Plasmacytoma

What is plasmacytoma?

Plasmacytoma is a very rare condition that is similar to multiple myeloma. Like multiple myeloma, plasmacytoma happens when plasma cells, sometimes called B cells, turn into abnormal cells that multiply and become single tumors that affect your bones, soft tissues in your head and neck, or any organ in your body like your bladder, lung or kidney. There are two types of plasmacytomas — solitary plasmacytoma of bone (SPB) and extramedullary plasmacytoma (EMP).

How do plasmacytomas affect my body?

Like multiple myeloma, plasmacytomas develop when healthy plasma cells turn into abnormal cells. Plasma cells are white blood cells and are part of your immune system. Healthy plasma cells (sometimes called B cells) make antibodies. These antibodies, called immunoglobulins, help fight infection and protect against illness. When healthy plasma cells become abnormal, they multiply and produce abnormal antibodies called M proteins. When M proteins multiply, your body has fewer normal plasma cells helping to protect against infection.

There are plasmacytoma types that can affect different parts of your body:

• Solitary plasmacytoma of bone (SPB): This happens when abnormal plasma cells form a tumor on one spot or site on one of your bones. SPB causes bone damage and pain. Approximately 50% of people who have SPB develop multiple myeloma.
• Extramedullary plasmacytoma (EMP): In EMP, a single tumor made of abnormal plasma cells affects soft tissue. Soft tissue connects, supports and surrounds your organs and bones. Your muscles, tendons, skin, fat and layers of connective tissue called fascia are examples of soft tissue. EMP can affect soft tissue throughout your body but typically affects your upper respiratory tract, which includes your nasal cavity, sinuses, nasopharynx and larynx, but can affect any organ. Approximately 15% of people who have EMP develop multiple myeloma.

Who is affected by plasmacytomas?

Plasmacytomas typically affect people ages 55 to 60. People assigned male at birth are more likely to develop plasmacytomas than those assigned female at birth.

How common is plasmacytomas?

Plasmacytoma is very rare. Each year, healthcare providers diagnose 450 cases of SPB and approximately 300 cases of EMP. However, people who've been battling multiple myeloma for some time may also develop plasmacytomas.

What are SPB symptoms?

Solitary plasmacytomas of bone cause bone pain or bone fracture. Other SPB symptoms include:

• Pain or bone fractures in your rib bones, thoracic vertebrae, femur and pelvis.
• Compression fractures in your spine, which may damage your spinal cord or nerve root. This can cause sharp, stabbing pains that may shoot down your legs and spine.
• Rarely, SPB can affect your skull, causing headaches, dizziness and vision problems.

What are EMP symptoms?

Extramedullary plasmacytomas symptoms may happen when the tumor presses on soft tissue, causing pain or affecting how your body functions. For example, an EMP in your nose may feel like there’s something stuck there that makes it hard for you to breathe through your nose. EMP may appear anywhere in your body, but 80% to 90% of EMPs appear in your head and neck. EMP symptoms include:

• Headache.
• Nasal discharge.
• Dysphagia (difficulty swallowing).
• Pharyngitis (sore throat).
• Epistaxis (nosebleed).

Rarely, EMP may affect your larynx (voice box), causing the following symptoms:

• Hoarseness.
• Obstructed airway.
• Wheezing.

Many plasmacytoma symptoms resemble common problems that may not be signs of serious illness. That said, you should contact your healthcare provider anytime you have symptoms that last longer than two weeks or get worse.

What causes plasmacytomas?

Plasmacytomas develop when healthy plasma cells turn into abnormal cells. Researchers don’t know what triggers this change.

How do healthcare providers diagnose plasmacytoma?

Healthcare providers use several tests to diagnose the different plasmacytoma types. They use what they learn to establish EMP stages.

• Computed tomography (CT) scan: This test uses a series of X-rays and a computer to produce a 3D image of soft tissues and bones. Providers use CT scans to look for possible bone damage caused by plasmacytoma.
• Magnetic resonance imaging (MRI): This test produces detailed images of your body without using radiation. Providers use MRI to evaluate soft tissue damage caused by EMP.
• Blood tests and urine test: Providers measure M protein levels with these tests.
• Nasal endoscopy: Providers may use this test to examine your nasal passages and sinus for signs of EMP.
• Fine-needle biopsy: Providers may use this test to obtain tissue and fluid from soft tissues affected by EMP.
• Biopsy. Providers may obtain tissue or fluid to examine plasma cells.

What are EMP stages?

Healthcare providers use the following factors to stage extramedullary plasmacytoma:

• Stage I: Tests show a single tumor in one spot in your body.
• Stage II: Tests show EMP cells in your lymph nodes.
• Stage III: Tests show more than one EMP tumor.

What are plasmacytoma treatments?

Healthcare providers treat solitary bone plasmacytoma with radiation therapy. (Providers often use multiple myeloma treatment in cases where people have abnormal proteins in their blood or urine and a plasmacytoma. Providers may treat extramedullary plasmacytoma with surgery and/ or chemotherapy or immunotherapy.

How can I prevent plasmacytoma?

Like multiple myeloma, there’s no known way to prevent plasmacytoma.

How long can you live with plasmacytoma?

Approximately 60% of people who have SBP are alive five years after diagnosis. Approximately 82% of people who have EMP are alive five years after diagnosis. Unfortunately, plasmacytomas can come back or develop in another part of your body.

Some people who have SBP or EMP develop multiple myeloma. This affects how long they may live.

How do I take care of myself?

Plasmacytoma can become multiple myeloma. If you have plasmacytoma, you'll need life-long follow-up care once you complete treatment. Follow-up care typically includes regular blood and imaging tests, so healthcare providers can watch for signs of multiple myeloma. If you have plasmacytoma, ask your healthcare provider what follow-up care you can expect.

A note from Cleveland Clinic

Plasmacytoma is a very rare blood disease that’s similar to multiple myeloma. Fortunately, healthcare providers can successfully treat many plasmacytomas, eliminating existing tumors and easing symptoms. Even so, being diagnosed with plasmacytoma means a lifetime of medical follow-up. It’s not easy to live with a disease that never really goes away. If you’re living with plasmacytoma, ask your provider for suggestions to help you cope with chronic illness. They’ll be glad to help.