Secondary peripheral chondrosarcoma arising in solitary osteochondroma: variables influencing prognosis and survival

Abstract

Background Secondary peripheral chondrosarcomas arising in solitary osteochondromas is an unusual complication, reported in small series. In this study, we aimed to present our experience with this rare variant of chondrosarcoma and compare results with already published data in order to determine prognostic factors for overall and disease-free survival. Methods The case study includes retrospective data from patients diagnosed at a single institution from 1943 to 2019. Clinical data were collected reviewing all available medical records from first to last follow-up visits. To exclude the presence of the Multiple Osteochondroma Hereditary Syndrome, few patients, with a suspect of a familial form of the disease, were evaluated for the presence of germline heterozygous variants in EXT1 and EXT2 genes. Results were summarized using descriptive statistics and statistical analysis were performed to reveal associations between variables. Results Two hundred and fourteen secondary peripheral chondrosarcomas that arose exclusively from solitary osteochondromas diagnosed in a multidisciplinary setting at the IRCCS Istituto Ortopedico Rizzoli were retrospectively identified, 66.4% males and 33.6% females with a median age at diagnosis of 38 years. The local recurrence rate was 17.3%, while the metastases one was 5.1%. Besides age, a high histologic grade is the only factor associated with worse 5-year and 10-year overall survival (log-rank p = 0.0005, HR = 3.74; 95% CI 1.69–8.26). Moreover, high histological grade (HR = 3.75; 95% CI = 1.69–8.34; p = 0.001) and surgical debulking (HR = 3.71; 95% CI = 1.57–8.79; p = 0.003) were associated with a significantly worse disease-free survival. Conclusions Our study confirm the low-grade behavior of secondary peripheral chondrosarcomas and demonstrate that the best choice of treatment for those arising in solitary osteochondromas is the wide surgical excision, when possible. Location per se is not a factor that affects prognosis, while the accurate histological grade assessment is correlated with the tumor aggressiveness and a long term follow up is necessary for this rare variant of chondrosarcoma.

Deep learning models for predicting the survival of patients with chondrosarcoma based on a surveillance, epidemiology, and end results analysis

“…Accurate prediction for chondrosarcoma survival is crucial for the counseling, follow-up, and treatment planning of patients. Previous studies have revealed various prognostic factors influencing the survival times of patients with chondrosarcoma, including patient age, tumor size, histological type, tumor grade, and metastasis ( 6 , 19 – 21 ).. At the same time, increasing amounts of imaging ( 22 , 23 ) and genetic data ( 2 , 24 ) are being mined for survival analysis of chondrosarcoma patients. In the face of high-dimensional data, the limitations of the linear relationship between variables assumed by the classical CoxPH model are evident ( 11 ).…”

Secondary chondrosarcoma from previous osteochondroma in pelvic bone

“…They proposed that secondary peripheral chondrosarcomas have a low-grade behavior and that, when possible, wide surgical excision is the best treatment option for those that arise in solitary osteochondromas. The tumor's location does not affect prognosis, but accurate histological grade assessment correlates with tumor aggressiveness, and longterm follow-up is required for this rare variant of chondrosarcoma [18]. Ahmed et al reviewed 107 patients with secondary chondrosarcoma arising from a single osteochondroma (61 patients) or multiple exostoses (46 patients).…”

Diagnostic and treatment of secondary chondrosarcoma of the femur with multiple recurrences. Case report

“…Кроме того, подтвержденный по результатам лучевого обследования вторичный характер опухоли на фоне остеохондромы позволил при первичной операции выбрать органосохраняющий объем. При опухолях G2 центральной локализации пациентам показана сегментарная резекция с эндопротезированием, при периферической локализации (на фоне остеохондромы, как в данном случае) зачастую возможно удаление опухоли с краевой резекцией кости, что и было сделано [40,41].…”