Difference between revisions of "Plasma cell neoplasms"

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'''Plasma cell neoplasms''' arise from plasma cells.  They are encountered by anatomical pathologists on occasion.
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Plasma cell neoplasm -- very high mag.jpg
| Width      =
| Caption    = Plasma cell neoplasm. [[H&E stain]]
| Synonyms  =
| Micro      = dyscohesive plasmacytoid cells (abundant cytoplasm, eccentrically placed nucleus), often prominent perinuclear hof, +/-nucleoli, +/-Russell bodies, +/-Dutcher bodies
| Subtypes  =
| LMDDx      = [[lymphoplasmacytic lymphoma]], [[neuroendocrine carcinoma]], poorly differentiated carcinoma, others
| Stains    =
| IHC        = [[CD138]] +ve, CD56 +ve, CD45 -ve/+ve, CD79a +ve
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = [[bone]] - [[bone marrow]]
| Assdx      = [[renal failure]], [[myeloma cast nephropathy]]
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence =
| Bloodwork  = +/-[[anemia]], +/-hypercalcemia
| Rads      = +/-lytic bone lesions
| Endoscopy  =
| Prognosis  = poor
| Other      =
| ClinDDx    = [[metastatic disease]]
| Tx        =
}}
'''Plasma cell neoplasms''' arise from [[plasma cell]]s.  They are encountered by anatomical pathologists on occasion.


[[VL]] does not tease apart ''plasma cell myeloma'', ''plasmacytoma'' and ''plasma cell neoplasm''; the first two of these terms redirect to this article.
''Plasma cell myeloma'', and ''plasmacytoma'' (solitary myeloma)<ref name=Ref_PCPBoD8_324>{{Ref PCPBoD8|324}}</ref> redirect to this article.


==General==
==General==
*Malignancy derived from the plasma cells.
*Malignancy derived from the plasma cells.
*Prognosis: poor.
*Prognosis: poor.
*Most common primary [[bone tumour]] in adults.
*Common primary [[bone tumour]] in adults.


Clinical:<ref name=Ref_PCPBoD8_323>{{Ref PCPBoD8|323}}</ref>
Clinical:<ref name=Ref_PCPBoD8_323>{{Ref PCPBoD8|323}}</ref>
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Note:
Note:
*Plasmacytoma = histology of '''multiple myeloma'''; to diagnose ''multiple myeloma'' other (non-pathology) criteria are needed.
*Plasmacytoma = histology of '''multiple myeloma'''; to diagnose ''multiple myeloma'' other (non-pathology) criteria are needed.
Classified by site:
*Medullary.
*Extramedullary - usu. upper aerodigestive tract.<ref name=pmid10357398>{{Cite journal  | last1 = Alexiou | first1 = C. | last2 = Kau | first2 = RJ. | last3 = Dietzfelbinger | first3 = H. | last4 = Kremer | first4 = M. | last5 = Spiess | first5 = JC. | last6 = Schratzenstaller | first6 = B. | last7 = Arnold | first7 = W. | title = Extramedullary plasmacytoma: tumor occurrence and therapeutic concepts. | journal = Cancer | volume = 85 | issue = 11 | pages = 2305-14 | month = Jun | year = 1999 | doi =  | PMID = 10357398 }}</ref>
===Multiple myeloma===
Diagnosis requires the following:<ref name=pmid18971951>{{cite journal |author=Kyle RA, Rajkumar SV |title=Criteria for diagnosis, staging, risk stratification and response assessment of multiple myeloma |journal=Leukemia |volume=23 |issue=1 |pages=3–9 |year=2009 |month=January |pmid=18971951 |pmc=2627786 |doi=10.1038/leu.2008.291 |url=http://www.nature.com/leu/journal/v23/n1/full/leu2008291a.html}}</ref>
#Clonal plasma cells. Must >10% if on bone marrow biopsy.
#Monoclonal protein, i.e. paraprotein, in serum or urine.
#End-organ damage thought to be due to the neoplasm - mnemonic ''CARL'':
#*Calcium (in the serum) is elevated.
#*[[Anemia]].
#*Renal failure.
#*Lytic bone lesions.
Note:
*''CRAB'' (calclium, renal failure, anemia, bony lesions) is another mnemonic.<ref name=pmid12780789>{{Cite journal  | title = Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: a report of the International Myeloma Working Group. | journal = Br J Haematol | volume = 121 | issue = 5 | pages = 749-57 | month = Jun | year = 2003 | doi =  | PMID = 12780789 }}</ref>


