Plasma cell neoplasms

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

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 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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