD1, myogenin, myoglobin, smooth-muscle actin, cytokeratin, neuron-specific enolase, and HMB-45. The expression of p30/32(MIC2) was typically restricted to the small cell component of the mesenchymal chondrosarcoma and could be documented in 17 tumors. The cartilaginous areas were positive for S-100 protein in 20 tumors. Scattered positivity of small cells for desmin was seen in 8 cases. In 2 primary tumors from different patients (1 intraosseous and 1 extraskeletal) a diffuse expression of desmin with focal coexpression of myoD1 was present within the small cell component of the tumor. The positivity for smooth-muscle actin was documented in 2 cases. Either the small cell or cartilaginous components were at least focally positive for neuron-specific enolase in 11 tumors. All tumors were negative for myogenin, myoglobin, cytokeratins (AE1/AE3, CAM5.2) and HMB-45. This study showed that, in addition to cartilaginous differentiation, mesenchymal chondrosarcomas may exhibit focal expression of desmin. In rare cases more diffuse rhabdomyoblastic differentiation can be seen within the small cell component of the tumor. Thus, mesenchymal chondrosarcoma is another primitive neoplasm with polyphenotypic differentiation and features that overlap those of other small cell malignances of bone and soft tissue. Int J Surg Pathol 8(4):291-301, 2000

Journal Article↗
PubMed1992

Amplification of the c-myc proto-oncogene in human chondrosarcoma.

The genomic organization of four oncogenes, i.e., c-myc, c-myb, c-Ha-ras, and c-fms, was investigated in fresh surgical specimens from 10 patients with cartilaginous tumors. Among nine chondrosarcomas, six were primary lesions and three local recurrences. The remaining case was a chondroblastoma. Amplification of the c-myc proto-oncogene was the sole abnormality detected in this series, occurring in two chondrosarcomas (four- and eight-fold). No other genetic alteration such as oncogene rearrangement was found. Nor was there any amplification of the other oncogenes studied. Both c-myc-amplified tumors were primary lesions and histologically classified as grade II; according to flow DNA cytometry, one was diploid and the other aneuploid. In our limited series, there was no overall relationship between c-myc amplification, on the one hand, and histologic subtype, malignancy grade, surgical stage, or ploidy level, on the other. Our study shows that amplification of the c-myc oncogene, presumed to be involved in the development of malignancy, is encountered in occasional human chondrosarcomas, however, without any relationship to other well-known features of this tumor entity. The clinical significance of this gene amplification remains to be established.

Adult↗
PubMed1997

Laryngeal chondrosarcomas: the Mayo Clinic experience.

BACKGROUND: Laryngeal chondrosarcomas occur infrequently. Their management is often guided by inferences made from the management of sarcomas arising from more commonly afflicted organs. METHOD: A retrospective analysis of patients with laryngeal chondrosarcomas treated at the Mayo Clinic between 1959 and 1992 was performed to assess prognostic factors and outcomes after various treatments. RESULTS: A total of 20 patients received treatment during this time period. All chondrosarcomas were low grade; 19 involved the cricoid cartilage and one arose in the supraglottic larynx. Initial treatment consisted of local excision (often subtotal removal) alone in 12 patients (60%), hemilaryngectomy in 2 (10%), near total laryngectomy in 2 (10%), and total laryngectomy in 4 (20%). Six patients (30%) had local recurrence: five initially had local excision and one had hemilaryngectomy. All local recurrences or tumor progression developed >3 years after initial treatment. Salvage surgery was performed in five of the six patients who had local recurrence, and the other patient was observed. Of the five patients who had salvage surgery, three required another resection because of a second recurrence. CONCLUSIONS: These results suggest that initial conservative subtotal laryngectomy should be explored further because this treatment may provide long-term voice preservation in most patients, and patients who experience a recurrence after local excision often have been given several years of voice preservation.

Aged↗
PubMed1996

Mesenchymal chondrosarcoma of the foot, an unusual location: case report and review of the literature.

We present a case of primary mesenchymal chondrosarcoma of the proximal phalanx of the first toe. The bones of the foot represent an infrequent primary site for this neoplasm. The tumour consisted of layers of undifferentiated round cells with scanty cytoplasm and hyperchromatic nuclei. The presence of brain, lung, and left auricle metastasis was demonstrated, and the patient died due to brain edema 18 days after admission. Mesenchymal chondrosarcoma is a rare tumor that more frequently involves the pelvic bones, the femur, and the humerus. To our knowledge, only nine cases of primary mesenchymal chondrosarcoma arising from the bones of the foot have been previously reported, with none involving the phalanx of the toe.

