Difference between revisions of "Malignant peripheral nerve sheath tumour"
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*Mitoses. | *Mitoses. | ||
*+/-Herring bone pattern. | *+/-Herring bone pattern. | ||
*Perivascular hypercellularity | *Perivascular hypercellularity. | ||
*Tumor herniation into vascular lumens | *Tumor herniation into vascular lumens. | ||
*"Pseudocapsule" with tumour invasion of adjacent tissue. | |||
Notes: | Notes: | ||
| Line 62: | Line 63: | ||
*Plexiform schwannoma. | *Plexiform schwannoma. | ||
*[[Malignant triton tumour]]. | *[[Malignant triton tumour]]. | ||
**aka. MPNST with mesenchymal differentiation. | |||
DDx of herring bone: | DDx of herring bone: | ||
| Line 80: | Line 82: | ||
===Grading=== | ===Grading=== | ||
*Can be graded histologically,<ref name=pmid14508395>{{cite journal |author=Zhou H, Coffin CM, Perkins SL, Tripp SR, Liew M, Viskochil DH |title=Malignant peripheral nerve sheath tumor: a comparison of grade, immunophenotype, and cell cycle/growth activation marker expression in sporadic and neurofibromatosis 1-related lesions |journal=Am. J. Surg. Pathol. |volume=27 |issue=10 |pages=1337–45 |year=2003 |month=October |pmid=14508395 |doi= |url=}}</ref> and this is prognostic.<ref name=pmid16923196>{{cite journal |author=Kar M, Deo SV, Shukla NK, ''et al.'' |title=Malignant peripheral nerve sheath tumors (MPNST)--clinicopathological study and treatment outcome of twenty-four cases |journal=World J Surg Oncol |volume=4 |issue= |pages=55 |year=2006 |pmid=16923196 |pmc=1560134 |doi=10.1186/1477-7819-4-55 |url=}}</ref> | *Can be graded histologically, according WHO<ref name=pmid14508395>{{cite journal |author=Zhou H, Coffin CM, Perkins SL, Tripp SR, Liew M, Viskochil DH |title=Malignant peripheral nerve sheath tumor: a comparison of grade, immunophenotype, and cell cycle/growth activation marker expression in sporadic and neurofibromatosis 1-related lesions |journal=Am. J. Surg. Pathol. |volume=27 |issue=10 |pages=1337–45 |year=2003 |month=October |pmid=14508395 |doi= |url=}}</ref> and this is prognostic.<ref name=pmid16923196>{{cite journal |author=Kar M, Deo SV, Shukla NK, ''et al.'' |title=Malignant peripheral nerve sheath tumors (MPNST)--clinicopathological study and treatment outcome of twenty-four cases |journal=World J Surg Oncol |volume=4 |issue= |pages=55 |year=2006 |pmid=16923196 |pmc=1560134 |doi=10.1186/1477-7819-4-55 |url=}}</ref> | ||
*grade IV: Presence of necrosis (3/4 of all MPNST). | |||
*grade III: Mitotic count exceeds 4/1 HPF. | |||
*grade II: nuclear size (<3x of neurofibroma cell), hyperchromatic nuclei. | |||
**overlap with cellular ("atypical") neurofibroma (DDx). | |||
Sarcoma grading system<ref name=pmid6693192>{{cite journal |author=Trojani M, Contesso G, Coindre JM, ''et al.'' |title=Soft-tissue sarcomas of adults; study of pathological prognostic variables and definition of a histopathological grading system |journal=Int. J. Cancer |volume=33 |issue=1 |pages=37–42 |year=1984 |month=January |pmid=6693192 |doi= |url=}}</ref> - based on: | Sarcoma grading system<ref name=pmid6693192>{{cite journal |author=Trojani M, Contesso G, Coindre JM, ''et al.'' |title=Soft-tissue sarcomas of adults; study of pathological prognostic variables and definition of a histopathological grading system |journal=Int. J. Cancer |volume=33 |issue=1 |pages=37–42 |year=1984 |month=January |pmid=6693192 |doi= |url=}}</ref> - based on: | ||
Revision as of 10:17, 9 May 2016
| Malignant peripheral nerve sheath tumour | |
|---|---|
| Diagnosis in short | |
 MPNST. H&E stain. | |
|
| |
| LM | spindle cell lesion (or very rarely epithelioid lesion) with nuclear atypia, mitotic activity, +/-herring bone pattern |
| Subtypes | malignant triton tumour |
| LM DDx | synovial sarcoma, fibrosarcoma, cellular schwannoma, plexiform schwannoma |
| Site | soft tissue |
|
| |
| Associated Dx | neurofibroma, plexiform neurofibroma |
| Syndromes | neurofibromatosis type 1 |
|
| |
| Signs | mass |
| Prognosis | poor |
Malignant peripheral nerve sheath tumour, abbreviated MPNST, is an uncommon malignant tumour of the nerve sheath.
