Download PDF
CC BY-NC-ND 4.0 · Indian J Med Paediatr Oncol 2017; 38(03): 387-390
DOI: 10.4103/ijmpo.ijmpo_113_17
Case Report

Metastatic Epithelioid Malignant Peripheral Nerve Sheath Tumor in a Known Case of Neurofibromatosis‑1, Cytomorphological Appearance, and Critical Analysis of Immunohistochemistry

Rakesh Kumar Gupta
Department of Pathology, GB Pant Institute of Postgraduate Medical Education and Research, New Delhi, India
,
Ravindra Kumar Saran
Department of Pathology, GB Pant Institute of Postgraduate Medical Education and Research, New Delhi, India
,
Deepak Ghuliani
Department of Surgery, Maulana Azad Medical College, New Delhi, India
,
Lalit Garg
Department of Radiodiagnosis, GB Pant Institute of Postgraduate Medical Education and Research, New Delhi, India
,
Abhijit Das
Department of Pathology, Hindu Rao Hospital, North Delhi Municipal Corporation, New Delhi, India
› Author Affiliations Financial support and sponsorship Nil.
› Further Information
  PDF Download Permissions and Reprints

Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue tumors commonly arising from nerve roots in the extremities with a predilection in neurofibromatosis (NF) patients. MPNSTs are known to behave aggressively, with worse prognosis in nonresectable cases, and a 5-year survival of about 52% and 15% in resectable and unresectable cases, respectively. Although cytological diagnosis in a known case of NF-1 is possible, however, in the absence of any associated syndrome in a sporadic case, it is very challenging to accurately diagnose this tumor. Till date, to the best of our knowledge, only three cases of epithelioid MPNST correctly diagnosed on cytological examination have been described. We are presenting another case of epithelioid MPNST in a 40-year-old patient with stigmata of NF-1 since childhood, diagnosed on fine-needle aspiration cytology from the left flank mass with subsequent histopathology from left inguinal lymph node metastasis.

Keywords

Cytology - epithelioid - fine-needle aspiration cytology - malignant peripheral nerve sheath tumors - neurofibromatosis-1


Publication History

Article published online:
04 July 2021

© 2017. Indian Society of Medical and Paediatric Oncology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/.)

Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India

 

  • References

  • 1 Weiss SW, Goldblum JR, Enzinger FM, editors. Enzinger and Weiss's Soft Tissue Tumors. St. Louis: Mosby Inc.; 2008.
  • 2 Ducatman BS, Scheithauer BW, Piepgras DG, Reiman HM, Ilstrup DM. Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases. Cancer 1986;57:2006-21.
  • 3 Rodriguez FJ, Folpe AL, Giannini C, Perry A. Pathology of peripheral nerve sheath tumors: Diagnostic overview and update on selected diagnostic problems. Acta Neuropathol 2012;123:295-319.
  • 4 Dodd LG, Scully S, Layfield LJ. Fine-needle aspiration of epithelioid malignant peripheral nerve sheath tumor (epithelioid malignant schwannoma). Diagn Cytopathol 1997;17:200-4.
  • 5 Jiwani S, Gokden M, Lindberg M, Ali S, Jeffus S. Fine-needle aspiration cytology of epithelioid malignant peripheral nerve sheath tumor: A case report and review of the literature. Diagn Cytopathol 2016;44:226-31.
  • 6 Kourea HP, Bilsky MH, Leung DH, Lewis JJ, Woodruff JM. Subdiaphragmatic and intrathoracic paraspinal malignant peripheral nerve sheath tumors: A clinicopathologic study of 25 patients and 26 tumors. Cancer 1998;82:2191-203.
  • 7 Lodding P, Kindblom LG, Angervall L. Epithelioid malignant schwannoma. A study of 14 cases. Virchows Arch A Pathol Anat Histopathol 1986;409:433-51.
  • 8 Laskin WB, Weiss SW, Bratthauer GL. Epithelioid variant of malignant peripheral nerve sheath tumor (malignant epithelioid schwannoma). Am J Surg Pathol 1991;15:1136-45.
  • 9 Klijanienko J, Caillaud JM, Lagacé R, Vielh P. Cytohistologic correlations of 24 malignant peripheral nerve sheath tumor (MPNST) in 17 patients: The institut curie experience. Diagn Cytopathol 2002;27:103-8.
  • 10 Wong WW, Hirose T, Scheithauer BW, Schild SE, Gunderson LL. Malignant peripheral nerve sheath tumor: Analysis of treatment outcome. Int J Radiat Oncol Biol Phys 1998;42:351-60.
  • 11 Stark AM, Buhl R, Hugo HH, Mehdorn HM. Malignant peripheral nerve sheath tumours – Report of 8 cases and review of the literature. Acta Neurochir (Wien) 2001;143:357-63.