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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| Format: | Article |
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Thieme Medical and Scientific Publishers Pvt. Ltd.
2017-01-01
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| 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. |
| format | Article |
| id | doaj-art-2387d0fecfd94d359f0dec0ff6968e17 |
| institution | DOAJ |
| issn | 0971-5851 |
| language | English |
| publishDate | 2017-01-01 |
| publisher | Thieme Medical and Scientific Publishers Pvt. Ltd. |
| record_format | Article |
| 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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