Acta Scientific Medical Sciences (ASMS)(ISSN: 2582-0931)

Case Report Volume 7 Issue 5

Malignant Transformation of Neurofibroma with Emphasis on S100 - A Rare Case

Varshini Sindhiya Chanda1* and Neethu GV2

1Postgraduate, JJM Medical College, RGUHS, Karnataka, India
2Associate Professor, JJM Medical College, RGUHS, Karnataka, India

*Corresponding Author: Varshini Sindhiya Chanda, Postgraduate, JJM Medical College, RGUHS, Karnataka, India.

Received: March 14, 2023; Published: April 29, 2023

Abstract

Malignant peripheral nerve sheath tumor (MPNST) is a rare neoplasm arising from peripheral nerve and may arise from a pre-existing nerve sheath tumor in neurofibromatosis type 1 (NF 1). This is a rare case of 26 year old female presented with swelling on posterior aspect of left thigh since 1 month. Fine needle aspiration cytology (FNAC) suggestive of Malignant spindle cell tumour. Surgery was done and specimen sent for biopsy showed transformation from Neurofibroma to Malignant peripheral nerve sheath tumour (MPNST). Immunohistochemistry with S-100 protein was diffusely positive. Complete surgical resection with wide negative margins is the optimum management of high-grade MPNST and that early diagnosis and potential prevention of MPNST should be prioritized. The 5-year overall survival (OS) rate of all patients with MPNST was 69.5%. Whereas the precursors for MPNST like neurofibroma should be surgically resected and wide margins is not necessary, and preservation of neurological function is of paramount importance. Thus the management of neurofibroma differs from MPNST as the survival rate of the former is better than the latter.

 Keywords: Biopsy; Fine- Needle; Immunohistochemistry; Adult; Female

References

  1. Miettinen MM., et al. “Histopathologic evaluation of atypical neurofibromatous tumors and their transformation into malignant peripheral nerve sheath tumor in patients with neurofibromatosis 1-a consensus overview”. Human Pathology 67 (2017): 1-10.
  2. Yamanaka R and Hayano A. “Radiation-Induced Malignant Peripheral Nerve Sheath Tumors: A Systematic Review”. World Neurosurgery 105 (2017): 961-970.
  3. Evans DG., et al. “Malignant peripheral nerve sheath tumours in neurofibromatosis 1”. Journal of Medical Genetics 5 (2002): 311-314.
  4. Rosenbaum T and Wimmer K. “Neurofibromatosis type 1 (NF 1) and associated tumors”. Klinische Padiatrie 226 (2014): 309-315.
  5. Krol EM., et al. “Solitary Neurofibroma with Malignant Transformation: Case Report and Review Of Literature”. Connecticut Medicine4 (2015): 217-219.
  6. Reilly KM., et al. “Neurofibromatosis Type 1-Associated MPNST State of the Science: Outlining a Research Agenda for the Future”. Journal of the National Cancer Institute 8 (2017): 109.
  7. Imura Y., et al. “Clinical Outcomes and Prognostic Factors for Patients with Malignant Peripheral Nerve Sheath Tumour”. Sarcoma (2021): 8335290.

Citation

Citation: Varshini Sindhiya Chanda and Neethu GV. “Malignant Transformation of Neurofibroma with Emphasis on S100 - A Rare Case”.Acta Scientific Medical Sciences 7.5 (2023): 214-216.

Copyright

Copyright: © 2023 Varshini Sindhiya Chanda and Neethu GV. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.




Metrics

Acceptance rate30%
Acceptance to publication20-30 days
Impact Factor1.403

Indexed In





News and Events


Contact US