It is also known neurofibrosarcoma[1], neurogenic sarcoma.[2] and malignant schwannoma - these terms are however depreceated.[3]
General
- Malignant - as the name implies.
- Usually assoc. with a peripheral nerve.[4]
- May be seen in the context of neurofibromatosis type 1 or radiation induced.[5]
- Approx 50-70% arise from (mostly plexiform) neurofibromas.
- Usu. adults
- Approx. 5% of all malignant soft tissue lesions.
MPNST in a NF1 case (WC/Filip em).
MPNST gross pathology (Flickr/drbloodmoney).
Microscopic
Features:[6]
- Cellular - usu. spindle cells.
- Very rarely epithelioid.[7]
- Nuclear atypia.
- Mitoses.
- +/-Herring bone pattern.
- Perivascular hypercellularity.
- Tumor herniation into vascular lumens.
- "Pseudocapsule" with tumour invasion of adjacent tissue.
Notes:
- May be diagnosed in a poorly diff. tumour if patient has NF1.
DDx:
- Cellular schwannoma.
- Plexiform schwannoma.
- Malignant triton tumour.
- aka. MPNST with mesenchymal differentiation.
DDx of herring bone:
- MPNST.
- Synovial sarcoma.
- Fibrosarcoma.
Images
MPNST - intermed. mag. (WC)
MPNST - high mag. (WC)
MPNST - very high mag. (WC)
MPNST with chondroid dedifferentiation (Triton tumor). (WC)
www:
Grading
- Can be graded histologically, according WHO[8] and this is prognostic.[9]
- grade IV: Presence of necrosis (3/4 of all MPNST).
- grade III: Mitotic count exceeds 4/1 HPF.
- grade II: nuclear size (<3x of neurofibroma cell), hyperchromatic nuclei.
- overlap with cellular ("atypical") neurofibroma (DDx).
Sarcoma grading system[10] - based on:
- Tumour differentiation.
- Mitotic rate.
- Necrosis.
IHC
Features:[11]
- S-100 +ve ~ 30% of tumours.
- SOX10 +ve ~ 50% of tumours.
- Neurofibromin (NFC) -ve (88% in NF1, 43% sporadic MPNST)[12]
- MIB-1 ≥20% is highly predictive of malignant peripheral nerve sheath tumor (87% sensitivity and 96% specificity).[13]
Others:[8]
- p53.
- p16 -ve.
- p27.
- p75NTR +ve (80%).
Molecular
- Atypical neurofibromas and low-grade MPNST have a common methylation profile and frequent losses of CDKN2A.
- A subset of MPNST show loss of trimethylation of histone H3 at lysine 27 (H3K27me3).
- P53 and beta-spectrin mutations during progression reported.
See also
References
- ↑ Mills, AM.; Karamchandani, JR.; Vogel, H.; Longacre, TA. (Mar 2011). "Endocervical fibroblastic malignant peripheral nerve sheath tumor (neurofibrosarcoma): report of a novel entity possibly related to endocervical CD34 fibrocytes.". Am J Surg Pathol 35 (3): 404-12. doi:10.1097/PAS.0b013e318208f72e. PMID 21317712.
- ↑ Sham, ME.; Ghorpade, A.; Shetty, S.; Hari, .; Vinay, . (Mar 2010). "Malignant peripheral nerve cell tumour.". J Maxillofac Oral Surg 9 (1): 68-71. doi:10.1007/s12663-010-0019-6. PMID 23139572.
