Extramedullary Plasmacytoma Workup

  • Author: Suzanne R Fanning, DO; Chief Editor: Emmanuel C Besa, MD   more...
 
Updated: Nov 22, 2011
 

Laboratory Studies

  • Solitary bone plasmacytoma (SBP)
    • Although levels are lower than in multiple myeloma, electrophoresis reveals a monoclonal protein in the serum or urine in 24-72% of patients.[22, 23, 24, 25, 26, 27, 28, 29]
    • Uninvolved immunoglobulin levels are usually within the reference range.
    • Peripheral blood cell count, renal function, and calcium are within the reference range.
  • Extramedullary plasmacytoma (EMP)
    • Protein electrophoresis shows a monoclonal component in 14-25% of cases.[4, 6, 15] In a series of 46 patients by Galieni and colleagues, all patients had normal uninvolved immunoglobulins.[6]
    • Peripheral blood cell count, renal function, and calcium are within the reference range.
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Imaging Studies

  • Solitary bone plasmacytoma (SBP)
    • On plain radiographs, solitary bone plasmacytoma (SBP) classically has a lytic appearance with clear margins and a narrow zone of transition to healthy surrounding bone.[5] Rare occurrences of a cyst, a trabeculated lesion resembling a giant cell tumor or an aneurysmal bone cyst, and sclerotic lesions have been described.[30] The sclerotic lesion is associated with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome.[31]
    • On MRI, the solitary bone plasmacytoma (SBP) exhibits abnormal signal intensity (low on T1-weighted imaging and high on T2-weighted or short tau inversion recovery [STIR] images) that, in the appropriate clinical setting, is consistent with solitary bone plasmacytoma (SBP).[32]
  • Extramedullary plasmacytoma (EMP)
    • Radiographic assessment shows local bone destruction in most patients with nasal cavity or maxillary sinus involvement.[15]
    • Computed tomography (CT) scanning, MRI, and complete endoscopic examination of the aerodigestive and gastrointestinal tracts are required to determine the exact extent of the tumor and its potential for resectability.[14] These lesions may be associated with variable mass effect, infiltration and/or destruction of adjacent bone, muscle, fat, or vascular encasement.[33]
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Histologic Findings

  • Solitary bone plasmacytoma (SBP): Biopsy of the lesion reveals infiltration of the bone by monoclonal plasma cells.
  • Extramedullary plasmacytoma (EMP): Biopsy of the soft-tissue lesion shows infiltration by monoclonal plasma cells.[34]
    • In extramedullary plasmacytoma (EMP), the soft-tissue lesion commonly exhibits submucosal growth, requiring deep biopsy, open biopsy, or complete excision depending on the tumor location.[14]
    • Histologically, extramedullary plasmacytoma (EMP) may be classified as low, intermediate, or high grade.[35]
    • Bone marrow biopsy shows less than 5% plasma cells without evidence of clonality.[6]
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Staging

  • Wiltshaw classified soft-tissue plasmacytoma into 3 clinical stages, as follows[7] :
    • Stage I – Limited to an extramedullary site
    • Stage II – Involvement of regional lymph nodes
    • Stage III – Multiple metastasis (although it is no longer a solitary plasmacytoma)
  • The therapeutic and prognostic value of this classification needs further evaluation.
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Contributor Information and Disclosures
Author

Suzanne R Fanning, DO  Fellow, Department of Hematology and Medical Oncology, Cleveland Clinic Foundation, 2004-2007 Director, Hematology, Greenville Memorial Health System, Greenville, SC Medical Oncologist/Hematologist/Transplant Physician, Cancer Centers of the Carolinas

Suzanne R Fanning, DO is a member of the following medical societies: American College of Physicians, American Medical Association, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, and American Society of Hematology

Disclosure: Millenium Pharmaceuticals Consulting fee Review panel membership; Celgene Pharmaceuticals Consulting fee Review panel membership

Coauthor(s)

Mohamad A Hussein, MD  Clinical Director, Malignant Hematology, Moffitt Cancer Center

Mohamad A Hussein, MD is a member of the following medical societies: American Association of Blood Banks, American College of Physicians, American Medical Association, and American Society of Hematology

Disclosure: Nothing to disclose.

Specialty Editor Board

Paul Schick, MD  Emeritus Professor, Department of Internal Medicine, Jefferson Medical College of Thomas Jefferson University; Research Professor, Department of Internal Medicine, Drexel University College of Medicine; Adjunct Professor of Medicine, Lankenau Hospital

Paul Schick, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Society of Hematology, International Society on Thrombosis and Haemostasis, and New York Academy of Sciences

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Wendy Hu, MD  Consulting Staff, Department of Hematology/Oncology and Bone Marrow Transplantation, Huntington Memorial Medical Center

Wendy Hu, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Physicians, American Society for Blood and Marrow Transplantation, American Society of Hematology, and Physicians for Social Responsibility

Disclosure: Nothing to disclose.

Rajalaxmi McKenna, MD, FACP  Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD  Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Clinical Oncology, American Society of Hematology, and New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous coauthor Dr Fernando Perez-Zincer to the development and writing of this article.

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