==Microscopic==
==Microscopic==
Features:
Features (plasmacytoma):
*Abundant eosinophilic cytoplasm.
*Abundant eosinophilic cytoplasm.
*Eccentrically placed nucleus.
*Eccentrically placed nucleus.
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*Russell bodies:
*Russell bodies:
**Eosinophilic, large (10-15 micrometres), homogenous immunoglobulin-containing inclusions.  
**Eosinophilic, large (10-15 micrometres), homogenous immunoglobulin-containing inclusions.  
***Images: [http://commons.wikimedia.org/wiki/File:Russell_bodies_2_high_mag_mini.jpg Russell bodies (WC)], [http://www.healthsystem.virginia.edu/internet/hematology/hessimages/russell-bodies-website-arrow.jpg Russell bodies (healthsystem.virginia.edu)], [http://www.pathguy.com/lectures/russ2.jpg Russell bodies - several in one cell (pathguy.com)].
*Dutcher bodies - intranuclear crystalline rods.
*Dutcher bodies - intranuclear crystalline rods.
**Dutcher bodies are ''PAS stain'' +ve.<ref>URL: [http://www.thefreelibrary.com/Dutcher+bodies+in+chronic+synovitis-a083551789 http://www.thefreelibrary.com/Dutcher+bodies+in+chronic+synovitis-a083551789]. Accessed on: 4 August 2010.</ref>
**Dutcher bodies are ''PAS stain'' +ve.<ref>URL: [http://www.thefreelibrary.com/Dutcher+bodies+in+chronic+synovitis-a083551789 http://www.thefreelibrary.com/Dutcher+bodies+in+chronic+synovitis-a083551789]. Accessed on: 4 August 2010.</ref>
**Image [http://ashimagebank.hematologylibrary.org/cgi/content/full/2003/0227/100629 Dutcher bodies (hematologylibrary.org)].
*Prominent ''perinuclear hof'' - cytoplasmic crescent shaped lucency adjacent to the nuclear membrane (due to large Golgi apparatus); nucleus has a [http://en.wikipedia.org/wiki/Bib_%28garment%29 "bib"].
*Prominent ''perinuclear hof'' - cytoplasmic crescent shaped lucency adjacent to the nuclear membrane (due to large Golgi apparatus); nucleus has a [http://en.wikipedia.org/wiki/Bib_%28garment%29 "bib"].


Images:  
DDx:
*[[Neuroendocrine carcinoma]] - nucleus often has a plasmacytoid (plasma cell-like) appearance.
<!-- [http://ashimagebank.hematologylibrary.org/cgi/content/full/2003/0227/100629 Dutcher bodies (hematologylibrary.org)]. -->
*[[Lymphoplasmacytic lymphoma]] ([[AKA]] Waldenström's macroglobulinemia).
*[[Syphilis]].
 
===Images===
=====Case=====
<gallery>
Image: Plasma cell neoplasm -- low mag.jpg | PCN - low mag.
Image: Plasma cell neoplasm -- intermed mag.jpg | PCN - intermed. mag.
Image: Plasma cell neoplasm -- high mag.jpg | PCN - high mag.
Image: Plasma cell neoplasm -- very high mag.jpg | PCN - very high mag.
</gallery>
=====Other cases=====
<gallery>
Image:Plasmacytoma1.jpg | Plasmacytoma. (WC)
File:Plasmocytoma HE high mag.jpg | Skull base plasmacytoma, higher magnification (WC/jensflorian)
File:Plasmocytoma_frozen_HE_x200.jpg | Plasmacytoma smear (WC/jensflorian)
File:Plasmocytoma_smear_mann_x200.jpg | Leptomeningeal plasmacytoma, frozen section (WC/jensflorian)
</gallery>
=====www=====
*[http://ashimagebank.hematologylibrary.org/cgi/content/full/2004/0126/100984 Various images (hematologylibrary.org)].
*[http://ashimagebank.hematologylibrary.org/cgi/content/full/2004/0126/100984 Various images (hematologylibrary.org)].
*[http://commons.wikimedia.org/wiki/File:Plasmacytoma1.jpg Plasmacytoma (WC)].
*[http://path.upmc.edu/cases/case515/images/fig05.jpg Multiple myeloma (upmc.edu)].<ref>URL: [http://path.upmc.edu/cases/case515.html http://path.upmc.edu/cases/case515.html]. Accessed on: 25 January 2012.</ref>


DDx:
====Russell bodies====
*Neuroendocrine carcinoma - nucleus often has a plasmacytoid (plasma cell-like) appearance.
<gallery>
Image:Russell_bodies_2_high_mag_mini.jpg | Russell bodies. (WC)
</gallery>
=====www=====
*[http://www.healthsystem.virginia.edu/internet/hematology/hessimages/russell-bodies-website-arrow.jpg Russell bodies (healthsystem.virginia.edu)].
*[http://www.pathguy.com/lectures/russ2.jpg Russell bodies - several in one cell (pathguy.com)].
 
