History

Solitary bone plasmacytoma

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.^{[7, 18]}

Compression fractures of the thoracic and lumbar vertebral bodies usually result in severe spasms and back pain. Patients with significant vertebral involvement may also have evidence of nerve root or spinal cord compression.^[7]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.

Extramedullary plasmacytoma

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 the following^{[13, 25, 26, 27]}:

Headache
Nasal discharge
Epistaxis
Nasal obstruction
Sore throat
Hoarseness
Dysphonia
Dysphagia
Dyspnea
Epigastric pain
Hemoptysis

Gastrointestinal EMPs can present as loss of appetite, bleeding, and abdominal discomfort or obstruction.^[11]

Symptoms from EMP in other tissues are associated with the site of the tumor, tumor size, and compression and/or involvement of the surrounding structures.

EMP involving the lung most commonly presents as a pulmonary nodule or hilar mass.

Solitary bone plasmacytoma

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 the lumbar, sacral, and cervical vertebrae.^[10] The rib, sternum, clavicle, or scapula is involved in 20% of cases.^[28] Physical findings are related to the site of involvement, patients may present with a painful mass, pathologic fracture (in those with long bone involvement^[10]), or root or spinal cord compression syndrome.

Occasionally, patients with SBP may present with peripheral polyneuropathy,^{[29, 30]} or with features that are consistent with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes).^[31]

Extramedullary plasmacytoma

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.^{[10, 13, 3, 15, 16]} 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 describe involvement of the following^{[10, 13, 3, 15, 16, 32]}:

Urinary bladder
Central nervous system
Orbit,
Gastrointestinal tract
Liver
Spleen
Pancreas
Lung
Breast,
Skin
Testis
Parotid gland
Mediastinum
Thyroid gland (associated with goiter and Hashimoto thyroiditis)

In 30-40% of cases, local lymph nodes are involved at presentation or upon relapse.^[14]

Differential Diagnoses

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Author

Suzanne R Fanning, DO Director of Hematology, Greenville Memorial Health System; 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, American Society of Hematology

Disclosure: Received consulting fee from Millenium Pharmaceuticals for review panel membership; Received consulting fee from Celgene Pharmaceuticals for 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, American Society of Hematology

Disclosure: Received income in an amount equal to or greater than $250 from: Celgene corporation.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, 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 Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

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 Society of Hematology

Disclosure: Nothing to disclose.

Acknowledgements

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.

Fernando Perez-Zincer, MD Senior Fellow, Department of Hematology and Medical Oncology, The Cleveland Clinic Foundation Cancer Center

Disclosure: Nothing to disclose.