Adult↗
PubMed1999

Molecular genetic characterization of both components of a dedifferentiated chondrosarcoma, with implications for its histogenesis.

Dedifferentiated chondrosarcoma is defined as a high-grade, anaplastic sarcoma adjacent to a low-grade malignant cartilage-forming tumour. Controversy remains as to whether the anaplastic and cartilaginous components are derived from a common precursor cell, or whether they represent separate genotypic lineages (collision tumour). Both components of a case of dedifferentiated chondrosarcoma were therefore separately investigated by loss of heterozygosity (LOH) analysis, comparative genomic hybridization (CGH), DNA flow cytometry, and p53 analysis. Both showed p53 overexpression and an identical somatic 6 bp deletion in exon 7 of p53. Combination of the CGH and LOH results revealed that both components had lost the same copy of chromosome 13. These results provide compelling evidence in this case for a common origin, instead of the 'collision tumour' theory. Certain genotypic alterations were not shared. The anaplastic component showed severe aneuploidy, LOH at additional loci, and amplification and deletion of several chromosome parts. In contrast, the cartilaginous component had lost chromosomes 5, 22, 17p and part of 16p and revealed an amplification of 17q. The LOH and CGH results further demonstrated that the two components had lost a different copy of chromosome 4. Thus, a substantial number of genetic alterations have occurred after the diversion of the two components, indicating that the separation of the two clones, derived from a single precursor, was a relatively early event in the histogenesis of this case of dedifferentiated chondrosarcoma.

Autoradiography↗
PubMed1996

Mesenchymal chondrosarcoma: a clinicopathologic and flow cytometric study of 13 cases presenting in the central nervous system.

BACKGROUND: Mesenchymal chondrosarcomas arising in the central nervous system are extremely rare. Morphologic features have not been found to correlate reliably with prognosis. METHODS: Eight intracranial and five intraspinal mesenchymal chondrosarcomas were reviewed with regard to location, treatment, and long term follow-up data. The histopathologic and immunohistochemical results, including Ki-67 nuclear staining frequency, were critically reviewed, and deoxyribonucleic acid content was analyzed by flow cytometry. RESULTS: Microscopically, all 13 cases were remarkably similar. Immunoreactivity in the small cell component included vimentin in 100% and cytokeratin and glial fibrillary acidic protein in 25% of cases. S-100 immunoreactivity was noted in the cartilaginous component of 100% of cases, and in rare cells in the small cell component along the interface. Flow cytometry of the eight tumors studied revealed a diploid pattern in six, aneuploidy in two, and a wide range of S-phase fractions (0-36.5%). CONCLUSIONS: Review of the literature and the findings of the current series indicates that mesenchymal chondrosarcomas presenting in the brain and spinal cord pursue a progressive course that correlates most reliably with extent of surgical resection. This limited retrospective study also suggests that survival may be shorter for those patients with a high S-phase fraction and a high Ki-67 staining frequency.

Adolescent↗
PubMed1997

Myxoid chondrosarcoma (chordoid sarcoma) of bone: a report of two cases and review of the literature.