====Dutcher bodies====
<gallery>
Image: Dutcher and Russell bodies.jpg | Dutcher bodies and Russell bodies. (WC/Gabriel Caponetti)
</gallery>


==IHC==
==IHC==
*[[CD138]] +ve.
*[[CD56]] +ve.<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/116930 http://www.ncbi.nlm.nih.gov/omim/116930]. Accessed on: 31 August 2010.</ref>
**Also +ve in NK/T cell lymphomas.
*Kappa -- usu. slightly stronger than lambda.
*Kappa -- usu. slightly stronger than lambda.
*Lambda.
*Lambda.
*CD56.<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/116930 http://www.ncbi.nlm.nih.gov/omim/116930]. Accessed on: 31 August 2010.</ref>
**Also +ve in NK/T cell lymphomas.
*CD57.  
*CD57.  
**Also +ve in T-cell large granular lymphocytic leukemia.<ref>URL: [http://www.nature.com/bmt/journal/v33/n1/full/1704298a.html http://www.nature.com/bmt/journal/v33/n1/full/1704298a.html]. Accessed on: 31 August 2010.</ref>.
**Also +ve in [[T-cell large granular lymphocytic leukemia]].<ref>URL: [http://www.nature.com/bmt/journal/v33/n1/full/1704298a.html http://www.nature.com/bmt/journal/v33/n1/full/1704298a.html]. Accessed on: 31 August 2010.</ref>
*CD138.
*CD38 +ve.<ref>{{Cite journal  | last1 = Tamamori | first1 = T. | last2 = Nakayama | first2 = F. | last3 = Sugimoto | first3 = H. | last4 = Fenxiang | first4 = J. | last5 = Iwatsuki | first5 = K. | last6 = Takigawa | first6 = M. | title = Extramedullary plasmacytoma: cytological and genotypic studies. | journal = Br J Dermatol | volume = 129 | issue = 4 | pages = 468-72 | month = Oct | year = 1993 | doi =  | PMID = 8217765 }}</ref>
*CD38. (???)
 
Others:<ref>URL: [http://e-immunohistochemistry.info/web/Plasmacytoma_plasma_cell_myeloma.htm http://e-immunohistochemistry.info/web/Plasmacytoma_plasma_cell_myeloma.htm]. Accessed on: 9 November 2015.</ref>
*CD79a +ve.
*CD45 -ve/+ve.
*CD10 -ve.
*CD5 -ve.
*Cyclin D1 -ve/+ve.
 
A panel:<!-- need a good panel - maybe here http://clincancerres.aacrjournals.org/content/15/2/714.full or here http://e-immunohistochemistry.info/web/Plasmacytoma_plasma_cell_myeloma.htm -->
*CD3, CD20, CD56, CD117, CD138, IG-kappa (plasma), IG-lambda (plasma).


