Clarifying prognostic factors of small cell osteosarcoma: A pooled analysis of 20 cases and the literature

Abstract

Introduction Small cell osteosarcoma (SCOS) is a rare subtype of osteosarcoma, with limited studies mainly focusing on histological features. Our study aims to analyze our own patients and those reported in the literature to increase the recognition of this rare disease, to evaluate patient survival and to further determine potential prognostic factors. Material and methods Twenty patients with SCOS were treated in our hospital between 2010 and 2019. Their follow-up data were collected retrospectively. A total of 336 literature cases from 58 manuscripts were retrieved by means of a PubMed search with the key word “small cell osteosarcoma”. Data pertaining to treatment and follow-up were extracted. We performed a pooled analysis for the survival of patients and the risk factors for local recurrence (LR), as well as metastatic disease (MD), in a total of 160 patients using the Kaplan-Meier method and Cox regression method. Results We reported our experience in diagnosing and treating SCOS. In our cases, elevated alkaline phosphatase (P = 0.013) and lactate dehydrogenase (P = 0.001) significantly impaired overall survival. In the pooled analysis, SCOS was diagnosed at the median age of 17 years and affected both sexes almost equally. The median follow-up duration was 19.5 months. In the pooled analysis cases, the 5-year overall survival rate was 38.6%, and 36.4% of patients survived 10 years. However, an increasing trend was detected, indicating recent improvements in management. The surgical margin status (P = 0.024) and metastases (P = 0.008) significantly impaired overall survival, and the response to chemotherapy was related to disease-free survival (P = 0.012). LR and MD were significantly correlated (P = 0.002) and could be observed after 5 years of follow-up. LR was significantly dependent on response to chemotherapy (P = 0.020). The development of MD seemed to be affected by response to chemotherapy (P = 0.060). Correlations between imaging features and prognosis were not detected. Conclusions This study suggested that positive margins, poor response to chemotherapy and MD are negative prognostic factors for SCOS, implied the potential role of laboratory examinations in the survival prediction and supported the need for prolonged or more intensive surveillance in patients with MD or LR. More well-documented literatures are encouraged to allow further confirmations.

Clinical and imaging features of 112 patients with irregular and flat bone osteosarcoma

“…Four cases of small-cell osteosarcomas were identified in the current study, including 2 in the ilium, 1 in the sacrum, and 1 in the lumbar spine. The osteoid matrix was observed in the soft tissues or the intramedullary compartment with the osteosarcoma, consistent with previous reports (28,29).…”

“…Moreover, T1-weighted images revealed soft tissue components of the tumor as isointense or hypointense, while on T2-weighted images, the signal intensity of soft tissue components of the tumor was classified as heterogeneous or hyperintense. The center of tumor masses was found to be accompanied by necrosis or hemorrhage, as well as an osteoid matrix (10,28). In the present investigation, image enhancement patterns were correlated with tumor characteristics, such as histological characteristics, intratumoral hemorrhage, angiogenesis, and necrotic or cystic components.…”

Functional interplay between long non-coding RNAs and the Wnt signaling cascade in osteosarcoma

“…Moreover, it is of great necessity to further validate the sensitivity and specificity of lncRNAs as biomarkers in clinical settings. Established serum markers, such as alkaline phosphatase (ALP) and lactate dehydrogenase (LDH), have been widely adopted to aid in diagnosing osteosarcoma for decades [ 185 , 186 ], and whether these Wnt-related lncRNAs have more advantages over these markers need to be further verified. Due to the easy accessibility and routine availability, the Wnt signaling-related lncRNAs with excellent diagnostic performance may be promising candidates in near future.…”

Preoperative Predictors of Early Mortality Risk in People with Osteosarcoma of the Extremities Treated with Standard Therapy

“…2 Unfortunately, the development of new drugs and other treatments has reached a plateau, and the prognosis of patients with osteosarcoma has not improved substantially since the 1970s. 3,4 Clinical characteristics can be used to identify high-risk patients and formulate corresponding treatment plans. However, patients with similar clinical features often have completely different prognoses, even after receiving the same standard treatment.…”