BACKGROUND: Chondrosarcoma of bone is a well recognized, relatively common clinicopathologic entity. Morphologically distinct soft tissue chordoid sarcoma (CS), or extraskeletal myxoid chondrosarcoma, is a relatively rare tumor that has generally been documented in extraosseous soft tissues. METHODS: The clinical and pathologic features of two patients with biopsy-proven CS from the pathology files of the Mayo Clinic and St. Thomas's Hospital were evaluated. Routine hematoxylin and eosin-stained slides were reviewed in both cases. Sections from both were examined immunohistochemically using the avidin-biotin-peroxidase technique and employing commercially available antibodies to the following antigens: S-100 protein, cytokeratin (AE1/AE3), epithelial membrane antigen (EMA), CD31, and factor VIII. Appropriate positive and negative controls were utilized throughout these procedures. Cytogenetic analysis was performed on fresh samples obtained from one tumor. Clinical data were obtained from the patients' medical records. RESULTS: The two cases of primary CS of bone arose from the right distal femur and right scapula, respectively, in 2 men ages 48 and 76 years, respectively. Morphologically, the tumors were lobulated, multinodular, and comprised of a uniform population of rounded to slightly spindled cells. Nuclei were hyperchromatic with inconspicuous nucleoli and surrounded by clear, vacuolated to eosinophilic cytoplasm. Neoplastic cells were arranged in anastomosing chords, strands, and, less often, nests and pseudopapillary structures embedded in an abundant, mostly hypovascular, mucinous matrix. Foci of hemorrhage and cystic degeneration were present in both tumors. No well developed hyaline cartilage or neoplastic osteoid was observed. Immunohistochemically, one neoplasm showed focal positivity for S-100 protein but was uniformly negative for cytokeratin (AE1/AE3), factor VIII, and CD31. The other tumor showed no immunopositivity with cytokeratin, EMA, or S-100 protein. Cytogenetic analysis in the latter tumor revealed a nonrandom reciprocal chromosomal translocation, t(9;22)(q22-31;q11-12). Both patients developed local recurrences and widespread distant metastases. Wide surgical excision was the primary mode of therapy. One patient died of tumor. CONCLUSIONS: Skeletal CS is an extraordinarily rare neoplasm with a distinct morphology. Although follow-up data were limited to only four examples, including two from the literature, the clinical course appears worse than that for usual chondrosarcoma of bone. Wide surgical resection appears to represent the best mode of therapy. The role of chemotherapy and radiation therapy has not been clearly defined.

Aged↗
PubMed1998

A new human chondrosarcoma cell line (OUMS-27) that maintains chondrocytic differentiation.

A new human chondrosarcoma cell line, OUMS-27, was established. Monolayer cultures consisted of elongated polygonal cells with a doubling time of 41 hr and a plating efficiency of 2.1%. After reaching confluence, the cells continued to slowly proliferate and formed nodule-like structures, which showed metachromasia when stained with toluidine blue, indicating the presence of proteoglycan. The cells in the nodules were round to polygonal in shape, multilayered and surrounded by abundant extracellular matrix. Types I, II and III collagens were identified by Northern blotting and immunostaining. The cells formed colonies (0.1%) in 0.3% soft-agar medium 3 weeks after inoculation. Inoculation of cells into athymic mice resulted in the formation of tumors at the injection site, resembling the original chondrosarcoma. These results demonstrated that OUMS-27 cells expressed a differentiated chondrocytic phenotype. Moreover, OUMS-27 cells had p53-gene mutation. Thus, the OUMS-27 cell line can provide a useful model not only for studies on human chondrocyte but also for basic studies on the diagnosis, treatment and etiology of human chondrosarcoma.

Bone Neoplasms↗
PubMed2000

Myxoid chondrosarcoma of the sphenoid sinus and chondromyxoid fibroma of the iliac bone: cytomorphologic findings of two distinct and uncommon myxoid lesions.

Myxoid chondrosarcoma (MCS) and chondromyxoid fibroma (CMF) are two uncommon myxoid cartilaginous neoplasms with distinct cytologic features, histologic patterns, and immunoprofiles. Because these neoplasms have characteristic biological behaviors and management, their correct diagnosis is crucial to avoid debilitating and unnecessary surgical procedures. We report the imprint cytology (IC) preparation findings along with the differential diagnosis in one case each of myxoid chondrosarcoma and chondromyxoid fibroma of the splenoid sinus and iliac bone, respectively. The two great mimickers for these neoplasms, chordoma and chondrosarcoma, represent difficult diagnostic challenges, especially when MCS and CMF occur in unusual locations. IC in conjunction with the clinical and radiologic findings can provide a rapid preliminary intraoperative diagnostic interpretation which can aid in planning the immediate surgical management, as well as guide specific tissue triage for key ancillary studies such as electron microscopy and cytogenetic analyses. To the best of our knowledge, there have been no cytologic reports of MCS of the sphenoid sinus and CMF of the iliac bone.

Adult↗
PubMed1998

Tracheal chondrosarcoma: a case report.

