PT  - JOURNAL ARTICLE
AU  - Orlando De Jesus
AU  - José G Sánchez Jiménez
AU  - George Santiago Quiñones
AU  - Román Vélez
TI  - Malignant peripheral nerve sheath tumour transformation of histological benign vestibular schwannoma after stereotactic radiosurgery in patients without neurofibromatosis
AID  - 10.1136/bcr-2021-246445
DP  - 2021 Nov 01
TA  - BMJ Case Reports
PG  - e246445
VI  - 14
IP  - 11
4099  - http://casereports.bmj.com/content/14/11/e246445.short
4100  - http://casereports.bmj.com/content/14/11/e246445.full
SO  - BMJ Case Reports2021 Nov 01; 14
AB  - Stereotactic radiosurgery (SRS) poses a minimal but important risk for tumour transformation, typically occurring 8–10 years after the treatment. Malignant peripheral nerve sheath tumour (MPNST) is the most common tumour arising from a vestibular schwannoma treated with SRS, with only 14 cases previously reported. We present the fifteenth case and describe its evolution and clinical course. A 56-year-old man without a history of neurofibromatosis was diagnosed 9 years prior with a vestibular schwannoma. SRS to the residual tumour was given 3 months later. During the current hospitalisation, he was reoperated where histology confirmed a MPNST. All 15 MPNST cases were analysed, showing a 77% female predominance presenting a malignant transformation at a mean age of 51. The diagnosis was made at a mean time of 74 months after SRS. The mean survival time after diagnosis was 16 months. MPNST arising from benign vestibular schwannoma after SRS treatment is an uncommon but devastating complication.