eMedicine Specialties > Hematology > Plasma Cell Disorders

Plasmacytoma, Extramedullary

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 follows^4,5

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 rate^8,9 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

Differential Diagnoses

Lymphoma, High-Grade Malignant Immunoblastic
Lymphoma, Non-Hodgkin
Marginal zone B-cell lymphoma
Plasma cell granuloma
Poorly differentiated neoplasms
Reactive plasmacytosis

Workup

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.^{21,22,23,24,25,26,27,28}
- 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.

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.²⁹ The sclerotic lesion is associated with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome.³⁰
- 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).³¹
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.³²

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.
- 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.³³
- Bone marrow biopsy shows less than 5% plasma cells without evidence of clonality.⁶

Staging

Wiltshaw classified soft-tissue plasmacytoma into 3 clinical stages, as follows⁷:
- 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.

Treatment

Medical Care

Solitary bone plasmacytoma (SBP)
- Local radiotherapy is the treatment of choice.^4,5,13,22 Treatment fields should be designed to encompass all disease observed on MRI and should include a margin of healthy tissue (at least 2 cm). For spinal lesions, the margins should include at least 1 uninvolved vertebra.
- Local control is achieved in 88-100% of patients. Virtually all patients have major symptom relief.¹³ and a local tumor recurrence rate of approximately 10%.
- Most centers use approximately 40 Gy for spinal lesions and 45 Gy for other bone lesions. For lesions larger than 5 cm, 50 Gy should be considered.
- No dose-response relationship between radiation dose and disappearance of monoclonal protein was noted in a series of patients with solitary bone plasmacytoma as reported by Liebross et al.²²
- Monoclonal protein is markedly reduced after radiotherapy in the majority of patients, but protein disappearance is observed in only 20-50% of patients.¹⁴
- Surgery is contraindicated in the absence of structural instability or neurologic compromise.¹²
- Chemotherapy may be considered for patients not responding to radiation therapy. Regimens useful in multiple myeloma can be considered.¹²
- No role exists for adjuvant chemotherapy in solitary bone plasmacytoma (SBP).
Extramedullary plasmacytoma (EMP)
- Based on the documented radiation sensitivity of plasma cell tumors, the accepted treatment is radiotherapy.
- When a lesion can be completely resected, surgery provides the same results as radiotherapy.
- Combined therapy (surgery and radiotherapy) is an accepted treatment depending on the resectability of the lesion.^4,6,13,14,15 In fact, combination treatment may provide the best results.¹⁴
- The optimal dose for local control is 40-50 Gy (depending on tumor size) delivered over 4-6 weeks.^13,14,15
- Because of the high rate of lymph node involvement, these areas should be included in the radiation field.¹³
- Adjuvant radiotherapy should be recommended to patients with positive surgical margins.
- Chemotherapy may be considered for patients with refractory or relapsed disease. Regimens used for multiple myeloma can be considered.¹²
- Adjuvant chemotherapy may be considered for patients with tumors larger than 5 cm, as well as those with high-grade histology.

Surgical Care

Although surgical resection is not advised for the treatment of solitary bone plasmacytoma (SBP), spine instrumentation or another procedure is sometimes necessary to try to reestablish the normal architecture of the spine or other bone affected.
If possible, a complete resection of the lesion, including lymph node dissection, should be attempted for soft-tissue plasmacytomas.

Consultations

Orthopedic evaluation is recommended for patients with solitary bone plasmacytoma (SBP) because lesions may cause spinal cord compression syndrome or impending fractures. Therapeutic procedures, such as kyphoplasty, can be implemented in order to restore vertebral structure.
An ear, nose, and throat evaluation is recommended for patients with extramedullary plasmacytoma (EMP) of the head and neck to precisely localize the lesion, obtain an adequate biopsy (including lymph nodes), and plan possible resection.

Medication

No defined role exists for chemotherapy treatment of solitary bone plasmacytoma (SBP) or extramedullary plasmacytoma (EMP), as mentioned above.

Follow-up

Further Outpatient Care

Periodic evaluation for progression and development of multiple myeloma is recommended every 6 weeks for the first 6 months for solitary bone plasmacytoma (SBP) and extramedullary plasmacytoma (EMP), with extension of clinic appointments thereafter. Besides a complete history and physical examination, the following tests are recommended:
- Complete blood cell (CBC) count
- Complete metabolic panel with lactic dehydrogenase (LDH), calcium, phosphorus, C-reactive protein (CRP), and beta2 microglobulin
- Erythrocyte sedimentation rate (ESR)
- Serum protein electrophoresis with immunofixation
- Serum immunoglobulin quantification
- Urinary protein electrophoresis with immunofixation (24-h urine sample)
- Skeletal bone survey
Orthopedic follow-up is recommended for solitary bone plasmacytoma (SBP) and/or ear-nose-throat follow-up is recommended extramedullary plasmacytoma (EMP), based on the tumor location.

Prognosis

Solitary bone plasmacytoma (SBP)
- Solitary bone plasmacytoma (SBP) progresses to multiple myeloma at a rate of 65-84% at 10 years and 65-100% at 15 years.
- The median onset of conversion to multiple myeloma is 2-5 years with a 10-year disease-free survival rate of 15-46%.
- The overall median survival time is 10 years.¹³
- Kyle described 3 patterns of treatment failure¹⁶: development of multiple myeloma (54%), local recurrence (11%), and development of new bone lesions in the absence of multiple myeloma (2%).
- Prognostic features for conversion of solitary bone plasmacytoma (SBP) to multiple myeloma, although controversial, include the following^1,22,27:
  - Lesion size of at least 5 cm
  - Patients aged 40 years and older
  - High M protein levels
  - Persistence of M protein after treatment
  - Spine lesions
- In a study by Wilder et al, 10-year myeloma-free survival was 91% versus 29% in patients whose M-protein did or did not resolve at 1 year following radiation therapy.³⁴
Extramedullary plasmacytoma (EMP)
- The 10-year overall survival rate is 70%.¹²
- The rate of progression to multiple myeloma is lower than in solitary bone plasmacytoma (SBP), ranging from 11% to 30% at 10 years.¹³ Patients with extramedullary plasmacytoma who progressed to multiple myeloma had a 5-year survival rate of 100%, compared with 33% for solitary bone plasmacytoma.³⁵ In a review of 721 cases by Alexiou and colleagues, after treatment, approximately 65% of patients were free of recurrence and did not progress to multiple myeloma, 22% experienced recurrence, and 15% of cases evolved to multiple myeloma.¹⁴
- In one study, local control following radiation therapy was achieved in 83% of patients with low-grade histology versus 17% of patients with intermediate- to high-grade tumors.³³

Miscellaneous

Medicolegal Pitfalls

Failure to diagnose spinal cord compression syndrome and pathologic and/or impending fractures can cause irreversible neurologic damage (eg, paraplegia, bowel or bladder dysfunction), chronic pain, or disabling musculoskeletal damage.
Inappropriate follow-up or failure to evaluate progression to multiple myeloma delays institution of appropriate therapy to control the well-known systemic complications of multiple myeloma (eg, generalized bone destruction, hypercalcemia, anemia, hyperviscosity, infections, amyloid).

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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

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.

Acknowledgments

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.

Further Reading