Hybrid nerve sheath tumour, a conglomerate of separate entities

Hybrid nerve sheath tumours (HNSTs) are benign tumours showing combined features of more than one peripheral nerve sheath tumour (PNST) entities. Most literature highlight combination of two entities. Only few reported cases involve all three components of PNST. Herein, we report a case of 33-year-o...

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Bibliographic Details
Main Authors: Omar, Noorjehan, Mohd Dusa, Noraini, Bakhrin, Ikmal Hisyam, Omar, Noor Azzizah, Vasavan, Yabitha
Format: Article
Language:English
Published: Universiti Putra Malaysia 2021
Online Access:http://psasir.upm.edu.my/id/eprint/97577/
http://psasir.upm.edu.my/id/eprint/97577/1/2021120708275619_MJMHS_0768.pdf
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Summary:Hybrid nerve sheath tumours (HNSTs) are benign tumours showing combined features of more than one peripheral nerve sheath tumour (PNST) entities. Most literature highlight combination of two entities. Only few reported cases involve all three components of PNST. Herein, we report a case of 33-year-old Malay lady, with left proximal middle finger soft tissue swelling for three months. Microscopically, the tumour is well-circumscribed, containing all three components of PNST, with each displaying its typical morphology and immunohistochemical staining pattern. This case suggests that PNSTs may be more closely related than what was earlier believed. However, whether HNSTs are part of the PNSTs spectrum with tumour syndromes association or a distinct entity is still a continuous debate. We also highlight the possibilities of recurrence and risk of malignant transformation in HNSTs. The exact pathogenesis, potential for recurrence and malignant transformation criteria for HNSTs are still vague due to their extreme rarity.