Primary synovial sarcoma of bone: A retrospective analysis of 25 patients

Alberto Righi, Marco Gambarotti, Stefania Benini, Dino Gibertoni, Sofia Asioli, Giovanna Magagnoli, Gabriella Gamberi, Marta Sbaraglia, Stefania Cocchi, Eric Staals, Emanuela Palmerini, Angelo Paolo Dei Tos

Research output: Contribution to journalArticlepeer-review

Abstract

AIMS: To evaluate diagnostic accuracy of SSX and SSX-SS18 antibodies in decalcified surgical specimens and outcome of synovial sarcomas (SS) of bone.

METHODS AND RESULTS: Twenty-five cases were classified as bone SS (prevalence 0.32% among malignant primary bone sarcoma). Median age was 34 years (range 9 -79) . 24/25 patients presented with non-metastatic tumors, one with lung metastases. The majority of tumors involved the long bones of extremities with metaphyseal origin. Mean size of the tumor was 7.1 cm. 20 cases (80%) were monophasic and 5 (20%) biphasic. SS18-SSX fusion-specific antibody had 92% sensitivity and 99% specificity for primary bone SS, whereas SSX C-terminus antibody had 100% sensitivity and 94% specificity. FISH analysis was feasible in 9 (36%) cases and detected SS18 rearrangement in all 9 cases. All patients underwent surgical removal of their primary tumor, with adequate margins in 18 (72%) patients. Chemotherapy with metothrexate, cisplatin, doxorubicin, ifosfamide was used in the 7 patients. Two patients with inadequate surgical margins received radiotherapy. With a median follow-up of 80 months (range 6-428), 5-year and 10-year overall survival (OS) were 66.6% and 47.9% respectively and 5-and 10-years disease-free survival (DFS) were 36.8% (95% C.I.: 18.0-55.7%), and 32.2% (95% C.I.: 14.6-51.2%) respectively A significant improvement in 10-years DFS in patients undergoing chemotherapy as compared with patients who did not was observed (p 0.039).

CONCLUSIONS: Our series highlights the utility of SS18-SSX fusion-specific and SSX C-terminus antibodies to support the diagnosis of SS, Adjustment chemotherapy was associated with improved prognosis in this series.

Original languageEnglish
JournalHistopathology
DOIs
Publication statusE-pub ahead of print - Nov 25 2021

Keywords

  • adoloscent and young adults
  • bone tumors
  • prognosis
  • synovial sarcoma
  • ultra rare bone sarcoma

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