Primary central nervous system angiosarcoma: two case reports

James R Hackney,Hassan M Fathallah-Shaykh,Louis B Nabors,Joel K Cure,Cheryl Ann Palmer,Kristen O Riley

doi:10.1186/1752-1947-6-251

James R Hackney, Hassan M Fathallah-Shaykh + Show 4 more

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https://doi.org/10.1186/1752-1947-6-251

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Abstract

IntroductionPrimary angiosarcoma of the brain is extremely rare; only 15 cases have been reported in adults over the last 25 years.Case presentationsWe describe two cases of primary angiosarcoma of the brain that are well characterized by imaging, histopathology, and immunohistochemistry. Case 1: our first patient was a 35-year-old woman who developed exophthalmos. Subtotal resection of a left extra-axial retro-orbital mass was performed. Case 2: our second patient was a 47-year-old man who presented to our facility with acute visual loss, word-finding difficulty and subtle memory loss. A heterogeneously-enhancing left sphenoid wing mass was removed. We also review the literature aiming at developing a rational approach to diagnosis and treatment, given the rarity of this entity.ConclusionsGross total resection is the standard of care for primary angiosarcoma of the brain. Adjuvant radiation and chemotherapy are playing increasingly recognized roles in the therapy of these rare tumors.

Highlights

Primary angiosarcoma of the brain is extremely rare; only 15 cases have been reported in adults over the last 25 years.Case presentations: We describe two cases of primary angiosarcoma of the brain that are well characterized by imaging, histopathology, and immunohistochemistry
Gross total resection is the standard of care for primary angiosarcoma of the brain
Primary angiosarcoma of the central nervous system (CNS) is an extremely rare malignancy, with only approximately 15 cases reported in adults in the literature over the last 25 years

Summary

Conclusions

The rarity of primary angiosarcoma of the brain and the resulting paucity of relevant studies and case reports precludes a definitive judgment concerning optimal therapy. Regardless, suggestions derived from the few published cases, along with the short follow-up from our two more recent cases, may allow the preliminary formulation of a potentially effective treatment plan using modern, multimodality therapy. Surgery with or without tumor embolization, with the goal of gross total resection, remains the standard of care. Post-operative adjuvant radiation therapy may provide a benefit, in cases where gross total resection is not possible. Treatment results for paclitaxel in soft tissue angiosarcoma suggest that there may be a role for this chemotherapeutic agent in primary CNS angiosarcoma. All authors read and approved the final manuscript

Introduction

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