eMedicine Specialties > Hematology > Plasma Cell Disorders

Plasmacytoma, Extramedullary

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
Coauthor(s): Mohamad A Hussein, MD, Clinical Director, Malignant Hematology, Moffitt Cancer Center
Contributor Information and Disclosures

Updated: Sep 16, 2008

Introduction

Background

A plasmacytoma is a discrete, solitary mass of neoplastic monoclonal plasma cells in either bone or soft tissue (extramedullary). The types of plasmacytomas are as follows:

  • Soft-tissue or nonosseous extramedullary plasmacytoma (EMP)
  • Solitary bone plasmacytoma (SBP)
  • Multifocal form of multiple myeloma
  • Multiple myeloma
  • Plasmablastic sarcoma

To simplify, solitary plasmacytomas can be divided into 2 groups according to location:

  • Plasmacytoma of the skeletal system (SBP)
  • Extramedullary plasmacytoma (EMP)

Diagnostic criteria for solitary bone plasmacytoma (SBP)

Criteria for identifying solitary bone plasmacytoma (SBP) vary among authors.1,2,3 Some include patients with more than one lesion and elevated levels of myeloma protein and exclude patients whose disease progressed within 2 years or whose abnormal protein persisted after radiotherapy. With the use of magnetic resonance imaging (MRI), flow cytometry, and polymerase chain reaction (PCR), the currently accepted criteria are as follows4,5

  • Single area of bone destruction due to clonal plasma cells
  • Bone marrow plasma cell infiltration not exceeding 5% of all nucleated cells
  • Absence of osteolytic bone lesions or other tissue involvement (no evidence of myeloma)
  • Absence of anemia, hypercalcemia, or renal impairment attributable to myeloma
  • Low, if present, concentrations of serum or urine monoclonal protein
  • Preserved levels of uninvolved immunoglobulins

Extramedullary plasmacytoma (EMP): Diagnostic criteria

Diagnostic criteria for extramedullary plasmacytoma (EMP) are as follows6 :

  • Tissue biopsy showing monoclonal plasma cell histology
  • Bone marrow plasma cell infiltration not exceeding 5% of all nucleated cells
  • Absence of osteolytic bone lesions or other tissue involvement (no evidence of myeloma)
  • Absence of hypercalcemia or renal failure
  • Low serum M protein concentration, if present

For excellent patient education resources, visit eMedicine's Blood and Lymphatic System Center. Also, see eMedicine's patient education article Myeloma.

Pathophysiology

A plasmacytoma can arise in any part of the body. A solitary bone plasmacytoma (SBP) arises from the plasma cells located in the bone marrow, whereas extramedullary plasmacytoma (EMP) is thought to arise from plasma cells located in mucosal surfaces.7 Both represent a different group of neoplasms in terms of location, tumor progression, and overall survival rate8,9  Some authors suggest a solitary bone plasmacytoma (SBP) represents marginal cell lymphomas with extensive plasmacytic differentiation.9 Both solitary bone plasmacytoma (SBP) and extramedullary plasmacytoma (EMP) do, however, share many of the biologic features of other plasma cell disorders.

Cytogenetic studies show recurrent losses in chromosome 13, chromosome arm 1p, and chromosome arm 14q, as well as gains in chromosome arms 19p, 9q, and 1q.10  Interleukin 6 (IL-6) is still considered the principal growth factor in the progression of plasma cell disorders.5

The specific roles of surface markers, adhesion molecules, and angiogenesis in solitary plasmacytoma need to be studied further.

In a study by Kumar et al, high-grade angiogenesis in the solitary bone plasmacytoma (SBP) was associated with increased progression to multiple myeloma and shorter progression-free survival. Some have postulated that solitary bone plasmacytoma (SBP) may be considered an intermediate step in the evolution from monoclonal gammopathy of undetermined significance to multiple myeloma.5

Frequency

United States

Solitary bone plasmacytoma (SBP) affects fewer than 5% of patients with plasma cell disorders.4,5 In a series of 263 patients with MGUS, 2 patients (0.8%) developed solitary bone plasmacytoma (SBP).11

Extramedullary plasmacytoma (EMP) represents approximately 3% of all plasma cell neoplasms.

Mortality/Morbidity

  • Solitary bone plasmacytoma (SBP) develops into multiple myeloma in 50-60% of patients.12 Median overall survival time is 10 years.13
  • Extramedullary plasmacytoma (EMP) progresses to multiple myeloma in 11-30% of patients at 10 years. Overall survival rate at 10 years is 70%.

