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Journal of Hand Surgery (Eur) 49(6)
outcome in children compared to adults, with a
5-year survival of 52% in NF1 MPNST and 76% in
non-NF1 MPNST (Martin et al., 2020a). A metaanalysis
of non-rhabdomyoblastic soft tissue sarcoma
(NRSTS) in children showed an overall 5-year
survival of 60%, 45% in non-NF1 MPNST and only
11% in NF1-associated MPNST (Ferrari et al., 2011).
Summary
To conclude, peripheral nerve tumours encompass a
diverse group, including benign schwannomas and
neurofibromas, as well as the rare occurrence of
MPNST. Malignant transformation is unlikely for
schwannomas but more probable for neurofibromas,
especially in patients with NF1. An algorithm has
been presented to guide management decisions.
While MRI helps to assess the relationship between
a swelling and nerves, it is not definitive for tissue
diagnosis. Increasing size, pain and neurological deficit
may signal malignant change. TruCut needle
biopsy is useful in clarifying diagnosis, but the possibility
of sampling error should be considered for
neurofibromas. Excision biopsy is commonly recommended
for benign tumours to relieve symptoms, but
surgery should aim to preserve nerve function.
Individual cases vary and decisions should be based
on careful clinical assessment and specific patient
factors.
Malignant tumours of nerves are rare and require
multimodal treatment in tertiary care hospitals.
Complete surgical excision (with clear margins) is
the only curative treatment. The prognosis, in spite
of surgery, chemotherapy and radiotherapy, still
remains poor and is worse in the presence of NF1
and post-irradiation tumours. Emerging treatment
using biologics and other newer agents are ongoing
and may improve the final outcome in the future.
Early detection and a comprehensive approach are
important for managing these complex conditions.
Acknowledgments TH acknowledges the audit of biopsies
of suspected peripheral nerve tumours and of outcomes
of excision of benign tumours carried out by Iain
McGraw, Consultant Hand Surgeon, Glasgow Royal
Infirmary, and Roberto Alho, Specialist Trainee in
Orthopaedic Surgery, West of Scotland. BPT acknowledges
the help in the audit of cases of malignant nerve tumours
by Sreekant Raveendran, Associate Professor, Paul Brand
Centre for Hand Surgery, Christian Medical College
Hospital, Vellore.
Declaration of conflicting interests The authors
declared no potential conflicts of interest with respect to
the research, authorship, and/or publication of this article.
Funding The authors received no financial support for the
research, authorship, and/or publication of this article.
ORCID iD Tim Hems https://orcid.org/0000-0001-76762819
Supplemental
material Supplemental material for this
article is available online.
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