#### Definition: --- Malignant peripheral nerve sheath tumor arising from a peripheral nerve, neurofibroma or in a patient with neurofibromatosis type 1 (NF1). If none of these criteria apply, then the diagnosis is relied on a combination of histology and immunophenotyping that reveal a differentiation from Schwann cells. #### General: --- Rare tumor (5% of soft tissue tumors) 50% associated with NF1, while 50% arise *de novo*. Patients are from 20-50 years, but may arise in children with NF1. Highly malignant and very aggressive The sciatic nerve is the most commonly involved nerve. #### Radiology: --- No distinct radiological sign, besides from that the tumor has its origo in a peripheral nerve. #### Macroscopic description: --- Gross, spindled mass that involves a big nerve. Often bigger than 5 cm, and the cut surface is whitish with bleedings and necrosis. ### Histologic description: --- Spindle cells growing in fasciculations and โback-to-backโ. Mixture of cellularly dense and less dense areas Geographic necrosis common Cells have hyperchromatic nuclei and pale cytoplasm. May have bizarre cells Frequent mitotic figures (>4 mitoses/ 10 HPF) #### Histologic images: --- ![1200px-Malignant_peripheral_nerve_sheath_tumour_-_high_mag.jpg](https://steemitimages.com/DQmPjGpSCfieGseYzUrMFuMGzsjwA5JHQDrvynhwbGPHcgj/1200px-Malignant_peripheral_nerve_sheath_tumour_-_high_mag.jpg) [source](https://en.wikipedia.org/wiki/Malignant_peripheral_nerve_sheath_tumor#/media/File:Malignant_peripheral_nerve_sheath_tumour_-_high_mag.jpg) #### Positive stains: --- S-100 (<50%), GFAP (20-30%) #### Negative stains: --- SMA, Desmin #### Molecular analysis and cytogenetics: --- Usually not used in diagnostics, but MPNST is overrepresented in NF1 patients. #### Differential diagnosis: --- Fibrosarcoma, monophasic synovial sarcoma, leiomyosarcoma, desmoplastic melanoma, neurofibroma with atypical features #### Treatment and prognosis: --- Radical surgery, often follow by radiation therapy. Poor prognosis (5 year survival rate of about 35%) Negative predictors: Localisation on truncus, tumor size > 5 cm, high grade tumor and radiation indused tumors. Recurrence risk 40-65% Metastasize to lung, bone, pleura, soft tissue, liver and brain Almost everyone with metastatic disease dies # References: *WHO Classification of Tumours of Soft Tissue and Bone (IARC WHO Classification of Tumours), 4th Edition, 2013, edited by Christopher D. M. Fletcher, Julia A. Bridge, Pancras C. W. Hogendoorn and Fredrik Mertens* *Enzinger & Weissโs Soft Tissue Tumours, 6th Edition, edited by John R. Goldblum, Andrew L. Folpe and Sharon W. Weiss* Content by @janusface
post_id | 37,842,346 |
---|---|
author | janusface |
permlink | soft-tissue-tumors-malignant-peripheral-nerve-sheath-tumor-mpnst |
category | medicine |
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Really i appreciate you. Good Post :)
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author | tetas08 |
permlink | re-janusface-soft-tissue-tumors-malignant-peripheral-nerve-sheath-tumor-mpnst-20180310t162045560z |
category | medicine |
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Wonderful ๐
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permlink | re-janusface-soft-tissue-tumors-malignant-peripheral-nerve-sheath-tumor-mpnst-20180310t162203248z |
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Really useful info of medicine, Great work
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permlink | re-janusface-soft-tissue-tumors-malignant-peripheral-nerve-sheath-tumor-mpnst-20180310t162618449z |
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WOW
post_id | 37,845,212 |
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author | varungohil |
permlink | re-janusface-soft-tissue-tumors-malignant-peripheral-nerve-sheath-tumor-mpnst-20180310t164201615z |
category | medicine |
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Thanks for sharing about it in details and in a simplified manner
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permlink | re-janusface-soft-tissue-tumors-malignant-peripheral-nerve-sheath-tumor-mpnst-20180310t165540678z |
category | medicine |
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disease,treatment and prevent is so important for to be cured... good topic and well writing .. prediction of Risk is also indicating your wisdom,sir... like your post @upvoted @resteem
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permlink | re-janusface-soft-tissue-tumors-malignant-peripheral-nerve-sheath-tumor-mpnst-20180310t173818452z |
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That's a weird post from you, I hope all is ok my friend!
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permlink | re-janusface-soft-tissue-tumors-malignant-peripheral-nerve-sheath-tumor-mpnst-20180310t182934747z |
category | medicine |
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very good post :) your post have alot of information that i need to know thank you so much for sharing with us :) @ janusface
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permlink | re-janusface-soft-tissue-tumors-malignant-peripheral-nerve-sheath-tumor-mpnst-20180310t192206305z |
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Have cells been tested for intracellular pH? Janus. For other reasons, contact me.
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permlink | re-janusface-soft-tissue-tumors-malignant-peripheral-nerve-sheath-tumor-mpnst-20180325t225834752z |
category | medicine |
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