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Eight patients with 12 chondrosarcomas were treated with radiation and razoxane (ICRF 159). Two tumors in 1 patient progressed unequivocally, 3 tumors in 3 patients showed no change, and 7 tumors in 5 patients had complete or partial (more than 50%) regressions. At least 2 complete regressions have responded for more than 2 1/2 years at the present time.
The case histories and tissue sections from 280 patients with chondrosarcoma of the bones of the limbs, limb girdles, and spinal column were studied retrospectively, and the findings were correlated with survival by actuarial methods. One hundred thirty patients with no prior treatment who underwent curative surgery were studied separately. Size and histologic grade of tumor were the most significant prognostic factors. Control of the primary lesion was found to be of critical importance; local recurrence adversely affected survival.
The current definition of osteosarcoma, based on the prescence of tumor osteoid, is unsatisfactory because it fails to identify some examples of chondroblastic, fibroblastic, and anaplastic osteosarcoma having no demonstrable tumor osteoid. The tumor cells in osteosarcoma, whether osteoblastic, chondroblastic, fibroblastic, or anaplastic, contain abundant alkaline phsophatase, whereas this enzyme is scanty or absent in condrosarcoma and fibrosarcoma. It is therefore proposed that these bone sarcomas are best defined according to the origin of the constituent tumor cells and their alkaline phosphatase content: osteosarcoma--a malignant tumor of osteoblasts (alkaline phosphatase positive); chondrosarcoma--a malignant tumor of chondroblasts (alkaline phosphatase negative); and fibrosarcoma--a malignant tumor of fibroblasts (alkaline phosphatase negative).
Almost a quarter of a century has passed since mesenchymal chondrosarcoma, a rare, insufficiently studied and still poorly understood cartilage neoplasm has been described. Based on 35 cases diagnosed and treated at this Cancer Center, this study found 20 males and 15 females with an average age of 26 years (range, 6-70 years). All but five of the tumors arose in the skeleton in the femur, humerus, and ilium in five cases each, while the os calcis (a rare site for any other osseous tumor) gave rise to this tumor in four instances. Pain was the cardinal symptom in 27 patients. The lesional size varied from 4 to 18 cm (average, 9.5 cm). Histologic examination revealed nine of the tumors to be of the small cell undifferentiated types while the others were of the "hemangiopericytomatoid" variant. According to this subclassification, patients with the small cell type of lesions responded to combination chemotherapy and irradiation, as usually do other small cell sarcomas. The addition of surgical resection may be of value especially in the patients with the hemangiopericytomatoid variant. Preliminary results in the treatment of five such patients with evaluable disease suggest that this combined treatment approach is encouraging. Follow-up analysis of all patients revealed a 37.9 months median survival, and 28% to be alive at ten years.
Two cases of extraskeletal myxoid chondrosarcoma are reported. The patients presented with metastasis to the lung, 10 years and 2 years, respectively, prior to discovery of the primary neoplasms in the soft tissues of the lower extremities.
Chondrosarcoma of the temporal bone is a rare lesion. Clinically it has been confused with multiple sclerosis, glomus jugulare tumors, meningioma, and chordomas. The cranial nerve palsies frequently observed with the tumors are related to the anatomic locations of the tumors. Thirteen patients with this entity are presented and the eleven other cases in the literature are reviewed. Histologically the tumors are low grade and exhibit myxoid features. The myxoid features must be differentiated from chordoma and chondroid chordoma. The tumor locations preclude surgical excision and conventional radiation therapy can cause unacceptable neurologic sequelae. Proton beam therapy has been effective in short-term results and appears capable of avoiding serious neurologic side effects.
The Swarm chondrosarcoma, a transplantable tumor maintained in rats, has a structure similar to hyaline cartilage. The extracellular matrix contains narrow, banded collagen fibrils and proteoglycan matrix granules. The cells are filled with organelles typically involved in synthesis and secretion, including an extensive rough endoplasmic reticulum with dilated cisternae, a large Golgi apparatus dispersed throughout the cell, and secretory vacuoles which fill directly from Golgi saccules. There is only one class of secretory vacuoles, suggesting that the secretory products, collagen and proteoglycan, are packaged and secreted together. The cells also contain unusual endoplasmic reticulum, many coated vesicles and multivesicular bodies, and abnormal mitochondria. The large amount of tissue available from the tumor, and its characterization biochemically (Choi et al., '71; Oegema et al., '75) and morphologically as cartilage, make the tumor valuable as a model tissue for studies of synthesis and secretion of extracellular matrix materials.
When chondrocytes from the Swarm rat chondrosarcoma are isolated by trypsin and collagenase digestion and cultured in Petri dishes, they form a new extracellular matrix within 24 hours, with proteoglycan matrix granules and collagen fibrils. This rapid synthesis of new matrix, together with the biochemical and morphological characterization of the proteoglycans as typical of cartilage, demonstrates the value of these cultures as a model system for studies of synthesis, secretion, and organization of extracellular matrix.