BACKGROUND: Tracheal neoplasms are extremely rare, representing only 0.2% of malignancies of the respiratory tract. A case of tracheal chondrosarcoma, with airway obstruction, seen in the Department of Otolaryngology Head and Neck Surgery at the University of Cincinnati is presented. Review of the literature was undertaken, revealing 10 previously described cases. Clinical presentation and treatment options are described. METHODS: A literature review of all reports of tracheal chondrosarcoma was undertaken. RESULTS: From this review, we identified only 10 single case reports. The majority of patients were elderly men, with lesions in the mid to distal trachea. Treatment predominantly consisted of tracheal resection. Recurrence was associated with failure to achieve complete resection. CONCLUSIONS: We conclude that tracheal chondrosarcoma is an exceedingly rare upper airway neoplasm. Treatment should be aimed at complete surgical removal.

Aged↗
PubMed1997

Absence of cell-surface annexin V is accompanied by defective collagen matrix binding in the Swarm rat chondrosarcoma.

Annexin V has been characterized as a major collagen type II binding cell-surface component of normal chondrocytes and is also called anchorin CII in chondrogenic populations. Herein we present evidence that in vitro cultured Swarm rat chondrosarcoma cells are not capable of binding collagen type II in significant quantities to their surfaces, as compared to normal rat chondrocytes. This finding coincides with a deficiency of annexin V on the surface of these cells. A small quantity of an intracellular polypeptide could be detected which is immunologically cross-reactive with annexin V but displayed a mobility in SDS-PAGE of less than 34 kD compared to the M(r) 36 kD of intact rat annexin V. By immunohistochemistry the protein could be localized in the cytoplasm of in vitro and in vivo grown tumor cells. By reverse transcription-polymerase chain reaction and Northern blot analysis, a regular-sized mRNA for annexin V could be detected in the chondrosarcoma cells that is expressed in only slightly lower quantities than in normal chondrocytes. Taken together, the data suggest a modified processing or turnover for annexin V in the chondrosarcoma excluding it from being a functionally active collagen type II binding protein. The findings support the hypothesis of cell-surface annexin V as a key component for the formation of the pericellular matrix of chondrocytes.

Amino Acid Sequence↗
PubMed1979

Clear-cell chondrosarcoma: a report of five cases including ultrastructural study.

Five cases of clear-cell variant of chondrosarcoma (Unni et al.) are reported. The tumors occurred in the epiphyseal region of long bones; three in the femoral head. Roentgenographically, the lesion was usually a well-defined and benign appearing one, either purely lytic (3 cases) or with central radiodensity (2 cases). Histologically, all five cases had areas of conventional chondrosarcoma; however, the greater portion of the tumor was made up of sheets of clear-cells intermixed with nonneoplastic bone trabeculae but devoid of chondroid matrix. Electron microscopic studies showed that these clear-cells possess cytoplasmic microvilli, abundant glycogen particles and prominent golgi complexes, like normal or tumorous chondroid cells usually have. In our experience, the best treatment seemed to be en bloc resection with joint replacement; indeed, despite the fact that they are true chondrosarcomas, these tumors usually have a very slow rate of growth.

Adolescent↗
PubMed1983

Myxoid chondrosarcoma of the synovial tissue. A clinicopathologic, histochemical, and ultrastructural analysis.

The first case of a myxoid chondrosarcoma, originating in the synovia of the joint and tendon sheaths of the ankle in a 37-year-old man, is reported. Light microscopically the tumor was similar to extraskeletal myxoid chondrosarcoma as previously described. The chondromatous nature of the tumor was further indicated by the histochemical analysis, which revealed the presence of chondroitin 4- and 6-sulphate in the tumor matrix. Ultrastructurally, chondroblast-like cells with dilated rough endoplasmic reticulum, abundant glycogen, some lipid droplets and numerous microvilli-like projections of the cytoplasm dominated. Also mitochondria-rich cells were observed as well as intermediate cell forms. Based on the observations in this case, the frequent occurrence of synovial chondromatosis, the assumed synovial origin of extraskeletal chondromas and the wide morphologic and functional spectrum synovial intima cells may cover, it is suggested that the myxoid chondrosarcoma originated from the synovial intima cells.

Adult↗
PubMed1984

Mesenchymal chondrosarcoma of the kidney.

A case of mesenchymal chondrosarcoma of the kidney, with osseous metastases, is presented in this article, and the clinical, radiologic, and histopathologic features of this uncommon neoplasm are discussed. The addition of this case to those previously reported in the literature brings the total number of reported cases of mesenchymal chondrosarcoma of extraskeletal origin to 42. None of the previously reported cases originated from the kidney. The metastatic pattern of this case illustrates the propensity of mesenchymal chondrosarcoma to metastatize to unusual locations.

