Difference between revisions of "Peripheral nerve sheath tumours"
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*[http://radiographics.rsna.org/content/24/suppl_1/S237/F10.expansion.html Plexiform neurofibroma (rsna.org)]. | *[http://radiographics.rsna.org/content/24/suppl_1/S237/F10.expansion.html Plexiform neurofibroma (rsna.org)]. | ||
*[http://path.upmc.edu/cases/case304/micro.html Plexiform neurofibroma - several images (upmc.edu)]. | *[http://path.upmc.edu/cases/case304/micro.html Plexiform neurofibroma - several images (upmc.edu)]. | ||
*[http://eyewiki.aao.org/File:Orbital_Neurofibroma_-_S100.JPG Neurofibroma - S-100 (eyewiki.aao.org)]. | |||
===IHC=== | ===IHC=== | ||
Revision as of 10:27, 5 September 2013
Peripheral nerve sheath tumours, abbreviated PNSTs, are common in neuropathology and occasionally show-up elsewhere. A very common PNST is the schwannoma.
Classification
A classification:[1]
- Benign:
- Malignant:
Specific diagnoses
Schwannoma
General
- A common neuropathology tumour that occasionally shows-up elsewhere.
- Tumour of tissue surrounding a nerve.
- Axons adjacent to the tumour are normal... but may be compressed.
- May be a part of neurofibromatosis type 2.
Microscopic
Features:[1]
- Antoni A:
- Cellular.
- 'Fibrillary, polar, elongated'.
- Antoni B:
- Pauci-cellular.
- Loose microcystic tissue.
- Verocay bodies - paucinuclear area surrounded by nuclei - diagnostic feature.
- Hyaline thickened blood vessels.
- Thick capsule.
- In the GI tract: classically have a peripheral lymphoid cuff.[2]
- +/-Hemosiderin deposition within tumour.
Notes:
- Tumour does not smear well.[3]
- Antoni A: may look somewhat like scattered matchsticks.
DDx:
- Meningioma.
- Intranodal palisaded myofibroblastoma - if surrounded by a rim of lymphoid tissue, i.e. intranodal.
- Leiomyoma.
Images:
- www:
- WC:
Schwannoma subtypes
There are four:[4]
- Conventional schwannoma.
- Cellular schwannoma.
- Plexiform schwannoma.
- Melanotic schwannoma.
Conventional schwannoma
- Most common.
Cellular schwannoma
- May mimic MPNST.
Images:
Plexiform schwannoma
- May mimic MPNST if cellular - esp. in childhood.
Images:
Melanotic schwannoma
- May be confused with melanoma.
- Psammomatous form (psammomatous melanotic schwannoma) associated with a heritable disorder (Carney complex).
Note:
- Carney complex:[4]
- Cutaneous lentigines.
- Myxomas (skin (subcutaneous), subcutanous, heart).
- Endocrine neoplasms.
Images:
IHC
Features:[5]
- S-100 +ve.
- Glut1 +ve.
- CD34 +ve.
- Cytokeratins ~70% +ve.[citation needed]
- SOX10 +ve.[6]
- -ve in synovial sarcoma, rhabdomyosarcoma, chondrosarcoma.
- EMA -ve. (???)
- Usually +ve (~75% of the time) in meningiomas.[7]
Perineurioma
General
- Benign tumour derived from perineurial cells.
Variant:
- Reticular perineurioma.[8]
Microscopic
Features:[9]
- Perineural epithelioid cells.
- Abundant pale, fluffy appearing cytoplasm.
Note:
- May be intraneural.[9]
DDx:
- Neuroma.
- Neurofibroma.
- Schwannoma.
- S100 +ve, EMA -ve.[9]
- Liposarcoma - reticular perineurioma.
Images:
IHC
- S100 -ve.
- EMA +ve.
- CD34 ~65% +ve.[10]
Traumatic neuroma
General
- Consequence of trauma - diagnosis requires history of trauma.
- May mimic a cancer recurrence at the site of a surgery.[12]
Microscopic
- +/-Nerve - that was injured.
- Grouping of disordered nerve fibre bundles in fibrous tissue (collagen) - key feature.