Sex

  • Solitary bone plasmacytoma (SBP) has a male-to-female ratio of 2:1.
  • Three fourths of extramedullary plasmacytoma (EMP) cases involve males.6,7,13,14,15

Age

  • The median age of patients with either solitary bone plasmacytoma (SBP) or extramedullary plasmacytoma (EMP) is 55 years.
  • This median age is 10 years younger than patients with multiple myeloma.6,7,13,14,15

Clinical

History

See Physical.

  • Solitary bone plasmacytoma (SBP)
    • The most common symptom of solitary bone plasmacytoma (SBP) is pain at the site of the skeletal lesion due to bone destruction by the infiltrating plasma cell tumor.5,16
    • Compression fractures of the thoracic and lumbar vertebral bodies usually result in severe spasms and back pain. Patients with important vertebral involvement may also have evidence of nerve root or spinal cord compression.5 Spinal cord compression represents an emergency that requires immediate diagnosis and treatment to avoid permanent neurologic damage (eg, paraplegia, bowel and bladder dysfunction, chronic pain).
    • Pleuritic pain from pathologic rib and clavicular fractures are associated with marked local tenderness.
  • Solitary extramedullary plasmacytoma (EMP)
    • Extramedullary plasmacytoma (EMP) presents as a mass growing in the aerodigestive tract in 80-90% of patients, often with spread to lymph nodes, although other sites are affected as well.
    • Common complaints include swelling, headache, nasal discharge, epistaxis, nasal obstruction, sore throat, hoarseness, dysphonia, dysphagia, dyspnea, epigastric pain, and hemoptysis.6
    • Symptoms from extramedullary plasmacytoma (EMP) in other tissues are associated with the site of the tumor, tumor size, and compression and/or involvement of the surrounding structures.
    • Extramedullary plasmacytoma (EMP) involving the lung most commonly presents as a pulmonary nodule or hilar mass.

Physical

  • Solitary bone plasmacytoma (SBP)
    • Solitary bone plasmacytoma (SBP) may involve any bone, but it has a predisposition for the red marrow–containing axial skeleton. Spinal disease is observed in 34-72% of cases. The thoracic vertebrae are most commonly involved, followed by lumbar, sacral, and cervical vertebrae.4 The rib, sternum, clavicle, or scapula is involved in 20% of cases.17 Physical findings are related to the site of involvement, presenting as a painful mass, pathologic fracture, or root or spinal cord compression syndrome.
    • Patients with long bone involvement may present with pathologic fracture.4
    • Occasionally, patients with solitary bone plasmacytoma (SBP) may present with peripheral polyneuropathy.18,19 or with features that are consistent with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes).20
  • Extramedullary plasmacytoma (EMP)
    • Although extramedullary plasmacytoma (EMP) can occur in any site, 80-90% of tumors develop in the head and neck area, especially in the aerodigestive tract.
    • Approximately 80% of cases involve the paranasal sinuses, pharynx, nasal cavity, or gums and oral mucosa.4,6,7,14,15 A mass (plasmacytoma) in these areas is the most common finding, with compression or invasion of the surrounding structures. Patients with tumors involving the base of the skull may present with cranial nerve palsies.
    • Case reports of involvement of the urinary bladder, central nervous system, orbit, gastrointestinal tract, liver, spleen, pancreas, lung, breast, skin, testis, parotid gland, mediastinum, and thyroid gland (associated with goiter and Hashimoto thyroiditis) exist.4,6,7,14,15
    • In 30-40% of cases, local lymph nodes are involved at presentation or upon relapse.13

Causes

  • No definite cause has been found for solitary bone plasmacytoma (SBP).
  • Because of its presentation in the mucosa of the aerodigestive tract (>80%), the etiology of extramedullary plasmacytoma (EMP) may be related to chronic stimulation of inhaled irritants or viral infection.7

More on Plasmacytoma, Extramedullary

Overview: Plasmacytoma, Extramedullary
Differential Diagnoses & Workup: Plasmacytoma, Extramedullary
Treatment & Medication: Plasmacytoma, Extramedullary
Follow-up: Plasmacytoma, Extramedullary
References
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Further Reading

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Keywords

extramedullary plasmacytoma, plasma cell tumor, plasmocytoma, plasmacytoma of the skeletal system, solitary bone plasmacytoma, SBP, soft tissue plasmacytoma, EMP, neoplastic monoclonal cell, bone marrow, multiple myeloma

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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.

Medical Editor

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

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

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.

CME Editor

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, 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 Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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