An Unknown Cause of Upper Gastrointestinal Bleeding: Metastatic Extraskeletal Chondrosarcoma

Damas, Oriana M.; Betesh, Andrea; Deshpande, Amar

doi:10.14309/00000434-201210001-00884

Cited by 1 publication

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“…The presence of cartilaginous matrix on histopathology is a critical diagnostic marker [ 1 ]. We excluded 6 cases of “extraskeletal myxoid chondrosarcomas” from analysis [ 74 75 76 77 78 79 ] because they lack cartilaginous differentiation and are not considered to be true “chondrosarcomas” [ 80 ]. Although chondrosarcomas do not have a specific molecular marker, somatic mutations of IDH1/IDH2 help differentiate chondromas/chondrosarcomas from other mesenchymal bone tumors.…”

Section: Discussionmentioning

confidence: 99%

Intracranial Metastasis of Extracranial Chondrosarcoma: Systematic Review With Illustrative Case

Mackel

Rosenberg

Varma

et al. 2023

Brain Tumor Res Treat

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Background Cerebral chondrosarcoma metastases are rare and aggressive neoplasms. The rarity of presentation has precluded rigorous analysis of diagnosis, risk factors, treatment, and survival. We analyzed every reported case through exhaustive literature review. We further present the first case with Maffucci syndrome. Methods Three databases, PubMed, Embase, and Google Scholar, and crossed references were queried for cerebral chondrosarcoma metastases. Extracted variables included demographics, risk factors, tumor characteristics, interventions, and outcomes. Univariate and multivariate analyses were performed. Results Fifty-six patients were included from 1,489 literature results. The average age at brain metastasis was 46.6±17.6 years and occurred at a median of 24±2.8 months from primary diagnosis. Primary tumor histology (dedifferentiated 5.0±1.5 months, mesenchymal 24±3.0 months, conventional 41±7.4 months, p <0.05) and grade (low grade 54±16.7 months vs. high-grade 10±6.4 months, p <0.001) correlated with time interval until brain metastasis. A multiple enchondromatosis syndrome occurred in 13.2% of cases. At time of brain metastases diagnosis, extracranial metastases were identified in 76.2% of cases. Median survival after the development of brain metastasis was 2.0±0.78 months with a 1-year survival of 10.0%. On regression analysis, surgery reduced brain metastasis mortality risk and radiation trended towards reduced mortality risk (surgery: hazard ratio [HR] 0.22, 95% confidence interval [CI] 0.064–0.763, p =0.017; radiation: HR 0.31, 95% CI 0.091–1.072, p =0.064). Conclusion We present a systematic review of cerebral chondrosarcoma metastases. Primary tumor histology and grade correlate with time until cerebral metastasis. Following cerebral metastasis, these tumors have poor prognosis and modestly benefit from surgery.

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