Adult↗
PubMed1986

Mesenchymal chondrosarcoma of bone and soft tissue. A review of 111 cases.

A series of 111 mesenchymal chondrosarcomas was reviewed. The ages of the patients ranged from 5 to 74 years, and approximately 60% of them were in the second and third decades of life. There was no significant sex predilection. Seventy-two tumors, including 5 that involved multiple skeletal sites, arose in bone. Thirty-eight tumors were found in extraskeletal sites. At initial diagnosis, multifocal involvement, both in bone and in soft tissue, was observed in one case. Roentgenographically, the lesions in bone frequently resembled ordinary chondrosarcomas, showing osteolytic and destructive appearances with stippled calcification. Tumors in extraskeletal sites were almost always identified as calcified masses. Histologically, a combination of cellular zones composed of undifferentiated small cells and chondroid zones typically presented a bimorphic appearance that was virtually pathognomonic in most cases. Ablative surgical treatment seemed to be the procedure of choice. The value of irradiation or chemotherapy (or both) was difficult to assess in the current study. Prognosis for patients with mesenchymal chondrosarcoma is usually poor, and long-term follow-up is necessary. In a group of 23 patients from the Mayo Clinic, the 5-year survival rate was 54.6% and the 10-year survival rate was 27.3%.

Adolescent↗
PubMed1988

Extraskeletal myxoid chondrosarcoma in young children.

Two extraskeletal myxoid chondrosarcomas with a solid soft tissue mass occurred on the right upper arm of a 4-year-old boy and on the chest wall of a 1-year-old boy. Microscopically, both tumors were characterized by lobular configuration and were sparsely cellular with a background of myxoid matrix. The cells were small and round, and appeared undifferentiated, sometimes with a narrow eosinophilic cytoplasm. They grew in nests or strands and sometimes in a single file. They were strongly positive for S-100 protein and vimentin. Ultrastructural features suggested that the cells had a poorly differentiated mesenchymal nature with chondrocytic differentiation. These are the sixth and seventh reported cases of extraskeletal myxoid chondrosarcoma occurring in children. There are definite differences between this tumor with immature features and the extraskeletal myxoid chondrosarcoma in adults. Problems of differential diagnoses from other small round cell sarcomas also are discussed.

Arm↗
PubMed1991

Chondrosarcoma of the larynx after radiation treatment for vocal cord cancer.

The case of a 57-year-old man with chondrosarcoma of the laryngeal cartilage is presented, occurring 16 years after radiation treatment for squamous cell carcinoma of the right true vocal cord. Chondrosarcoma of the larynx is an uncommon tumor. The location, grade, and time elapsed from initial treatment make it probably that this patient's chondrosarcoma is associated with his prior radiation treatment. However, it is a rare occurrence, this being the second case reported in the literature.

Adult↗
PubMed1993

Primary chondrosarcoma of the lung. A clinicopathologic study.

BACKGROUND: Primary pulmonary chondrosarcoma is a rare neoplasm. A 73-year-old Japanese man had chondrosarcoma in the right lung. The tumor was considered to be of pulmonary origin because of the absence of extrapulmonary primary lesions for 2 years after lung resection. METHODS: The histologic sections were stained with hematoxylin and eosin, conventional special staining, and immunohistochemical staining. The authors discussed the differential diagnosis and growth pattern, in addition to the histopathologic findings in the tumor cells. In a review of the literature, the authors compared the characteristics between the major bronchus (MB) and lung types. RESULTS: Histopathologically, the tumor cells showed atypical cartilaginous differentiation without osteoid formation, benign or malignant-appearing epithelium, or sarcomatous components other than chondrosarcoma. The tumor showed expansive proliferation, invasion through the alveolar spaces, massive proliferation along the bronchial lumen, significant invasion into small vessels, and extrathoracic metastases. The review of the literature showed that the clinical period ending with surgical treatment was longer in the lung tumors than in the MB tumors. CONCLUSIONS: Although MB tumors were reported to be discovered early, the invasion to major arteries or trachea often led to inoperability; however, lung tumors were considered to be resectable until they grew very large, even though the clinical period from onset to surgical treatment was longer in this type. Recent advancements in diagnostic and surgical techniques are expected to promote early discovery and improve prognosis whether the tumor occurs in the MB or lung.

Aged↗