==Molecular==
==Molecular==
*t(4;14)(p16.3;q32.3) / IGH–MMSET.<ref name=pmid9787135>{{Cite journal  | last1 = Chesi | first1 = M. | last2 = Nardini | first2 = E. | last3 = Lim | first3 = RS. | last4 = Smith | first4 = KD. | last5 = Kuehl | first5 = WM. | last6 = Bergsagel | first6 = PL. | title = The t(4;14) translocation in myeloma dysregulates both FGFR3 and a novel gene, MMSET, resulting in IgH/MMSET hybrid transcripts. | journal = Blood | volume = 92 | issue = 9 | pages = 3025-34 | month = Nov | year = 1998 | doi =  | PMID = 9787135 }}</ref>
*t(4;14)(p16.3;q32.3) / IGH–MMSET.<ref name=pmid9787135>{{Cite journal  | last1 = Chesi | first1 = M. | last2 = Nardini | first2 = E. | last3 = Lim | first3 = RS. | last4 = Smith | first4 = KD. | last5 = Kuehl | first5 = WM. | last6 = Bergsagel | first6 = PL. | title = The t(4;14) translocation in myeloma dysregulates both FGFR3 and a novel gene, MMSET, resulting in IgH/MMSET hybrid transcripts. | journal = Blood | volume = 92 | issue = 9 | pages = 3025-34 | month = Nov | year = 1998 | doi =  | PMID = 9787135 }}</ref>
**Assoc. with poor prognosis.<ref name=pmid12393535>{{Cite journal  | last1 = Keats | first1 = JJ. | last2 = Reiman | first2 = T. | last3 = Maxwell | first3 = CA. | last4 = Taylor | first4 = BJ. | last5 = Larratt | first5 = LM. | last6 = Mant | first6 = MJ. | last7 = Belch | first7 = AR. | last8 = Pilarski | first8 = LM. | title = In multiple myeloma, t(4;14)(p16;q32) is an adverse prognostic factor irrespective of FGFR3 expression. | journal = Blood | volume = 101 | issue = 4 | pages = 1520-9 | month = Feb | year = 2003 | doi = 10.1182/blood-2002-06-1675 | PMID = 12393535 }}</ref>
**Associated with poor prognosis.<ref name=pmid12393535>{{Cite journal  | last1 = Keats | first1 = JJ. | last2 = Reiman | first2 = T. | last3 = Maxwell | first3 = CA. | last4 = Taylor | first4 = BJ. | last5 = Larratt | first5 = LM. | last6 = Mant | first6 = MJ. | last7 = Belch | first7 = AR. | last8 = Pilarski | first8 = LM. | title = In multiple myeloma, t(4;14)(p16;q32) is an adverse prognostic factor irrespective of FGFR3 expression. | journal = Blood | volume = 101 | issue = 4 | pages = 1520-9 | month = Feb | year = 2003 | doi = 10.1182/blood-2002-06-1675 | PMID = 12393535 }}</ref>
*13q deletions.<ref>{{Ref PCPBoD8|317}}</ref>
*13q deletion.
**Worse prognosis.<ref name=Ref_PCPBoD8_324>{{Ref PCPBoD8|324}}</ref>
*17q deletion.
**Worse prognosis.<ref name=Ref_PCPBoD8_324>{{Ref PCPBoD8|324}}</ref>


==See also==
==See also==
*[[Lymphoma]].
*[[Lymphoma]].
*[[Haematopathology]].
*[[Haematopathology]].
*[[Plasma cell]].


==References==
==References==
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[[Category:Haematopathology]]
[[Category:Haematopathology]]
[[Category:Diagnosis]]

Latest revision as of 17:22, 12 December 2016

Plasma cell neoplasms
Diagnosis in short

Plasma cell neoplasm. H&E stain
LM dyscohesive plasmacytoid cells (abundant cytoplasm, eccentrically placed nucleus), often prominent perinuclear hof, +/-nucleoli, +/-Russell bodies, +/-Dutcher bodies
LM DDx lymphoplasmacytic lymphoma, neuroendocrine carcinoma, poorly differentiated carcinoma, others
IHC CD138 +ve, CD56 +ve, CD45 -ve/+ve, CD79a +ve
Site bone - bone marrow
Associated Dx renal failure, myeloma cast nephropathy
Blood work +/-anemia, +/-hypercalcemia
Radiology +/-lytic bone lesions
Prognosis poor
Clin. DDx metastatic disease

Plasma cell neoplasms arise from plasma cells. They are encountered by anatomical pathologists on occasion.

Plasma cell myeloma, and plasmacytoma (solitary myeloma)[1] redirect to this article.

General

  • Malignancy derived from the plasma cells.
  • Prognosis: poor.
  • Common primary bone tumour in adults.

Clinical:[2]

  • Bence Jones protein (urine).
  • Abnormal protein electrophoresis (monoclonal gammopathy, dysproteinemia, paraproteinemia).

Note:

  • Plasmacytoma = histology of multiple myeloma; to diagnose multiple myeloma other (non-pathology) criteria are needed.

Classified by site:

  • Medullary.
  • Extramedullary - usu. upper aerodigestive tract.[3]

Multiple myeloma

Diagnosis requires the following:[4]

  1. Clonal plasma cells. Must >10% if on bone marrow biopsy.
  2. Monoclonal protein, i.e. paraprotein, in serum or urine.
  3. End-organ damage thought to be due to the neoplasm - mnemonic CARL:
    • Calcium (in the serum) is elevated.
    • Anemia.
    • Renal failure.
    • Lytic bone lesions.

