| Peer-Reviewed

Malignant Peripheral Nerve Sheath Tumour with Heterologous Differentiation: A Case Report

Received: 4 March 2022     Accepted: 28 March 2022     Published: 20 April 2022
Views:       Downloads:
Abstract

Malignant peripheral nerve sheath tumour is one of the rare soft tissue tumours in the younger age group. The tumour arises from peripheral nerves with a predominant lesion on the extremities or limbs, head and neck, and trunk. It can arise de novo also known as sporadic or in patients with neurofibromatosis type 1. It is an infiltrative, aggressive tumour with a high rate of recurrence and metastases. The microscopic examination of both NF1 gene-associated and sporadic MPNST are similar but different prognoses aided and abetted by other co-morbid factors or disease conditions. Histologic examination of the surgically resected specimen is the goal standard for diagnosis with an ancillary test comprising immunostaining of the tumour cells. This tumour has the propensity to undergo diverse differentiation towards osseous, chondroblastic, and rhabdomyoblastic cells. The rhabdomyoblastic differentiation is strongly associated with a poor prognosis. Thus, creating a diagnostic challenge for general pathologists and pathologists in the trainee. The specific cause of this tumour is not known but is strongly linked to Neurofibromatosis type 1 in a few groups of patients with multiple cutaneous plexiform neurofibromas which are not concordant with the index case. We, therefore, present a sporadic subcutaneous MPNST with heterologous differentiation comprising rhabdmyoblastic cells, chondroblast, and osseous in a 15-year-old woman.

Published in American Journal of Laboratory Medicine (Volume 7, Issue 2)
DOI 10.11648/j.ajlm.20220702.13
Page(s) 32-36
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2022. Published by Science Publishing Group

Keywords

Malignant Peripheral Nerve Sheath Tumour (MPNST), NF: Neurofibromatosis, Haematoxylin, Eosin (H&E)

References
[1] Masood Hasan Khan, Kausar J Khwaja, Nasir Ahmed Salati, Minhaj Anwar. Malignant Peripheral nerve sheath tumor of the mandible in a young female - A case Report. Annals of Dental Specialty. 2014; 2 (4): 155-157.
[2] Ruay-Sheng Lai, Shong-Ling Lin, Shu-Shong Hsu, Min-Ting Wu. Intrathoracic paraspinal Malignant peripheral nerve sheath tumour. J Chin Med Assoc. 2006; 69 (1): 37-41.
[3] Neurofibromatosis. Conference statement. National Institutes of Health Development Conference. Archives of Neurology. 1988; 45 (5): pp. 575–578.
[4] Guler Berkiten, Muhlis Bal, Yavuz Atar, Ziya Salturk, Onder Dogan, Caglar Cakir. Malignant Peripheral nerve sheath tumor of the neck. Otolaryngology Online Journal. 2014; 4 (1): 1-7.
[5] Ozmen Ozt¨urk, and Alper Tutkun. A Case Report of a Malignant Peripheral Nerve Sheath Tumor of the Oral Cavity in Neurofibromatosis Type 1. Case Reports in Otolaryngology. Hindawi Publishing Corporation. 2012. 1-3.
[6] Shilpa Patel, Jigna Pathak, Kamlesh Dekate, and Neeta Mohanty. Malignant peripheral nerve sheath tumour (MPNST) of the mandible: solving the perplexity. BMJ Case Rep. 2015; 2015: bcr2014207790.
[7] Jude-Kennedy C Emejulu, Okechukwu H Ekwunife, Titus Og Chukwuanukwu, Ikechukwu V Okpalike, Kodilinye O Izuora, Ofodile C Ekweogwu. Malignant Peripheral Nerve Sheath Tumour in a 10-year old. Journal of Clinical and Applied Neurosciences. 2017; 2 (1): 36-41.
[8] W B Laskin, S W Weiss, G L Bratthauer. Epithelioid variant of malignant peripheral nerve sheath tumor (malignant epithelioid schwannoma). Am J Surg Pathol. 1991; 15 (12): 1136-45.
[9] James E Bates, Carl R Peterson, Sughosh Dhakal, Ellen J Giampoli, Louis S Constine Malignant peripheral nerve sheath tumors (MPNST): A SEER Analysis of incidence across the age spectrum and therapeutic interventions in the pediatric population. Pedtiatr Blood Cancer 2014; 61: 1955-1960.
[10] Widemann BC. Current status of sporadic and neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors. Curr Oncol Rep. 2009; 11: 322–328.
[11] Christopher J Stasik, Ossama Tawfik. Malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation (Malignant Triton Tumor). Arch Pathol Lab Med. 2006; 130: 1878-1881.
[12] Fazil, Marickar, Y. M, Betty Abraham. Malignant peripheral nerve sheath tumour – A long story: A case report. International Journal of Surgery Case Reports. 2020; volume 77: 618–623.
[13] Han-Ju Chen, Huan-Sen Chen, Yen-Liang Chang, Yi-Yiing Wu. Malignant peripheral nerve sheath tumor of the neck: Transformation from a recurrent neurofibroma in a patient without neurofibromatosis. TZU CHI MED J; 2010 Dec; Vol 22 (4): 195-199.
[14] Kamran SC, Howard SA, Shinagare AB, Krajewski KM, Jagannathan JP, Hornick JL et al Malignant peripheral nerve sheath tumors: prognostic impact of rhabdomyoblastic differentiation (malignant triton tumors), neurofibromatosis 1 status and location. Eur J Surg Oncol. 2013; 39 (1): 46-52.
Cite This Article
  • APA Style

