Anaplastic pleomorphic xanthoastrocytoma with disseminated growth pattern at the time of diagnosis as well as after treatment: case report and review of literature

Meng Zhu,Weicheng Yao,Shizhu Yu,Chen Zhang,Jian Sun,Leilei Wang,Yugong Feng,Xuejun Yang,Cuiyun Sun,Kai Zhao

doi:10.1186/s41016-017-0087-2

Abstract

BackgroundPleomorphic xanthoastrocytoma (PXA) is usually considered a relatively benign and localized entity. However, cases of PXA with anaplastic features have been reported in recent years. Anaplastic pleomorphic xanthoastrocytoma has been added to the 2016 WHO classification of CNS tumors as a distinct entity.Case presentationWe describe a rare case of PXA with dissemination, both at the time of diagnosis and after treatment. The 20-year-old male presented with signs of high intracranial pressure and sudden-onset transient seizures. Imaging examinations showed diffuse lesions widely distributed in the left hemisphere, and on histopathological examination, he was diagnosed with anaplastic PXA. The patient underwent surgical treatment and adjuvant concurrent chemoradiation. Follow-up MRI revealed early recurrence and distant spread of the tumor.ConclusionsAnaplastic PXA usually has unique characteristics, including dissemination, early recurrence, and chemoresistance. A strategy based on early diagnosis and aggressive treatment is warranted. However, sufficiently powered studies are required to generate evidence-based guidelines.

Highlights

Pleomorphic xanthoastrocytoma (PXA) is usually considered a relatively benign and localized entity
We suggest that PXA with dissemination, early recurrence, or resistance to adjuvant therapy should be treated as anaplastic PXA
Consistent with the results reported by Marucci et al [17], we raise doubts about the benefits of TMZ in the treatment of anaplastic PXA

Summary

Conclusions

We report a case of anaplastic PXA which is difficult to manage. The clinical course of PXA has been acknowledged as being benign, the probability of dissemination, early recurrence, and malignant progression should be noted. Standardized treatment strategy for anaplastic PXA has not been established owing to the rarity of the disease. Early diagnosis and aggressive treatment appears to be the best strategy in these patients

Background

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Discussion