Note:

  • CRAB (calclium, renal failure, anemia, bony lesions) is another mnemonic.[5]

Microscopic

Features (plasmacytoma):

  • Abundant eosinophilic cytoplasm.
  • Eccentrically placed nucleus.
    • Usually with "clock face" morphology.
      • "Clock face" morphology = chromatin clumps around the edge of the nucleus, like the numbers on a clock face.
    • May have nucleoli.
  • Russell bodies:
    • Eosinophilic, large (10-15 micrometres), homogenous immunoglobulin-containing inclusions.
  • Dutcher bodies - intranuclear crystalline rods.
    • Dutcher bodies are PAS stain +ve.[6]
  • Prominent perinuclear hof - cytoplasmic crescent shaped lucency adjacent to the nuclear membrane (due to large Golgi apparatus); nucleus has a "bib".

DDx:

Images

Case

  • PCN - low mag.

  • PCN - intermed. mag.

  • PCN - high mag.

  • PCN - very high mag.

Other cases

  • Plasmacytoma. (WC)

  • Skull base plasmacytoma, higher magnification (WC/jensflorian)

  • Plasmacytoma smear (WC/jensflorian)

  • Leptomeningeal plasmacytoma, frozen section (WC/jensflorian)

www

Russell bodies

  • Russell bodies. (WC)

www

Dutcher bodies

  • Dutcher bodies and Russell bodies. (WC/Gabriel Caponetti)

IHC

Others:[11]

  • CD79a +ve.
  • CD45 -ve/+ve.
  • CD10 -ve.
  • CD5 -ve.
  • Cyclin D1 -ve/+ve.

A panel:

  • CD3, CD20, CD56, CD117, CD138, IG-kappa (plasma), IG-lambda (plasma).

Molecular

  • t(4;14)(p16.3;q32.3) / IGH–MMSET.[12]
    • Associated with poor prognosis.[13]
  • 13q deletion.
    • Worse prognosis.[1]
  • 17q deletion.
    • Worse prognosis.[1]

See also

References

  1. ↑ 1.0 1.1 1.2 Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 324. ISBN 978-1416054542.
  2. ↑ Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 323. ISBN 978-1416054542.
  3. ↑ Alexiou, C.; Kau, RJ.; Dietzfelbinger, H.; Kremer, M.; Spiess, JC.; Schratzenstaller, B.; Arnold, W. (Jun 1999). "Extramedullary plasmacytoma: tumor occurrence and therapeutic concepts.". Cancer 85 (11): 2305-14. PMID 10357398.
  4. ↑ Kyle RA, Rajkumar SV (January 2009). "Criteria for diagnosis, staging, risk stratification and response assessment of multiple myeloma". Leukemia 23 (1): 3–9. doi:10.1038/leu.2008.291. PMC 2627786. PMID 18971951. http://www.nature.com/leu/journal/v23/n1/full/leu2008291a.html.
  5. ↑ "Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: a report of the International Myeloma Working Group.". Br J Haematol 121 (5): 749-57. Jun 2003. PMID 12780789.
  6. ↑ URL: http://www.thefreelibrary.com/Dutcher+bodies+in+chronic+synovitis-a083551789. Accessed on: 4 August 2010.
  7. ↑ URL: http://path.upmc.edu/cases/case515.html. Accessed on: 25 January 2012.
  8. ↑ URL: http://www.ncbi.nlm.nih.gov/omim/116930. Accessed on: 31 August 2010.
  9. ↑ URL: http://www.nature.com/bmt/journal/v33/n1/full/1704298a.html. Accessed on: 31 August 2010.
  10. ↑ Tamamori, T.; Nakayama, F.; Sugimoto, H.; Fenxiang, J.; Iwatsuki, K.; Takigawa, M. (Oct 1993). "Extramedullary plasmacytoma: cytological and genotypic studies.". Br J Dermatol 129 (4): 468-72. PMID 8217765.
  11. ↑ URL: http://e-immunohistochemistry.info/web/Plasmacytoma_plasma_cell_myeloma.htm. Accessed on: 9 November 2015.
  12. ↑ Chesi, M.; Nardini, E.; Lim, RS.; Smith, KD.; Kuehl, WM.; Bergsagel, PL. (Nov 1998). "The t(4;14) translocation in myeloma dysregulates both FGFR3 and a novel gene, MMSET, resulting in IgH/MMSET hybrid transcripts.". Blood 92 (9): 3025-34. PMID 9787135.
  13. ↑ Keats, JJ.; Reiman, T.; Maxwell, CA.; Taylor, BJ.; Larratt, LM.; Mant, MJ.; Belch, AR.; Pilarski, LM. (Feb 2003). "In multiple myeloma, t(4;14)(p16;q32) is an adverse prognostic factor irrespective of FGFR3 expression.". Blood 101 (4): 1520-9. doi:10.1182/blood-2002-06-1675. PMID 12393535.
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