Plasma cell neoplasms

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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
Other cases
www

Russell bodies

www

Dutcher bodies

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.