BACKGROUND: Extraskeletal myxoid chondrosarcoma (EMC) is a rare malignant neoplasm. Despite a consensus for the distinct clinicopathologic entity of EMC, its clinical features remain controversial. In addition, most studies have contained a small number of patients who underwent definitive surgical treatment. METHODS: Forty-two cases of EMC, which had been identified from files of eight affiliated hospitals and confirmed for histologic diagnosis at the Pathology Center, were analyzed for histologic grade, demographics, treatments, outcomes, and prognostic factors. The average follow-up period was 7.4 years. RESULTS: Included in the study were 20 men and 22 women with a mean age at diagnosis of 52.1 years. The tumors were located mainly in the lower extremities (69%). Thirty-three tumors (79%) were classified as Grade 1 and nine as Grade 2 according to the modified French System. Overall survival was 100% at 5 years and 88% at 10 years. Disease-free survival was 45% at 5 years and 36% at 10 years. Inadequate initial surgery was defined as a significant risk factor for local recurrence by univariate analysis of all 42 patients but not by the analysis of those 30 patients who had undergone wide tumor excision or amputation. Wide excision led to the recurrence rate of 14%. CONCLUSIONS: These findings supported the role of wide excision in the local control of EMC, irrespective of the previous excision procedure or recurrence. The protracted clinical course of the tumors and the presence of patients who had distant metastasis develop after definitive surgery of the primary tumor represented EMC as intermediate malignancy.
Fine-needle aspiration (FNA) is a reliable, safe and cost-effective procedure with an established role in the diagnosis of various solid tissue neoplasms. However, the role of FNA in the diagnosis of primary bone tumors, including chondrosarcoma (CS) is controversial. To determine the accuracy of FNA as a diagnostic procedure, the author reviewed the institutional experience of a series of patients with CS who underwent FNA for diagnosis. The author's objectives were to determine the accuracy of the technique as well as possible limitations to sensitivity and specificity, and perhaps to suggest the most appropriate use for this procedure. Computer records and then subsequently archives of the department were searched for patients diagnosed and treated for CS between 1993 and 2003. Patients without adequate clinical follow-up, missing materials or records otherwise unavailable for review were eliminated from study. All patients who underwent FNA for a diagnosis had to have a subsequent histological confirmation to be included in the study. FNAs were largely performed with image-guided assistance. In those that were palpable, the aspiration was performed by the aspiration cytologist using standard methods. Histologic materials were processed according to standard methods. All cytological and histologic materials were reviewed for accuracy and appropriateness of diagnosis by the author. There were 34 aspirates from 32 patients with CS (2 patients with 2 aspirates each). Attempts at diagnoses were made from 27 primary lesions, 6 recurrent lesions, and one metastatic lesion. There were an additional two patients who were assigned a diagnosis of CS on FNA who ultimately were proven to have chondroblastic osteosarcoma. Of the primary CS, 18 were definitively diagnosed as CS or "malignant chondroid neoplasm," 8 of the aspirates were considered equivocal in that an additional diagnostic procedure was required to clarify or confirm the diagnosis. Two aspirates were diagnosed as negative. Both of the false negatives were due to inadequate sampling of the lesion on FNA. Diagnostic accuracy of FNA for primary CS in this series was 67% (18/27). Accuracy for recurrent or metastatic lesions was higher at 86% (6/7). FNA appears to be a reliable means of diagnosis of recurrent and/or metastatic CS in patients with a documented history. In primary lesions, however, the accuracy of the technique is lower. In addition, there are problems of sampling chondroid components of non-CS lesions such as this study's experience with chondroblastic osteosarcoma.
A 6-yr-old boy already treated (surgery and chemotherapy) for a diffuse anaplastic Wilms' tumor discovered at the age of 10 mo, presented with a large tumor in the left femur. This proved to be a chondrosarcoma after fine-needle aspiration cytology, a diagnosis later confirmed by histology of the tumor. The patient was treated with marginal excision and prosthetic replacement but died 1 yr after this operation with lung metastasis. It is proposed that this case may represent a new association of unusual types of tumors.
Extraskeletal myxoid chondrosarcoma, an unusual soft tissue lesion which preferentially affects the extremities, is a prime candidate for "pathologist performed" fine-needle aspirates (FNA) because it preferentially affects the extremities [Enzinger and Shiraki, Hum Pathol 1972;3:421-435] and is usually an accessible lesion. Cytomorphologically, the neoplasm consists of clusters of fairly uniform cells, lacking cartilaginous differentiation, with a metachromatic matrix when stained with Diff-Quik (DQ). While subtle, this neoplasm has distinct features and must be distinguished for other neoplasms in the differential diagnosis because patient evaluation, prognosis, and therapy will vary greatly. We present a case characteristic of this tumor in which FNA derived material was subjected to light microscopy, X-ray microanalysis, and immunohistochemical studies; each modality provided data essential to the correct diagnosis.
