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Case Reports: Rare disease
Bulky malignant peripheral nerve sheath tumour of the left thigh in a pregnant woman presenting with a pathological fracture of the proximal femur
  1. Diogo Catelas1,2,
  2. Duarte Sousa1,
  3. Ana Patrícia Rodrigues3 and
  4. Pedro Cardoso1,2
  1. 1Department of Orthopedic Surgery, Centro Hospitalar Universitario de Santo António, Porto, Portugal
  2. 2School of Medicine and Biomedical Sciences, University of Porto, Porto, Portugal
  3. 3Department of Surgical Pathology, Centro Hospitalar Universitário de Santo António, Porto, Portugal
  1. Correspondence to Professor Pedro Cardoso; pffcardoso{at}gmail.com

Abstract

Malignant peripheral nerve sheath tumour (MPNST) is an aggressive soft tissue sarcoma with a poor prognosis, affecting most commonly the extremities. The lungs constitute the most frequent location for distant metastases. Half of all MPNSTs arise in patients with neurofibromatosis type 1, while approximately 10% are radiation induced and the rest are sporadic.

The authors present a pregnant woman in her 40s with a sporadic MPNST of the lower limb and with lung metastases at diagnosis. Treatment consisted of interilioabdominal amputation, followed by adjuvant chemotherapy. Partial response and disease stabilisation were achieved with chemotherapy.

Surgical resection with negative margins is the only potentially curative therapy, while radiation therapy and chemotherapy might be useful in the neoadjuvant or adjuvant setting, but their advantage in survival is not demonstrated. In the reported case, chemotherapy permitted the achievement of partial response and stabilisation of the disease.

  • Cancer intervention
  • Orthopaedics
  • Orthopaedic and trauma surgery

https://doi.org/10.1136/bcr-2022-253070

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    Footnotes

    • Contributors DC and PC contributed to the study's conception and design. The first draft of the manuscript was written by DC with input from PC, APR and DS. The manuscript was then revised by PC. DC was responsible for the final edition. DC, DS, APR and PC read and approved the final manuscript.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.