Clinical epidemiology and treatment outcomes of spindle cell non-osteogenic bone sarcomas - A nationwide population-based study

J Bone Oncol. 2018 Nov 20;14:002-2. doi: 10.1016/j.jbo.2018.11.002. eCollection 2019 Feb.

Abstract

Purpose: To describe epidemiological and clinical characteristics, as well as long-term treatment outcomes of spindle cell non-osteogenic bone sarcomas (SCS), comprising leiomyosarcoma, fibrosarcoma and undifferentiated pleomorphic sarcoma in bone.

Method: We have analysed a nationwide cohort of 104 patients with histologically verified SCS diagnosed between 1975 and 2009, based on registry sources supplemented with clinical records from Norwegian hospitals involved in sarcoma management.

Results: In this unselected cohort, a stable annual incidence for SCS patients of slightly below 0.6 per million was observed, with a dominant peak among elderly patients. SCS is mostly a high-grade malignancy (92%) with a male to female ratio of 1.6 for all patients. The axial to appendicular ratio was 0.7, seemingly independent of age. More than one fourth of the patients (29%) had primary metastatic disease. Another 32 patients (46%) developed metastases during follow-up and 12 (17%) experienced local relapses. The five-year sarcoma-specific survival rate was 37%, with no documented improvement over time. Primary metastatic disease was an adverse prognostic factor for survival. Predisposing factors were documented in 19 patients (18%). Negative prognostic factors for overall survival were tumour size >9 cm, age > 40 years, axial tumour localization, FS as subtype and pathologic fracture at time of diagnoses. As expected, patients who received both surgery and chemotherapy as their primary treatment for high-grade SCS (25%) significantly had best sarcoma specific five years survival (62%).

Conclusion: We confirm SCS as a rare high-grade bone sarcoma entity, mostly among elderly patients and with a poor overall outcome. The combined treatment of surgery and chemotherapy is essential to achieve optimal long-term survival of SCS.

Keywords: Nationwide; Overall survival; Spindle cell; Treatment.