MALIGNANT PERIPHERAL NERVE SHEATH TUMORS SPINE METASTASES - DOCUMENT REVIEW A CASE REPORTS
Main Article Content
Abstract
Malignant Peripheral Nerve Sheath Tumors (MPNSTs) are rare, high-grade soft tissue sarcomas
of extranodal origin and often metastasize early. The World Health Organization has coined the
term MPNST to replace previously heterogeneous and often confusing terms, such as malignant
schwannoma, malignant neurilemmoma, neurogenic sarcoma and neurofibrosarcoma. MPNSTs
arise from the sheaths of peripheral nerve fibers, either from Schwann cells or from neural crestderived pluripotent cells. Schwann cells are believed to be responsible for the formation of benign as
well as malignant tumors in the nerve fiber sheath. MPNST can appear in many places in the body,
but in the lungs is extremely rare. Imaging is usually nonspecific. The diagnosis was confirmed
by immunohistochemistry. We report a case of MPNST in the lung, with spinal metastases; was
confirmed by biopsy of metastatic lesions and immunohistochemistry at the National Lung Hospital.
Article Details
Keywords
Malignant Peripheral Nerve Sheath Tumor, malignant neurilemmoma, malignants chwannoma, neurofibrosarcoma.
References
Classification: What’s New in Soft Tissue Tumor
Pathology? Am J Surg Pathol. 2021 Jan; 45(1):e1-
e23. doi: 10.1097/PAS.0000000000001552.
PMID: 32796172.
[2] Gupta G, Maniker A, Malignant peripheral
nerve sheath tumors. Neurosurg Focus. 2007 Jun
15;22(6):E12. doi: 10.3171/foc.2007.22.6.13.
PMID: 17613203
[3] Martin E, Flucke UE, Coert JH et al., Treatment
of malignant peripheral nerve sheath tumors in
pediatric NF1 disease. Childs Nerv Syst. 2020
Oct; 36(10):2453-2462. doi: 10.1007/s00381-
020-04687-3. Epub 2020 Jun 3.PMID: 32494969.
[4] Walczak A, Radek M, Majsterek I, The Role of
ER Stress-Related Phenomena in the Biology
of Malignant Peripheral Nerve Sheath Tumors.
Int J Mol Sci. 2021 Aug 30;22(17):9405. doi:
10.3390/ijms22179405.PMID: 34502310.
[5] Gupta G, Mammis A, Maniker A, Malignant
peripheral nerve sheath tumors. Neurosurg Clin
N Am. 2008 Oct;19(4):533-43, v. doi: 10.1016/j.
nec.2008.07.004.PMID: 19010279.
[6] Pendleton C, Howe BM, Spinner RJ, Percutaneous
image-guided biopsy in malignant peripheral
nerve sheath tumors. Acta Neurochir (Wien).
2021 Feb;163(2):515-519. doi: 10.1007/s00701-
020-04556-7. Epub 2020 Sep 8.PMID: 32901394.
[7] Noreña-Rengifo BD, Cadavid-Alvarez LM,
Malignant peripheral nerve sheath tumor in a child.
Radiol Case Rep. 2020 Nov 12;16(1):145-151.
doi: 10.1016/j.radcr.2020.10.028. eCollection
2021 Jan.PMID: 33224401.
[8] Thway K, Fisher C, Malignant peripheral
nerve sheath tumor: pathology and genetics.
Ann Diagn Pathol. 2014 Apr;18(2):109-16. doi:
10.1016/j.anndiagpath.2013.10.007. Epub 2013
Nov 23.PMID: 24418643
[9] Everson MC, Pendleton C, Jack MM et al., Sporadic
Malignant Perineurioma: A Rare Diagnosis Among
Malignant Peripheral Nerve Sheath Tumors.
World Neurosurg. 2021 May;149:e36-e41. doi:
10.1016/j.wneu.2021.02.099. Epub 2021 Feb
27.PMID: 33647483
[10] Bai M, Govindaraj V, Chauhan AS et al., Metastatic
malignant peripheral nerve sheath tumor. A
diagnostic surprise. Monaldi Arch Chest Dis. 2021
May 6;91(2). doi: 10.4081/monaldi.2021.1658.
PMID: 34256542.