    Rasheed Mumini Wemimo, Afolayan Enoch Abiodun, Jatto Hamza Ibrahim, Adekunle Adebayo Ayoade, Atiku Hafiz, et al. (2022). Malignant Peripheral Nerve Sheath Tumour with Heterologous Differentiation: A Case Report. American Journal of Laboratory Medicine, 7(2), 32-36. https://doi.org/10.11648/j.ajlm.20220702.13

    Copy | Download

    ACS Style

    Rasheed Mumini Wemimo; Afolayan Enoch Abiodun; Jatto Hamza Ibrahim; Adekunle Adebayo Ayoade; Atiku Hafiz, et al. Malignant Peripheral Nerve Sheath Tumour with Heterologous Differentiation: A Case Report. Am. J. Lab. Med. 2022, 7(2), 32-36. doi: 10.11648/j.ajlm.20220702.13

    Copy | Download

    AMA Style

    Rasheed Mumini Wemimo, Afolayan Enoch Abiodun, Jatto Hamza Ibrahim, Adekunle Adebayo Ayoade, Atiku Hafiz, et al. Malignant Peripheral Nerve Sheath Tumour with Heterologous Differentiation: A Case Report. Am J Lab Med. 2022;7(2):32-36. doi: 10.11648/j.ajlm.20220702.13