- ↑ {WHOCNS2007}
- ↑ {WHOCNS2007}
- ↑ Ducatman, BS.; Scheithauer, BW.; Piepgras, DG.; Reiman, HM.; Ilstrup, DM. (May 1986). "Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases.". Cancer 57 (10): 2006-21. PMID 3082508.
- ↑ Pekmezci, M.; Reuss, DE.; Hirbe, AC.; Dahiya, S.; Gutmann, DH.; von Deimling, A.; Horvai, AE.; Perry, A. (Feb 2015). "Morphologic and immunohistochemical features of malignant peripheral nerve sheath tumors and cellular schwannomas.". Mod Pathol 28 (2): 187-200. doi:10.1038/modpathol.2014.109. PMID 25189642.
- ↑ Carter, JM.; O'Hara, C.; Dundas, G.; Gilchrist, D.; Collins, MS.; Eaton, K.; Judkins, AR.; Biegel, JA. et al. (Jan 2012). "Epithelioid malignant peripheral nerve sheath tumor arising in a schwannoma, in a patient with neuroblastoma-like schwannomatosis and a novel germline SMARCB1 mutation.". Am J Surg Pathol 36 (1): 154-60. doi:10.1097/PAS.0b013e3182380802. PMID 22082606.
- ↑ 8.0 8.1 Zhou H, Coffin CM, Perkins SL, Tripp SR, Liew M, Viskochil DH (October 2003). "Malignant peripheral nerve sheath tumor: a comparison of grade, immunophenotype, and cell cycle/growth activation marker expression in sporadic and neurofibromatosis 1-related lesions". Am. J. Surg. Pathol. 27 (10): 1337–45. PMID 14508395.
- ↑ Kar M, Deo SV, Shukla NK, et al. (2006). "Malignant peripheral nerve sheath tumors (MPNST)--clinicopathological study and treatment outcome of twenty-four cases". World J Surg Oncol 4: 55. doi:10.1186/1477-7819-4-55. PMC 1560134. PMID 16923196. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1560134/.
- ↑ Trojani M, Contesso G, Coindre JM, et al. (January 1984). "Soft-tissue sarcomas of adults; study of pathological prognostic variables and definition of a histopathological grading system". Int. J. Cancer 33 (1): 37–42. PMID 6693192.
- ↑ Nonaka D, Chiriboga L, Rubin BP (September 2008). "Sox10: a pan-schwannian and melanocytic marker". Am. J. Surg. Pathol. 32 (9): 1291–8. doi:10.1097/PAS.0b013e3181658c14. PMID 18636017.
- ↑ Reuss, DE.; Habel, A.; Hagenlocher, C.; Mucha, J.; Ackermann, U.; Tessmer, C.; Meyer, J.; Capper, D. et al. (Apr 2014). "Neurofibromin specific antibody differentiates malignant peripheral nerve sheath tumors (MPNST) from other spindle cell neoplasms.". Acta Neuropathol 127 (4): 565-72. doi:10.1007/s00401-014-1246-6. PMID 24464231.
- ↑ Pekmezci, M.; Reuss, DE.; Hirbe, AC.; Dahiya, S.; Gutmann, DH.; von Deimling, A.; Horvai, AE.; Perry, A. (Feb 2015). "Morphologic and immunohistochemical features of malignant peripheral nerve sheath tumors and cellular schwannomas.". Mod Pathol 28 (2): 187-200. doi:10.1038/modpathol.2014.109. PMID 25189642.
- ↑ Röhrich, M.; Koelsche, C.; Schrimpf, D.; Capper, D.; Sahm, F.; Kratz, A.; Reuss, J.; Hovestadt, V. et al. (Feb 2016). "Methylation-based classification of benign and malignant peripheral nerve sheath tumors.". Acta Neuropathol. doi:10.1007/s00401-016-1540-6. PMID 26857854.
- ↑ Hirbe, AC.; Dahiya, S.; Miller, CA.; Li, T.; Fulton, RS.; Zhang, X.; McDonald, S.; DeSchryver, K. et al. (Sep 2015). "Whole Exome Sequencing Reveals the Order of Genetic Changes during Malignant Transformation and Metastasis in a Single Patient with NF1-plexiform Neurofibroma.". Clin Cancer Res 21 (18): 4201-11. doi:10.1158/1078-0432.CCR-14-3049. PMID 25925892.