- +/-Myxoid change.
- +/-Axonal swellings (ovoid pink/purple blobs).
DDx:
- Morton neuroma (foot).
Images:
- Traumatic neuroma (nih.gov).[12]
- Traumatic neuroma - several images (upmc.edu).
- Traumatic neuroma (sarcomaimages.com).
- Traumatic neuroma (nih.gov).[14]
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SOFT TISSUE LESION, RIGHT WRIST, EXCISION: - TRAUMATIC NEUROMA. - BENIGN FIBROADIPOSE TISSUE.
Micro
The sections show disordered nerve fibre bundles in fibrous tissue.
Palisaded encapsulated neuroma
- Abbreviated PEN.
- AKA palisaded and encapsulated neuroma.
General
- Flesh-colour papule - classically on the face.[15]
- Isolated finding - not associated with a systemic disease or malignancy.[16]
- Superficial skin.[17]
Microscopic
Features:[15]
- Encapsulated dermal spindle cell lesion.
- Fasciular arrangement.
- Neural-type spindle cells:
- Not vacuolated.
- Nuclei have pointy ends.
- Intralesional clefts.
- Useful to differentiate from schwannoma.
DDx:
- Schwannoma:[15]
- No intralesional clefts.
- More variability in the cellularity.
- May be deep.
Other considerations:
- Leiomyoma - cytoplasm not vacuolated, nuclei more elliptical.
Images:
IHC
Features:[16]
- S100 +ve.
- EMA +ve (capsule of lesion).
Neurofibroma
General
- May be a part of neurofibromatosis 1 (NF1).
- A painful skin lesion.
- Composed of Schwann cells, axons, fibrous material.[1]
Classification:[18]
- Localized - sporatic.
- Diffuse - usually poorly defined, young adults and children; sporatic.
- Plexiform - associated with NF1.
Gross/radiologic
Gross features (plexiform NF):[18]
- "Bag of worms" appearance.
Radiologic:[18]
- Fusiform mass.
Microscopic
Features:
- Spindle cells with wavy nuclei without pleomorphism - key feature.
- Often described as "shredded carrots".
- May be arranged in fascicles and intermixed with collagen.
- Often no pattern is apparent.
- Moderate increase of cellularity vis-a-vis normal dermis.
- May be poorly or well-circumscribed.
- +/-Plexiform growth pattern - "bag of worms".[1]
- Multiple well-circumscribed nests.
- Mast cells[19] - one has to look for them at high power.
- Very useful for confirming the low power suspicion.
DDx:
- Schwannoma.
- Dermatofibrosarcoma protuberans (DFSP) - S-100 -ve, CD34 +ve.
- Ganglioneuroma.
- Neurotized melanocytic nevus - melanocyte nests make the diagnosis, otherwise immunostains are needed to differentiate.[20]
- Usually have more mast cells than neurofibromas.[21]
Images
Neurofibroma - low mag. (WC)
Neurofibroma - intermed. mag. (WC)
Neurofibroma - high mag. (WC)
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.jpg)
www:
- Plexiform neurofibroma (rsna.org).
- Plexiform neurofibroma (rsna.org).
- Plexiform neurofibroma - several images (upmc.edu).
- Neurofibroma - S-100 (eyewiki.aao.org).
IHC
Features:[5]
- S100 +ve -- wavy pattern.[22]
- CD34 +ve.
- Glut1 +ve.
- EMA +ve/-ve.
- NF +ve/-ve.[22]
- MART-1 -ve.[22]
- Positive in neurotized melanocytic nevi.
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FOURTH TOE, LEFT, EXCISION: - NEUROFIBROMA.
Micro
The sections show skin with a lesion composed of irregular-shaped groups of bland dermal spindle cells with wavy nuclei and pale-eosinophilic cytoplasm. Mast cells are seen scattered throughout the lesion. Thick collagen separates the clusters of the spindle cells. There is no nuclear atypia. Mitotic activity is not appreciated. No melanocytic nests are identified.
The overlying epidermis matures to the surface.
Neurothekeoma
General
- Rare.
- Female > male.
