Difference between revisions of "Malignant peripheral nerve sheath tumour"

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{{ Infobox diagnosis
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Name      = {{PAGENAME}}
| Image      =  
| Image      = Malignant_peripheral_nerve_sheath_tumour_-_high_mag.jpg
| Width      =
| Width      =
| Caption    =  
| Caption    = MPNST. [[H&E stain]].
| Micro      =
| Micro      =
| Subtypes  =
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| Grossing  =
| Grossing  =
| Site      = [[soft tissue lesions|soft tissue]]
| Site      = [[soft tissue lesions|soft tissue]]
| Assdx      =
| Assdx      = [[neurofibroma]], [[plexiform neurofibroma]]
| Syndromes  =
| Syndromes  = [[neurofibromatosis type 1]]
| Clinicalhx =
| Clinicalhx =
| Signs      =
| Signs      = mass
| Symptoms  =
| Symptoms  =
| Prevalence =
| Prevalence =
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| Rads      =
| Rads      =
| Endoscopy  =
| Endoscopy  =
| Prognosis  =
| Prognosis  = poor
| Other      =
| Other      =
| ClinDDx    =
| ClinDDx    =

Revision as of 05:52, 25 September 2013

Malignant peripheral nerve sheath tumour
Diagnosis in short

MPNST. H&E stain.
LM DDx synovial sarcoma, fibrosarcoma, cellular schwannoma, plexiform schwannoma, malignant triton tumour.
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] and neurogenic sarcoma.[2]

General

Microscopic

Features:

  • Cellular.
  • Nuclear atypia.
  • Mitoses.
  • +/-Herring bone pattern.

Notes:

  • May be diagnosed in a poorly diff. tumour if patient has NF1.

DDx:

DDx of herring bone:

Images

www:

Grading

  • Can be graded histologically,[3] and this is prognostic.[4]

Sarcoma grading system[5] - based on:

  • Tumour differentiation.
  • Mitotic rate.
  • Necrosis.

IHC

Features:[6]

  • S-100 +ve ~ 30% of tumours.
  • SOX10 +ve ~ 50% of tumours.

Others:[3]

  • p53.
  • p16.
  • p27.
  • MIB1.

See also

References

  1. 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.
  2. 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.
  3. 3.0 3.1 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.
  4. 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/.
  5. 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.
  6. 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.