tumors

Plasmacytoma: What is it? Causes, Symptoms, Diagnosis, Therapy and Prognosis of A.Griguolo

Generality

Plasmacytoma is a tumor of the blood, which is characterized by the abnormal and isolated proliferation of plasma cells, at bone and medullary level (bone variant) or at soft tissue level (extramedullary variant).

Due to reasons that for the moment are still unclear, plasmacytoma is a tumor similar to multiple myeloma, which often constitutes the prelude to the onset of the latter.

Plasmacytoma causes different symptoms depending on the site in which there is abnormal plasma cell proliferation.

In general, the diagnosis of plasmacytoma requires: physical examination, medical history, seroprotein electrophoresis, research in the urine of Bence Jones protein, complete blood count, tumor biopsy and imaging tests, such as magnetic resonance and PET.

In most cases, the treatment of plasmacytoma is based on the use of radiotherapy; more rarely, it involves surgery or radiation therapy associated with chemotherapy.

What is Plasmacytoma?

Plasmacytoma is a blood tumor, characterized by the abnormal and isolated proliferation of plasma cells, at bone level (where "bone" also means bone marrow ) or soft tissue (soft tissues are muscles, tendons, nerves etc.).

The adjective "isolated" indicates the presence of a single and circumscribed proliferating tumor mass.

Also known as isolated plasmacytoma, plasmacytoma is strongly similar to the more common multiple myeloma, another blood tumor; to distinguish these blood neoplasms is the fact that the second - multiple myeloma - is a widespread condition, in which there are more proliferating tumor masses and not just one (as is the case in plasmacytoma).

Many medical texts and experts in the field describe and consider multiple myeloma a progressed and widespread form of plasmacytoma, so much so that they define it with the expression "multiple plasmacytoma".

Short review of what a Plasmacellula is

Plasmocellulas, or plasmocytes, are the differentiated cells of the immune system, which produce antibodies, or glycoproteins that defend the human body against infections.

The plasma cells derive from the B lymphocytes, under stimulation of the T helper lymphocytes and following a process that experts in the field call antigen recognition .

In immunology, any substance or molecule that the immune system recognizes as foreign or potentially dangerous is called an antigen .

Causes

In general, tumors are the result of genetic aberrations ( mutations ), which specifically concern genes responsible for the control of growth, development and / or cell replication.

Currently, the causes of plasmacytoma - that is what determines genetic mutations at the origin of plasmacytoma - are unknown .

However, experts in the field have observed that the tumor in question is more frequent in the presence of:

  • A past history of exposure to ionizing radiation ;
  • A past history of exposure to particular chemicals, including petroleum derivatives, pesticides, solvents and asbestos;
  • State of immunodeficiency (due, for example, to AIDS or organ transplantation), to which a viral infection supported by herpes virus 8 or herpes virus 4 (or Epstein-Barr virus) is superimposed.

Curiosity

The factors favoring plasmacytoma are the same risk factors as multiple myeloma; this is a further confirmation of the analogies present between these two neoplastic conditions.

Types of Plasmacytoma

Doctors recognize two types of plasmacytoma: solitary plasmacytoma of the bone (or solitary bone plasmacytoma ) and solitary extramedullary plasmacytoma .

  • Solitary bone plasmacytoma: it is the plasmacytoma in which the phenomenon of anomalous and isolated proliferation of plasma cells takes place at bone and bone marrow level.

    The solitary plasmacytoma of the bone is usually located on the vertebral column, but it could also affect, alternatively, the bones of the pelvis, the ribs, the bones of the upper limbs (humerus, radius and ulna), the bones of the face, the bones of the skull, femur or sternum.

    Solitary plasmacytoma of the bone is a tumor strongly associated with the development, in later times, of multiple myeloma; the doctors, in fact, have observed that 50-70% of the cases of solitary plasmacytoma of the bone leads, within the following 5-10 years from the appearance, to a multiple myeloma;

  • Extramedullary solitary plasmacytoma: it is the plasmacytoma in which the phenomenon of anomalous and isolated proliferation of the plasma cells is located on soft tissues, outside the bone and bone marrow.

    Extramedullary solitary plasmacytoma affects the upper airways (in particular, nose, paranasal sinuses and throat) more frequently (85% of cases), but could also alternatively affect the gastrointestinal tract, lymph nodes or lungs.

    Extramedullary solitary plasmacytoma can also result in multiple myeloma, but this phenomenon - it should be pointed out - is much more rare (only in 10% of clinical cases) than in the case of solitary plasmacytoma of the bone.

As the reader may note, distinguishing the two types of recognized plasmacytoma is the site of tumor onset (bone and bone marrow or soft tissue).

Among the two types of recognized plasmacytomas, the solitary plasmacytoma of the bone appears to be the most common statist in the hand.

Curiosity

The typological classification of plasmacytoma was delineated by the US non-profit organization known as the International Myeloma Working Group, which includes doctors and researchers specialized in the diagnosis and treatment of multiple myeloma and related tumors (such as plasmacytoma).

Epidemiology

Plasmacytoma is an uncommon tumor.

Statistics in the hand, the most affected are middle-aged people ( 50 years old ) and the elderly around 60-65 ; in young and young adults, its onset is to be considered a true rarity.