Microscopic
Features:[23]
- Superficial dermal lesion:
- Usu. lobulated or micronodular architecture - key feature.
- +/-Focal sheeting.
- Spindle/epithelioid morphology with pale eosinophilic cytoplasm - key feature.
- +/-Inflammation around lesion.
- +/-Surrounded by collagen.
- Usu. lobulated or micronodular architecture - key feature.
Notes:
- No atypia.
- Mitoses rare/none.
- Often poorly circumscribed.
Subtypes:[24]
- Cellular.
- Myxoid.
- Intermediate.
DDx:
- Dermatofibroma.
- Angiomatoid fibrous histiocytoma -- have cystic blood filled spaces, inflammation.[25]
Images:
IHC
Features:[23]
- NKI/C3 (AKA NKI-C3) +ve.
- NSE +/-ve.
Others:[26]
- Vimentin +ve.
- CD10 +ve.
- Microphthalmia transcription factor +ve.
- PGP9.5 +ve.
Exclusionary:
- S100 -ve.
- Exclude other peripheral nerve sheath tumours. (???)
Malignant peripheral nerve sheath tumour
General
- Malignant - as the name implies.
- Usu. assoc. with a peripheral nerve.[citation needed]
- May be seen in the context of neurofibromatosis type 1.
Microscopic
Features:
- Cellular.
- Nuclear atypia.
- Mitoses.
- +/-Herring bone pattern.
Notes:
- May be diagnosed in a poorly diff. tumour if patient has NF1.
DDx:
- Cellular schwannoma.
- Plexiform schwannoma.
- Malignant triton tumour.
DDx of herring bone:
- MPNST.
- Synovial sarcoma.
- Fibrosarcoma.
Images:
- MPNST - intermed. mag. (WC).
- MPNST - high mag. (WC).
- MPNST - several images (upmc.edu).
- MPNST - case 2 - several images (upmc.edu).
Grading
Sarcoma grading system[30] - based on:
- Tumour differentiation.
- Mitotic rate.
- Necrosis.
IHC
Features:[6]
- S-100 +ve ~ 30% of tumours.
- SOX10 +ve ~ 50% of tumours.
Others:[28]
- p53.
- p16.
- p27.
- MIB1.
Malignant triton tumour
- Abbreviated MTT.
- AKA malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation.[31]
General
- Rare.
- Considered to be a variant of MPNST.
- Prognosis worse that conventional MPNST.[31]
- Five year survival ~14%.[32]
- Diagnosis may require clinical information, i.e. individual has a history of neurofibromatosis type 1 (NF1).
Note:
- A handful of benign triton tumours are reported; these are considered neuromuscular hamartomas.[33]
Microscopic
Features - Woodruff criteria - all three required:[31]
- (a) Tumour arise from a peripheral nerve or (b) individual has NF1 or (c) lesion a metastasis arising in the context of (a) or (b).
- Schwann cell tumour characteristics.
- Rhabdomyoblasts.
- Eccentric nucleus.
- Moderate amount of eosinophilic cytoplasm.
- +/-Cross-striations.
DDx:
- Malignant peripheral nerve sheath tumour.
- Adult fibrosarcoma.
- Synovial sarcoma.
- Rhabdomyosarcoma.
- Carcinosarcoma.
IHC
Features:
- S100 +ve/-ve -- usu. focal if positive.[31]
- Leu-7 +ve/-ve.
- Myelin basic protein +ve/-ve.
Rhabdomyoblastic differentiation:[31]
- Desmin.
- Actin.
- Myogenin.
EM
- +/-Sarcomeres.[31]
Morton neuroma
General
- Benign foot condition.
- Uncommon.
- Usu. interdigital nerves.
Etiology:
- Indirect nerve trauma.
Clinical:[34]
- Foot pain.
Microscopic
Features:[34]
- Extensive fibrosis around and within the nerve.
- Digital artery:
- +/-Thrombosis.
- +/-Arterial thickening.
DDx:
Images:
See also
References
- ↑ 1.0 1.1 1.2 1.3 Wippold FJ, Lubner M, Perrin RJ, Lämmle M, Perry A (October 2007). "Neuropathology for the neuroradiologist: Antoni A and Antoni B tissue patterns". AJNR Am J Neuroradiol 28 (9): 1633–8. doi:10.3174/ajnr.A0682. PMID 17893219. http://www.ajnr.org/cgi/reprint/28/9/1633.
