Late relapse of malignant peripheral nerve sheath tumor after 18 years. Case report with differential diagnosis.

  • Csaba Biró Department of Pathology Institute of Oncology Vol. Elizabeth , Bratislava , Slovakia
  • Katarína Macháleková
  • Štefan Galbavý
  • Gabriel Bognár
  • Sarah Catharina Hubinská
  • Martin Kopáni
  • Karol Kajo
  • Dagmar Kalátová
Keywords: MPNS, differential diagnosis, immunohistochemistry, molecular genetics

Abstract

Malignant mesenchymal tumors represent a heterogeneous group of neoplasms. Diagnostics is based on a comprehensive clinical and pathological assessment that takes into account the results of imaging methods and molecular genetics. The exact diagnosis of these neoplasms is important for their subsequent therapeutic management. Authors describe a case of a 47-year-old female patient with poorly differentiated sarcoma in the left neck region which they, based on histomorphological features, a wide immunohistochemistry panel, and detailed history, diagnosed as a late recurrence of MPNST 18 years after the primary diagnosis. Tumors of the Ewing sarcoma/PNET, synovial sarcoma, which have similar histomorphological features, were excluded in the process of differential diagnostics. MPNST is characterized as a malignant tumor with unpredictable biological behavior, and as the case described above indicates, it may even return many years after the primary diagnosis. 

Author Biography

Csaba Biró, Department of Pathology Institute of Oncology Vol. Elizabeth , Bratislava , Slovakia
Department of Pathology Institute of Oncology Vol. Elizabeth , Bratislava , Slovakiadoctor

References

1. Guo A, Liu A, Wei L, Xin Song. Malignant peripheral nerve sheath tumors: Differentiation patterns and immunohistochemical features – a mini review and our new findings. J Cancer. 2012 :3, 303-9.
2. Anderson JL, Denny CT, Tap WD, Federman N. Pediatric sarcomas: translating molecular pathogenesis to disease to novel therapeutic possibilities. Pediatr Res 2012;72: 112-21
3. Miettinen M. Modern soft tissue pathology : tumors and non-neoplastic conditions, Cambridge University Press, 2010, 703-716,
4. Stephen HO, Thomas DG, Lucas DR. Cluster analysis of immunohistochemical profiles in synovial sarcoma, malignant peripheral nerve sheath tumor, and Ewing sarcoma. Modern Pathology 2006:19; 659
5. Shimada STT, Nagasaka T, Kuroda M, et al. Nestin expression in malignant peripheral nerve sheath tumor (MPNST) and other soft tissue tumors. Mod Pathol 2005;1:22A.
6. Sandberg AA, Stone JF. The Genetics and Molecular Biology of Neural Tumors. Springer Science & Business Media, 2008
Published
2016-11-05
Section
Case Report