Brain Tumor Res Treat.  2023 Apr;11(2):103-113. 10.14791/btrt.2023.0003.

Intracranial Metastasis of Extracranial Chondrosarcoma: Systematic Review With Illustrative Case

• Mackel CE
• Rosenberg H
• Varma H
• Uhlmann EJ
• Vega RA
• Alterman RL

• KMID: 2542082
• DOI: http://doi.org/10.14791/btrt.2023.0003

Abstract

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.

Keyword

Cerebral; Chondrosarcoma; Maffucci syndrome; Metastases

Figure

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  Fig. 1 Axial fat-suppressed post-gadolinium of the calf. Soft tissue hemangioma (arrow) in the lateral head of the left gastrocnemius in the presence of enchondromas consistent with Maffucci syndrome.

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  Fig. 2 Imaging of the left distal femur over time, demonstrating a soft tissue mass with cortical destruction. A: Left femoral enchondroma on axial CT 2 years prior to malignant conversion with avidity of 2.6 on associated FDG-PET. B: Same femoral enchondroma on axial CT at time of dedifferentiation demonstrating cortical thinning and breakthrough. C: FDG-PET avidity in femoral chondrosarcoma increased to 17.6. Remainder of body scan negative.

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  Fig. 3 Axial T1 post-gadolinium MRI demonstrating heterogeneously enhancing, intra-axial right parietal brain metastasis and surrounding vasogenic edema 4 months after primary diagnosis.

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  Fig. 4 Histochemistry of malignant tissue from femoral (A and B) and cerebral (C) tumor sites (hematoxylin and eosin stain). A: At low power (×10), high-grade chondrosarcoma on the right with areas of tumor necrosis on the left. B: Higher power (×20) highlights striking nuclear pleomorphism, prominent nucleoli, and mitotic activity. C: Malignant neoplasm in the brain (×20) with similar morphological features as femoral biopsy and nuclear pleomorphism, prominent nucleoli, abundant mitoses.

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  Fig. 5 PRISMA flow chart of search results for articles.

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  Fig. 6 Survival following extracranial chondrosarcoma metastatic to brain. A: Kaplan-Meier survival graph for chondrosarcomas following brain metastasis. B: Comparison of survival between patients with surgical resection versus non-surgical management of brain metastases with significantly different survival outcomes on log-rank test (p=0.024).

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