- ↑ Levy AD, Quiles AM, Miettinen M, Sobin LH (March 2005). "Gastrointestinal schwannomas: CT features with clinicopathologic correlation". AJR Am J Roentgenol 184 (3): 797–802. PMID 15728600. http://www.ajronline.org/cgi/content/full/184/3/797.
- ↑ MUN. 24 November 2010.
- ↑ 4.0 4.1 Kurtkaya-Yapicier O, Scheithauer B, Woodruff JM (July 2003). "The pathobiologic spectrum of Schwannomas". Histol. Histopathol. 18 (3): 925–34. PMID 12792904.
- ↑ 5.0 5.1 Hirose T, Tani T, Shimada T, Ishizawa K, Shimada S, Sano T (April 2003). "Immunohistochemical demonstration of EMA/Glut1-positive perineurial cells and CD34-positive fibroblastic cells in peripheral nerve sheath tumors". Mod. Pathol. 16 (4): 293–8. doi:10.1097/01.MP.0000062654.83617.B7. PMID 12692193. http://www.nature.com/modpathol/journal/v16/n4/full/3880761a.html.
- ↑ 6.0 6.1 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.
- ↑ Rushing, EJ.; Bouffard, JP.; McCall, S.; Olsen, C.; Mena, H.; Sandberg, GD.; Thompson, LD. (Jun 2009). "Primary extracranial meningiomas: an analysis of 146 cases.". Head Neck Pathol 3 (2): 116-30. doi:10.1007/s12105-009-0118-1. PMID 19644540.
- ↑ Graadt van Roggen, JF.; McMenamin, ME.; Belchis, DA.; Nielsen, GP.; Rosenberg, AE.; Fletcher, CD. (Apr 2001). "Reticular perineurioma: a distinctive variant of soft tissue perineurioma.". Am J Surg Pathol 25 (4): 485-93. PMID 11257623.
- ↑ 9.0 9.1 9.2 Mills, Stacey E; Carter, Darryl; Greenson, Joel K; Reuter, Victor E; Stoler, Mark H (2009). Sternberg's Diagnostic Surgical Pathology (5th ed.). Lippincott Williams & Wilkins. pp. 424. ISBN 978-0781779425.
- ↑ 10.0 10.1 Hornick, JL.; Fletcher, CD. (Jul 2005). "Soft tissue perineurioma: clinicopathologic analysis of 81 cases including those with atypical histologic features.". Am J Surg Pathol 29 (7): 845-58. PMID 15958848.
- ↑ Tsang, WY.; Chan, JK.; Chow, LT.; Tse, CC. (Aug 1992). "Perineurioma: an uncommon soft tissue neoplasm distinct from localized hypertrophic neuropathy and neurofibroma.". Am J Surg Pathol 16 (8): 756-63. PMID 1497116.
- ↑ 12.0 12.1 12.2 Li, Q.; Gao, EL.; Yang, YL.; Hu, HY.; Hu, XQ. (2012). "Traumatic neuroma in a patient with breast cancer after mastectomy: a case report and review of the literature.". World J Surg Oncol 10: 35. doi:10.1186/1477-7819-10-35. PMID 22330690.
- ↑ Tadrous, Paul.J. Diagnostic Criteria Handbook in Histopathology: A Surgical Pathology Vade Mecum (1st ed.). Wiley. pp. 317. ISBN 978-0470519035.
- ↑ Kwon, JH.; Ryu, SW.; Kang, YN.. "Traumatic neuroma around the celiac trunk after gastrectomy mimicking a nodal metastasis: a case report.". Korean J Radiol 8 (3): 242-5. PMC 2627419. PMID 17554193. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2627419/.
- ↑ 15.0 15.1 15.2 Busam, Klaus J. (2009). Dermatopathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Saunders. pp. 536. ISBN 978-0443066542.
