Neurological Surgery No Shinkei Geka Volume 44, Issue 8 (August 2016)

Radiation-Induced Malignant Peripheral Nerve Sheath Tumor of the High Cervical Spine Tatsuya MAEGAWA 1 , Motohiro HIRASAWA 1 , Atsushi SASAHARA 1 , Shigeru TANI 1 , Shinji HAGIWARA 1 , Hirokazu KOSEKI 1 , Chika YOSHIMURA 1 , Yuichi TAKAHASHI 1 , Asami KIKUCHI 1 , Hidetoshi KASUYA 1 1Department of Neurosurgery, Tokyo Women's Medical University Medical Center East Keyword: malignant peripheral nerve sheath tumor , radiation-induced , spine pp.691-698
Published Date 2016/8/10
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 Case:A 30-year-old woman presented with posterior cervical pain and left-sided omalgia. The patient had a history of non-Hodgkin's lymphoma for which she had received prophylactic whole-brain irradiation(including at the upper cervical level)17 years previously. A magnetic resonance imaging(MRI)scan obtained 1 month previously showed an intradural extramedullary mass lesion at the left C1/2 level. We initially considered the tumor to be a benign schwannoma, but the patient subsequently developed left hemiparesis and was consequently admitted 2 days after her first visit. A second MRI scan showed that the tumor had progressed markedly. Hence, the patient underwent emergency surgical excision of the tumor. However, the tumor could only be partially removed because it had strongly adhered to the ventral aspect of the spinal cord. The tumor was pathologically diagnosed as a malignant peripheral nerve sheath tumor(MPNST). The residual tumor was subjected to local irradiation and surgery, but the treatment was unsuccessful, and the patient died on the 91st day of her illness.

 Conclusion:We report a case of radiation-induced high cervical MPNST arising from a benign schwannoma. All 9 previously reported cases of radiation-induced spinal MPNST were reviewed. Intraspinal MPNST of the high cervical region are extremely rare and are associated with a very poor prognosis. The 5-year survival rate of such tumors is markedly worse than that of other types of MPNST, and no standard treatment has been established for this condition.

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Neurological Surgery 脳神経外科
44巻8号 (2016年8月)
電子版ISSN 1882-1251 印刷版ISSN 0301-2603 医学書院