Metastatic epithelioid malignant peripheral nerve sheath tumor in a known case of neurofibromatosis-1, cytomorphological appearance, and critical analysis of immunohistochemistry

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 ab...

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Main Authors: Rakesh Kumar Gupta, Ravindra Kumar Saran, Deepak Ghuliani, Lalit Garg, Abhijit Das
Format: Article
Language:English
Published: Thieme Medical and Scientific Publishers Pvt. Ltd. 2017-01-01
Series:Indian Journal of Medical and Paediatric Oncology
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Online Access:http://www.ijmpo.org/article.asp?issn=0971-5851;year=2017;volume=38;issue=3;spage=387;epage=390;aulast=Gupta
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author Rakesh Kumar Gupta
Ravindra Kumar Saran
Deepak Ghuliani
Lalit Garg
Abhijit Das
author_facet Rakesh Kumar Gupta
Ravindra Kumar Saran
Deepak Ghuliani
Lalit Garg
Abhijit Das
author_sort Rakesh Kumar Gupta
collection DOAJ
description 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.
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series Indian Journal of Medical and Paediatric Oncology
spelling doaj-art-2387d0fecfd94d359f0dec0ff6968e172025-08-20T03:09:44ZengThieme Medical and Scientific Publishers Pvt. Ltd.Indian Journal of Medical and Paediatric Oncology0971-58512017-01-0138338739010.4103/ijmpo.ijmpo_113_17Metastatic epithelioid malignant peripheral nerve sheath tumor in a known case of neurofibromatosis-1, cytomorphological appearance, and critical analysis of immunohistochemistryRakesh Kumar GuptaRavindra Kumar SaranDeepak GhulianiLalit GargAbhijit DasMalignant 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.http://www.ijmpo.org/article.asp?issn=0971-5851;year=2017;volume=38;issue=3;spage=387;epage=390;aulast=GuptaCytologyepithelioidfine-needle aspiration cytologymalignant peripheral nerve sheath tumorsneurofibromatosis-1
spellingShingle Rakesh Kumar Gupta
Ravindra Kumar Saran
Deepak Ghuliani
Lalit Garg
Abhijit Das
Metastatic epithelioid malignant peripheral nerve sheath tumor in a known case of neurofibromatosis-1, cytomorphological appearance, and critical analysis of immunohistochemistry
Indian Journal of Medical and Paediatric Oncology
Cytology
epithelioid
fine-needle aspiration cytology
malignant peripheral nerve sheath tumors
neurofibromatosis-1
title Metastatic epithelioid malignant peripheral nerve sheath tumor in a known case of neurofibromatosis-1, cytomorphological appearance, and critical analysis of immunohistochemistry
title_full Metastatic epithelioid malignant peripheral nerve sheath tumor in a known case of neurofibromatosis-1, cytomorphological appearance, and critical analysis of immunohistochemistry
title_fullStr Metastatic epithelioid malignant peripheral nerve sheath tumor in a known case of neurofibromatosis-1, cytomorphological appearance, and critical analysis of immunohistochemistry
title_full_unstemmed Metastatic epithelioid malignant peripheral nerve sheath tumor in a known case of neurofibromatosis-1, cytomorphological appearance, and critical analysis of immunohistochemistry
title_short Metastatic epithelioid malignant peripheral nerve sheath tumor in a known case of neurofibromatosis-1, cytomorphological appearance, and critical analysis of immunohistochemistry
title_sort metastatic epithelioid malignant peripheral nerve sheath tumor in a known case of neurofibromatosis 1 cytomorphological appearance and critical analysis of immunohistochemistry
topic Cytology
epithelioid
fine-needle aspiration cytology
malignant peripheral nerve sheath tumors
neurofibromatosis-1
url http://www.ijmpo.org/article.asp?issn=0971-5851;year=2017;volume=38;issue=3;spage=387;epage=390;aulast=Gupta
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