- ↑ 16.0 16.1 16.2 Newman, MD.; Milgraum, S. (2008). "Palisaded encapsulated neuroma (PEN): an often misdiagnosed neural tumor.". Dermatol Online J 14 (7): 12. PMID 18718196.
- ↑ S. Sade. 8 September 2011.
- ↑ 18.0 18.1 18.2 Wilkinson, LM.; Manson, D.; Smith, CR. (Oct 2004). "Best cases from the AFIP: plexiform neurofibroma of the bladder.". Radiographics 24 Suppl 1: S237-42. doi:10.1148/rg.24si035170. PMID 15486243.
- ↑ Staser, K.; Yang, FC.; Clapp, DW. (Jul 2010). "Mast cells and the neurofibroma microenvironment.". Blood 116 (2): 157-64. doi:10.1182/blood-2009-09-242875. PMID 20233971.
- ↑ Gray, MH.; Smoller, BR.; McNutt, NS.; Hsu, A. (Jun 1990). "Neurofibromas and neurotized melanocytic nevi are immunohistochemically distinct neoplasms.". Am J Dermatopathol 12 (3): 234-41. PMID 1693815.
- ↑ Carr, NJ.; Warren, AY. (Jan 1993). "Mast cell numbers in melanocytic naevi and cutaneous neurofibromas.". J Clin Pathol 46 (1): 86-7. PMID 8432898.
- ↑ 22.0 22.1 22.2 Chen, Y.; Klonowski, PW.; Lind, AC.; Lu, D. (Jul 2012). "Differentiating neurotized melanocytic nevi from neurofibromas using Melan-A (MART-1) immunohistochemical stain.". Arch Pathol Lab Med 136 (7): 810-5. doi:10.5858/arpa.2011-0335-OA. PMID 22742554.
- ↑ 23.0 23.1 Hornick JL, Fletcher CD (March 2007). "Cellular neurothekeoma: detailed characterization in a series of 133 cases". Am. J. Surg. Pathol. 31 (3): 329–40. doi:10.1097/01.pas.0000213360.03133.89. PMID 17325474.
- ↑ Wang AR, May D, Bourne P, Scott G (November 1999). "PGP9.5: a marker for cellular neurothekeoma". Am. J. Surg. Pathol. 23 (11): 1401–7. PMID 10555009.
- ↑ URL: http://surgpathcriteria.stanford.edu/softfib/angiomatoid_fibrous_histiocytoma/. Accessed on: 11 May 2011.
- ↑ Fetsch JF, Laskin WB, Hallman JR, Lupton GP, Miettinen M (July 2007). "Neurothekeoma: an analysis of 178 tumors with detailed immunohistochemical data and long-term patient follow-up information". Am. J. Surg. Pathol. 31 (7): 1103–14. doi:10.1097/PAS.0b013e31802d96af. PMID 17592278.
- ↑ 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.
- ↑ 28.0 28.1 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.
- ↑ 31.0 31.1 31.2 31.3 31.4 31.5 Stasik, CJ.; Tawfik, O. (Dec 2006). "Malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation (malignant triton tumor).". Arch Pathol Lab Med 130 (12): 1878-81. doi:10.1043/1543-2165(2006)130[1878:MPNSTW]2.0.CO;2. PMID 17149968.
- ↑ McConnell, YJ.; Giacomantonio, CA. (Jan 2012). "Malignant triton tumors-complete surgical resection and adjuvant radiotherapy associated with improved survival.". J Surg Oncol. doi:10.1002/jso.23042. PMID 22253011.
- ↑ Castro, DE.; Raghuram, K.; Phillips, CD. (Apr 2005). "Benign triton tumor of the trigeminal nerve.". AJNR Am J Neuroradiol 26 (4): 967-9. PMID 15814954.
- ↑ 34.0 34.1 Mills, Stacey E; Carter, Darryl; Greenson, Joel K; Oberman, Harold A; Reuter, Victor E (2004). Sternberg's Diagnostic Surgical Pathology (4th ed.). Lippincott Williams & Wilkins. pp. 243. ISBN 978-0781740517.
- ↑ URL: http://www.sciencedirect.com/science/article/pii/S1027811711000863. Accessed on: 27 December 2012.