Malignant peripheral nerve sheath tumour

From Libre Pathology
Revision as of 09:56, 7 October 2013 by Michael (talk | contribs)
Jump to navigation Jump to search

Malignant peripheral nerve sheath tumour, abbreviated MPNST, is an uncommon malignant tumour of the nerve sheath.

Malignant peripheral nerve sheath tumour
Diagnosis in short

MPNST. H&E stain.
LM spindle cell lesion (or very rarely epithelioid) 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

It is also known neurofibrosarcoma[1] and neurogenic sarcoma.[2]

General

Microscopic

Features:

  • Cellular - usu. spindle cells.
    • Rarely epithelioid.
  • Nuclear atypia.
  • Mitoses.
  • +/-Herring bone pattern.

Notes:

  • May be diagnosed in a poorly diff. tumour if patient has NF1.
  • Rarely epithelioid.[3]

DDx:

DDx of herring bone:

Images

  • MPNST - intermed. mag. (WC)

  • MPNST - high mag. (WC)

www:

Grading

  • Can be graded histologically,[4] and this is prognostic.[5]

Sarcoma grading system[6] - based on:

  • Tumour differentiation.
  • Mitotic rate.
  • Necrosis.

IHC

Features:[7]

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

Others:[4]

  • 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. ↑ 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.
  4. ↑ 4.0 4.1 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.
  5. ↑ 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/.
  6. ↑ 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.
  7. ↑ 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.
Retrieved from "https://librepathology.org/w/index.php?title=Malignant_peripheral_nerve_sheath_tumour&oldid=24756"