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Solitary Plasmacytoma- In Bone, Outside of Bone?
Solitary plasmacytomas most frequently occur in bone (plasmacytoma of bone), but can also be found outside bone in soft tissues (extramedullary plasmacytoma) [1-4]
There is full-blown multiple myeloma and there is pre-multiple myeloma. Pre-MM is not a blood cancer. Conventional oncology calls pre-MM a “blood disorder.” Pre-MM is usually just MGUS or SMM. The study linked below includes solitary bone plasmacytoma (SBP) and solitary extramedullary plasmacytoma (SEMP).
In short, both SBP and SEMP are pre-MM aka blood disorders. As always, the key issue is the risk of SBP or SEMP becoming an incurable blood cancer called multiple myeloma.
Why is diagnosing these two types of single, not multiple myeloma so difficult? Because the occurrence of both SBP and SEMP are rare. The studies below maintain that SBP (IN Bone) occur 4% of the time mm is diagnosed and SEMP (OUTSIDE of bone) occurs 3% of the time.
Think about it. MM itself is rare accounting for less than 2% of all cancer diagnoses in the U.S. annually. Pre-MM is rare when compared to full-blown MM diagnoses. SBP and SEMP is only 4% and 3% of the rare diagnosis of pre-mm.
This is where I come it. I was diagnosed with SPB (my c5). I was 34 year of age at the time (rare). I was non-secretory. Also rare. Rare of rare of rare. Think of the odds…:-)
But I digress.
I may have been misdiagnosed at the time. I’ll never know. The man who diagnosed me was a general oncologist. Highly unlikely that Dr. Berger had ever come across anything like me before or since.
And that is the moral of this blog post. You don’t want to put your life (diagnosis and treatment) in the hands of an oncologist who has never seen anything like you before. If you have a SPB, SEMP, Pre-MM, etc. get a second opinion from a MM specialist.
To Learn More About Solitary Bone Plasmacytoma- click now
Reach out to me on PeopleBeatingCancer.
Thanks for your time and attention.
David Emerson
- MM Survivor
- MM Cancer Coach
- Director PeopleBeatingCancer
Recommended Reading:
- Is a Diagnosis of “Plasma Cell Dyscrasia” a Diagnosis of MGUS, SMM or MM?
- Familial Multiple Myeloma Survivor Increases Your Risk of MM
- Beating Myeloma: “If I Knew Then What I Know Now”
18F-FDG PET/CT in the Evaluation of Solitary Extramedullary Plasmacytoma: A Case Series
“The role of fluorine-18-fluorodeoxyglucose positron emission tomography/ computed tomography (18F-FDG PET/CT) in patients with multiple myeloma (MM) and other plasma cell disorders is well-known.
Solitary plasmacytoma (SP), an extremely rare form within this entity accounting for approximately 4% of plasma cell malignancies, can be classified as
- solitary bone plasmacytoma (SBP) or
- solitary extramedullary plasmacytoma (SEMP).
Extramedullary plasmacytoma (EMP) is a rare neoplasm characterized by the monoclonal proliferation of plasma cells outside the bone marrow. Breast and craniocerebral regions are the uncommon sites of the presentations of EMP, rarely reported in the literature.
The most frequent site of presentation is the upper airways. The EMPs have similar pathogenesis as MM; however, they differ in management as they are radiosensitive in nature, and radiotherapy is the preferred treatment modality.
As SEMP has a better prognosis than SPB with a lower conversion rate to MM, accurate staging is essential to plan for the treatment. The 18F-FDG PET/CT has higher sensitivity for the evaluation of treatment response. In the present case series, it was aimed to depict the role of 18F-FDG PET/CT in newly diagnosed SEMP with different sites of origin to exclude further lesions leading to changes in the treatment plan and treatment response assessment.”
Diagnosis and management of solitary extramedullary plasmacytoma
“Plasma cell neoplasms (plasma cell dyscrasias) are a group of entities characterized by the neoplastic proliferation of a single clone of plasma cells, typically producing a monoclonal immunoglobulin. Plasma cell neoplasms can present as a single lesion (solitary plasmacytoma) or as multiple lesions (multiple myeloma). Solitary plasmacytomas most frequently occur in bone (plasmacytoma of bone), but can also be found outside bone in soft tissues (extramedullary plasmacytoma) [1-4].
Why some patients develop multiple myeloma and others a single plasmacytoma is not understood, but might be related to differences in cellular adhesion molecules or chemokine receptor expression profiles of the malignant plasma cells [5].
Solitary extramedullary plasmacytomas (SEP; solitary extraosseous plasmacytoma) are plasma cell tumors that arise outside of the bone marrow. They are solitary lesions, and are most often located in the head and neck region, mainly in the upper aerodigestive tract, but may also occur in the gastrointestinal tract, urinary bladder, central nervous system, thyroid, breast, testes, parotid gland, lymph nodes, and skin.
SEP refers to a solitary non-osseus plasma cell neoplasm in the absence of any other sign of multiple myeloma. The diagnosis and management of SEP will be discussed here. Note that extramedullary plasmacytomas can arise in patients with multiple myeloma at any time during the course of the disease, and should not be confused with SEP. The diagnosis and treatment of other plasma cell disorders (eg, solitary plasmacytoma of bone, multiple myeloma, primary AL amyloidosis, monoclonal gammopathy of undetermined significance) are discussed separately. (See “Diagnosis and management of solitary plasmacytoma of bone” and “Multiple myeloma: Clinical features, laboratory manifestations, and diagnosis” and “Clinical presentation, laboratory manifestations, and diagnosis of immunoglobulin light chain (AL) amyloidosis” and “Diagnosis of monoclonal gammopathy of undetermined significance”.)…
EPIDEMIOLOGY
SEPs account for approximately 3 percent of plasma cell malignancies [6,7]. The median age at diagnosis is 55 to 60 years, and approximately two-thirds of patients are male.
Diagnosis and management of solitary plasmacytoma of bone
“Plasma cell neoplasms (plasma cell dyscrasias) are a group of entities characterized by the neoplastic proliferation of a single clone of plasma cells, typically producing a monoclonal immunoglobulin. Plasma cell neoplasms can present as a single lesion (solitary plasmacytoma) or as multiple lesions (multiple myeloma [MM]). Solitary plasmacytomas most frequently occur in bone (plasmacytoma of bone), but can also be found outside bone in soft tissues (extramedullary plasmacytoma) [1-4].
Why some patients develop MM and others plasmacytoma is not understood, but might be related to differences in cellular adhesion molecules or chemokine receptor expression profiles of the malignant plasma cells [5].
Solitary plasmacytoma of bone (SPB, also called osseous plasmacytoma) is a localized tumor in the bone comprised of a single clone of plasma cells in the absence of other features of MM (ie, anemia, hypercalcemia, renal insufficiency, or multiple lytic bone lesions) [6,7].
The diagnosis and management of SPB will be reviewed here. The diagnosis and treatment of other plasma cell disorders (eg, solitary extramedullary plasmacytoma, MM, primary AL amyloidosis, monoclonal gammopathy of undetermined significance) are discussed separately.”