Extraskeletal myxoid chondrosarcoma (EMC) is a malignant soft tissue tumor which typically presents as an enlarging mass in an extremity. In this location it is amenable to sampling and diagnosis by fine-needle aspiration biopsy. We present our experience with three cases of EMC and discuss the differential diagnosis of myxoid soft tissue tumors.
Extraskeletal myxoid chondrosarcoma (EMC) is a soft-tissue neoplasm cytogenetically characterized by the translocations t(9;22)(q22;q11-12) or t(9;17)(q22;q11), generating EWS/CHN or RBP56/CHN fusion genes, respectively. In the present study, 18 EMCs were studied both cytogenetically and at the molecular level. Chromosomal aberrations were detected in 16 samples: 13 with involvement of 9q22 and 22q11-12, and three with rearrangements of 9q22 and 17q11. Fifteen cases had an EWS/CHN fusion transcript and three had an RBP56/CHN transcript. The most frequent EWS/CHN transcript (type 1; 10 tumors), involved fusion of EWS exon 12 with CHN exon 3, and the second most common (type 5; two cases) was fusion of EWS exon 13 with CHN exon 3. In all tumors with RBP56/CHN fusion, exon 6 of RBP56 was fused to exon 3 of CHN. By genomic XL PCR and sequence analyses, the breakpoints from 14 cases were mapped in the EWS, RBP56, and CHN genes. In CHN, 12 breakpoints were found in intron 2 and only two in intron 1. In EWS, the breaks occurred in introns 7 (one break), 12 (eight breaks), and 13 (one break), and in RBP56 in intron 6. Repetitive elements such as Alu and LINE sequences seem to have limited, if any, importance in the genesis of EWS/CHN and RBP56/CHN chimeras. Furthermore, there were no chi, chi-like, topoisomerase II, or translin consensus sequences in the introns harboring the translocation breakpoints, nor could the number of topo I sites in EWS, RBP56, and CHN introns explain the uneven distribution of the breakpoints among EWS or CHN introns. Additional genetic events, such as nucleotide insertions, homologies at the junction, deletions, duplications, and inversions, were found to accompany the translocations, indicating that the chromosomal translocations do not require sequence-specific recombinases or extensive homology between the recombined sequences.
Extraskeletal myxoid chondrosarcoma (EMC) is characterized by recurrent chromosomal translocations resulting in fusions of the nuclear receptor gene NOR1 (also known as CHN or TEC) to various N-terminal partners, including EWS and TAF2N (or RBP56). Significant structural homology of EWS or TAF2N to TLS (or FUS) prompted us to investigate a potential novel gene fusion of NOR1 to TLS in EMCs without detectable known NOR1 fusions. In one of the EMCs examined, our reverse-transcription polymerase chain reaction using NOR1 and TLS primers unexpectedly amplified a cDNA sequence derived not from a TLS/NOR1 fusion but from a TFG/NOR1 fusion, a hitherto undescribed fusion type in EMC, probably a result of incidental misannealing by the TLS primer, which has a sequence partially identical to TFG. Encoding a protein with a putative coiled-coil structure, TFG previously was identified by a homology search in the Expressed Sequence Tag Database as having an SPYGQ-rich region similar to the N-terminal parts of EWS and TLS. TFG/NOR1 fusion appears to play an oncogenic role equivalent to those of other NOR1 fusions in EMC.
Chondrosarcoma arising in the nasal septum has been previously described only 11 times. In this report, another case occurring in an 89-year-old man is discussed. The rarity of such a tumor arising in previously normal cartilage is emphasized. Prognostic factors and surgical treatment are discussed.
Chondrosarcoma of the head and neck region is a rarely encountered tumor. Aggressive surgical excision offers the best chance for cure. In our case, combined diagnostic techniques consisting of fine needle aspiration (FNA), computed tomography (CT), and magnetic resonance imaging (MRI) made an accurate preoperative assessment possible and greatly influenced the treatment provided.
N-terminal sequences of 19 and 11 amino acids obtained from 2 different tryptic fragments of the tumour-specific antigen on the chemically induced rat chondrosarcoma HSN show a 100% homology with the rat chondroitin sulfate proteoglycan NG2. Using a scheme of overlapping oligonucleotide primers we have cloned by PCR amplification the cDNA for the specific antigen of the HSN tumour that is immunogenic in immunocompetent CBH/Cbi rats and now report that its cDNA sequence is identical to that of NG2. The cDNA codes for a transmembrane protein of 2,325 amino acids with a large extracellular domain of 2,224 amino acids containing 2 cysteine-rich regions, a transmembrane domain (25 amino acids) and a short cytoplasmic tail (76 amino acids). The tumour-specific determinant was found to lie between amino acid residues 556 and 992 on the core glycoprotein.