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Surgical Pathology Criteria
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Osteosclerotic Myeloma

Definition

  • Plasmacytoma with osteosclerosis accompanied by clinical POEMS syndrome and lymph node changes resembling plasma cell variant of Castleman disease

Alternate/Historical Names

  • POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, Skin changes)
  • Multicentric Castleman disease
  • Crowe-Fukase syndrome

Diagnostic Criteria

  • Osteosclerotic plasmacytoma
    • Plasma cells entrapped in fibrosis
    • Thickened trabecular bone
    • Usually lambda light chain restricted
    • Usually does not meet criteria for classical myeloma
      • Bone marrow plasmacytosis usually <5-10%, if present
      • Serum M protein usually <3.5 g/dL, if present
      • Bence Jones protein usually <1 g/24hr, if present
  • Lymph nodes may show changes of multicentric plasma cell type Castleman disease
    • Reactive follicular hyperplasia
    • Interfollicular plasmacytosis
    • Some follicles with hyaline vascular changes
  • Clinical findings of POEMS syndrome present
    • Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, Skin changes
  • Serum VEGF often markedly elevated
    • (Under consideration as a diagnostic criterion)

Dita Gratzinger MD PhD

Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342

Initial posting/updates : 9/1/07, 4/15/10

Supplemental studies

  • Clonal plasma cells can be demonstrated by flow, immunohistology or in situ hybridization
    • Usually lambda light chain
    • Bone marrow plasmacytosis usually <5-10%, if present

Useful Laboratory Tests

  • Serum or urine protein electrophoresis, immunofixation, light chain quantification
    • Serum M protein usually <3.5 g/dL, if present
    • Bence Jones protein usually <1 g/24hr, if present
  • Quantitation and typing of monoclonal immunoglobulin / light chain
    • Usually IgA or IgG lambda
  • Serum free light chain analysis may be required to demonstrate clonal light chains
  • These studies may be used to
    • Establish presence of a monoclonal plasma cell population
    • Quantitation helps subtype the plasma cell dyscrasia (i.e. >3g/dL serum monoclonal protein is a major criterion for myeloma)
    • Track disease burden over time
  • Serum VEGF often markedly elevated

 

Differential Diagnosis

Osteosclerotic Myeloma Myeloma Plasmacytoma
Osteosclerotic lesion Osteolytic lesions Osteolytic lesion
<5% plasma cells usually >> 5% plasma cells < 5% plasma cells
POEMS syndrome present No POEMS syndrome No POEMS syndrome
Castleman like node changes may be present No Castleman changes No Castleman changes

  • POEMS = Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, Skin changes
  • Hematolymphoid Disorders with Marrow Fibrosis (overview)
    Disorder Distinguishing Feature(s)
    Polycythemia Vera, Fibrotic Phase History of PV / JAK2+
    Essential Thrombocythemia, Fibrotic Phase (rare) History of ET / may be JAK2+
    CML, Fibrotic Phase History of CML / BCR-ABL1+
    MDS with Fibrosis Dysplastic features in marrow
    Acute Megakaryoblastic Leukemia Blasts, acute onset
    Osteosclerotic Myeloma Plasma cells. light chain restricted
    Acute Pan-myelosis with Myelofibrosis Bone pain, acute onset
    Primary Myelofibrosis Clustered atypical megakaryocytic hyperplasia
    Hairy cell leukemia Clonal B cells
    MDS with Fibrosis = descriptive term that includes primary MDS (often RAEB2) with fibrosis and therapy related myeloid neoplasms with fibrosis

    Clinical

    • Rare, apparently more prevalent in Japan
    • Median age 50 years
    • May be associated with HHV8 in setting of multicentric Castleman disease
    • Signs and symptoms of POEMS syndrome
      • Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, Skin changes
      • Occasional findings
        • Edema/effusions/ascites, papilledema, clubbing
        • Thrombocytosis, polycythemia
        • Bone pain, fatigue
    • Better prognosis than usual myeloma
      • Median survival >14 years

    Grading / Staging / Report

    • Grading is not applicable

    Durie-Salmon Staging System

    • Stage I
      • Hemoglobin >10 g/dL
      • Serum IgG <5 g/dL
      • Serum IgA <3 g/dL
      • Normal serum calcium
      • Urine monoclonal protein excretion <4 g/day
      • No generalized lytic bone lesions
    • Stage II
      • Intermediate between stage I and III
    • Stage III
      • Hemoglobin <8.5 g/dL
      • Serum IgG >7 g/dL
      • Serum IgA >5 g/dL
      • Serum calcium >12 g/dL
      • Urine monoclonal protein excretion >12 g/day
      • Advanced lytic bone lesions

    The pathology report should contain the following information:

    • Diagnosis in the World Health Organization (WHO) classification
      • Equivalent diagnosis in other classifications used by relevant clinicians
    • Results of supplementary studies if performed
    • Relationship to other specimens from the same patient
    • Information relevant to staging if available

     

    Lists

    Plasma cell neoplasms / Immunosecretory disorders (WHO 2008)

    Bibliography

    • Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW . WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, International Agency for Research on Cancer, Lyon, 2008
    • Warnke RA, Weiss LM, Chan JKC, Cleary ML, Dorfman RF . Tumors of the Lymph Nodes and Spleen, Atlas of Tumor Pathology, AFIP Third Series, Fascicle 14, 1995
    • Dispenzieri A.POEMS syndrome. Blood Rev. 2007 Nov;21(6):285-99.
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