    Copy | Download

  • @article{10.11648/j.ajlm.20220702.13,
      author = {Rasheed Mumini Wemimo and Afolayan Enoch Abiodun and Jatto Hamza Ibrahim and Adekunle Adebayo Ayoade and Atiku Hafiz and Odetayo Omolara Foluke},
      title = {Malignant Peripheral Nerve Sheath Tumour with Heterologous Differentiation: A Case Report},
      journal = {American Journal of Laboratory Medicine},
      volume = {7},
      number = {2},
      pages = {32-36},
      doi = {10.11648/j.ajlm.20220702.13},
      url = {https://doi.org/10.11648/j.ajlm.20220702.13},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajlm.20220702.13},
      abstract = {Malignant peripheral nerve sheath tumour is one of the rare soft tissue tumours in the younger age group. The tumour arises from peripheral nerves with a predominant lesion on the extremities or limbs, head and neck, and trunk. It can arise de novo also known as sporadic or in patients with neurofibromatosis type 1. It is an infiltrative, aggressive tumour with a high rate of recurrence and metastases. The microscopic examination of both NF1 gene-associated and sporadic MPNST are similar but different prognoses aided and abetted by other co-morbid factors or disease conditions. Histologic examination of the surgically resected specimen is the goal standard for diagnosis with an ancillary test comprising immunostaining of the tumour cells. This tumour has the propensity to undergo diverse differentiation towards osseous, chondroblastic, and rhabdomyoblastic cells. The rhabdomyoblastic differentiation is strongly associated with a poor prognosis. Thus, creating a diagnostic challenge for general pathologists and pathologists in the trainee. The specific cause of this tumour is not known but is strongly linked to Neurofibromatosis type 1 in a few groups of patients with multiple cutaneous plexiform neurofibromas which are not concordant with the index case. We, therefore, present a sporadic subcutaneous MPNST with heterologous differentiation comprising rhabdmyoblastic cells, chondroblast, and osseous in a 15-year-old woman.},
     year = {2022}
    }
    

    Copy | Download

  • TY  - JOUR
    T1  - Malignant Peripheral Nerve Sheath Tumour with Heterologous Differentiation: A Case Report
    AU  - Rasheed Mumini Wemimo
    AU  - Afolayan Enoch Abiodun
    AU  - Jatto Hamza Ibrahim
    AU  - Adekunle Adebayo Ayoade
    AU  - Atiku Hafiz
    AU  - Odetayo Omolara Foluke
    Y1  - 2022/04/20
    PY  - 2022
    N1  - https://doi.org/10.11648/j.ajlm.20220702.13
    DO  - 10.11648/j.ajlm.20220702.13
    T2  - American Journal of Laboratory Medicine
    JF  - American Journal of Laboratory Medicine
    JO  - American Journal of Laboratory Medicine
    SP  - 32
    EP  - 36
    PB  - Science Publishing Group
    SN  - 2575-386X
    UR  - https://doi.org/10.11648/j.ajlm.20220702.13
    AB  - Malignant peripheral nerve sheath tumour is one of the rare soft tissue tumours in the younger age group. The tumour arises from peripheral nerves with a predominant lesion on the extremities or limbs, head and neck, and trunk. It can arise de novo also known as sporadic or in patients with neurofibromatosis type 1. It is an infiltrative, aggressive tumour with a high rate of recurrence and metastases. The microscopic examination of both NF1 gene-associated and sporadic MPNST are similar but different prognoses aided and abetted by other co-morbid factors or disease conditions. Histologic examination of the surgically resected specimen is the goal standard for diagnosis with an ancillary test comprising immunostaining of the tumour cells. This tumour has the propensity to undergo diverse differentiation towards osseous, chondroblastic, and rhabdomyoblastic cells. The rhabdomyoblastic differentiation is strongly associated with a poor prognosis. Thus, creating a diagnostic challenge for general pathologists and pathologists in the trainee. The specific cause of this tumour is not known but is strongly linked to Neurofibromatosis type 1 in a few groups of patients with multiple cutaneous plexiform neurofibromas which are not concordant with the index case. We, therefore, present a sporadic subcutaneous MPNST with heterologous differentiation comprising rhabdmyoblastic cells, chondroblast, and osseous in a 15-year-old woman.
    VL  - 7
    IS  - 2
    ER  - 

    Copy | Download

Author Information
  • Department of Anatomic Pathology, Federal Medical Centre, Birnin Kudu, Nigeria

  • Department of Pathology, University of Ilorin Teaching Hospital, Ilorin, Nigeria

  • Department of Surgery, Federal Medical Centre, Birnin Kudu, Nigeria

  • Department of Morbid Anatomy and Histopathology, Ladoke Akintola University of Technology, Ogbomosho, Nigeria

  • Department of Radiology, Federal Medical Centre, Birnin Kudu, Nigeria

  • Department of Radiology, Olabisi Onabanjo University Teaching Hospital, Sagamu, Nigeria

  • Sections