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. |
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Copyright © The Author(s), 2024. Published by Science Publishing Group |
Malignant Peripheral Nerve Sheath Tumour (MPNST), NF: Neurofibromatosis, Haematoxylin, Eosin (H&E)
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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
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
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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}
}
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 -
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