MYXOID CHONDROSARCOMA (CHORDOID SARCOMA) OF BONE - A REPORT OF 2 CASES AND REVIEW OF THE LITERATURE

Citation
Se. Kilpatrick et al., MYXOID CHONDROSARCOMA (CHORDOID SARCOMA) OF BONE - A REPORT OF 2 CASES AND REVIEW OF THE LITERATURE, Cancer, 79(10), 1997, pp. 1903-1910
Citations number
42
Categorie Soggetti
Oncology
Journal title
CancerACNP
ISSN journal
0008543X
Volume
79
Issue
10
Year of publication
1997
Pages
1903 - 1910
Database
ISI
SICI code
0008-543X(1997)79:10<1903:MC(SOB>2.0.ZU;2-9
Abstract
BACKGROUND. Chondrosarcoma of bone is a well recognized, relatively co mmon clinicopathologic entity. Morphologically distinct soft tissue ch ordoid sarcoma (CS), or extraskeletal myxoid chondrosarcoma, is a rela tively rare tumor that has generally been documented in extraosseous s oft tissues. METHODS, The clinical and pathologic features of two pati ents with biopsy-proven CS from the pathology files of the Mayo Clinic and St. Thomas's Hospital were evaluated. Routine hematoxylin and eos in-stained slides were reviewed in both cases. Sections from both were examined immunohistochemically using the avidin-biotin-peroxidase tec hnique and employing commercially available antibodies to the followin g antigens: S-100 protein, cytokeratin (AE1/AE3), epithelial membrane antigen (EMA), CD31, and factor VIII. Appropriate positive and negativ e controls were utilized throughout these procedures. Cytogenetic anal ysis was performed on fresh samples obtained from one tumor. Clinical data were obtained from the patients' medical records. RESULTS. The tw o cases of primary CS of bone arose from the right distal femur and ri ght scapula, respectively, in 2 men ages 48 and 76 years, respectively Morphologically, the tumors were lobulated, multinodular, and compris ed of a uniform population of rounded to slightly spindled cells. Nucl ei were hyperchromatic with inconspicuous nucleoli and surrounded by c lear, vacuolated to eosinophilic cytoplasm. Neoplastic cells were arra nged in anastomosing chords, strands, and, less often, nests and pseud opapillary structures embedded in an abundant, mostly hypovascular, mu cinous matrix. Foci of hemorrhage and cystic degeneration were present in both tumors. No well developed hyaline cartilage or neoplastic ost eoid was observed. Immunohistochemically, one neoplasm showed focal po sitivity for S-100 protein but was uniformly negative for cytokeratin (AE1/AE3), factor VIII, and CD31. The other tumor showed no immunoposi tivity with cytokeratin, EMA, or S-100 protein. Cytogenetic analysis i n the latter tumor revealed a nonrandom reciprocal chromosomal translo cation, t(9;22)(q22-31; q11-12). Both patients developed local recurre nces and widespread distant metastases. Wide surgical excision was the primary mode of therapy. One patient died of tumor. CONCLUSIONS, Skel etal CS is an extraordinarily rare neoplasm with a distinct morphology . Although follow-up data were limited to only four examples, includin g 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 radia tion therapy has not been clearly defined. (C) 1997 American Cancer So ciety.