Solitary bone plasmacytoma represents 3-5% of all forms of cancer affecting plasma cells.

In each of its variants (therefore both when it concerns the bone marrow and when it concerns the soft tissues), plasmacytoma is more frequent in male persons (the ratio with women is 2: 1, in the case of solitary plasmacytoma of the bone, and 3: 1, in the case of extramedullary solitary plasmacytoma).

Did you know that ...

Multiple myeloma affects a population of even greater age more frequently than that affected by plasmacytoma.

Specifically, multiple myeloma is particularly common in people over seventy.

Symptoms and Complications

Plasmacytoma symptoms vary depending on where the abnormal plasma cell proliferation has taken place ; a plasmacytoma with a bony seat (solitary plasmacytoma of the bone), in fact, produces a symptomatology different from a plasmacytoma based on a soft tissue (extramedullary solitary plasmacytoma).

Solitary Plasmacytoma of the Bone: details on the Symptoms

Solitary bone plasmacytoma is typically responsible for severe pain and repeated fractures at the bone portion involved.

Extramedullary Solitary Plasmacytoma: details on Symptoms

Extramedullary solitary plasmacytoma produces symptoms related to a malfunction or discomfort of the affected soft tissue ; for example, those who develop an extramedullary solitary plasmacytoma along a tract of the upper airways (as already stated, this site is characteristic of 85% of the cases of extramedullary solitary plasmacytoma) may complain of disorders such as: rhinorrhea, epistaxis and nasal obstruction.

Complications

The tendency of this tumor to wear the plasmacycoma complication is, above all in the bone variant (solitary plasmacytoma of the bone), to become multiple myeloma.

Multiple myeloma resulting from a plasmacytoma is equivalent to a sort of evolution of the latter.

As stated in the description of the types of plasmacytoma, the chances of the latter deriving a multiple myeloma are greater in the bone variant .

When should I go to the doctor?

Especially in an individual at risk of plasmacytoma (and, given the affinity, of multiple myeloma), it is a valid reason to contact a doctor or go to the nearest hospital, being a victim of a bone fracture in the absence of trauma or conditions such as osteoporosis, or the appearance, for no apparent reason, of symptoms such as rhinorrhea, nasal obstruction and / or epistaxis (NB: in the light of the above, in most cases, extramedullary solitary plasmacytoma produces these symptoms ).

Diagnosis

To make the diagnosis of plasmacytoma, a doctor generally uses the information provided by:

  • Physical examination and medical history . They serve to clarify the symptomatology and general state of health of the patient.

    They are essential to understand with which other investigations continue the diagnostic research.

  • A blood test called seroprotein electrophoresis . It is a test for the analysis of antibodies present in an individual's serum.

    The seroprotein electrophoresis is useful, because it allows to identify a serological anomaly characteristic of many cases of plasmacytoma, which is called paraproteinemia and which consists, in a few words, in an unusual accumulation of a certain type of monoclonal antibody;

  • Urine testing of Bence Jones protein . The Bence Jones protein is an unusual protein, whose presence in the urine is often associated with a blood tumor that has the plasma cells as its object;
  • The complete blood count . It is especially useful for distinguishing plasmacytoma from its most advanced form, multiple myeloma; in fact, unlike multiple myeloma, plasmacytoma does not produce high levels of calcium in the blood, reduced renal function and lack of red blood cells;
  • A biopsy of bone marrow or soft tissue considered to be affected. It serves to identify anomalous plasma cells and to outline their features; moreover, it helps to distinguish the plasmacytoma from multiple myeloma, through the counting of the proliferation sites (a solitary proliferation is an index of plasmacytoma);
  • Imaging tests, such as magnetic resonance and / or PET. They help in the distinction between plasmacytoma and multiple myeloma, because they are able to detect the proliferation sites of plasma cells.

Did you know that ...

Statistics in hand, paraproteinemia characterizes about 60% of cases of solitary plasmacytoma of the bone and about 25% of cases of solitary extramedullary plasmacytoma.

Therapy

In most cases, the treatment of plasmacytoma is based on the use of radiotherapy ; more rarely, it involves surgery or radiation therapy associated with chemotherapy .

Radiotherapy: What is and why is the treatment of first choice?

Radiation therapy against a tumor consists in exposing the area of ​​tumor proliferation to a certain dose of high-energy ionizing radiation (X-rays), which have the purpose of destroying the neoplastic cells.

Radiation therapy lends itself very well to the treatment of isolated and circumscribed tumors, which is why it is the first choice treatment against plasmacytoma (a neoplasm, precisely, isolated and circumscribed).

Did you know that ...

Tumors that, being isolated and circumscribed, lend themselves very well to radiotherapy are defined with the expression "radiosensitive tumors".

Surgery: What do you foresee and when do you need it?

Very rarely applied, the surgical treatment of plasmacytoma consists in removing the area of ​​tumor proliferation.

To justify the use of surgery can be:

  • The presence of an extramedullary solitary plasmacytoma, except in cases of cancer of the nose area (for aesthetic reasons);
  • The presence of a solitary plasmacytoma of the bone that produces skeletal instability or continuous fractures of the affected bone portion.

Chemotherapy: What is and when is it needed?

Chemotherapy consists of the administration of drugs capable of killing rapidly growing cells, including cancer ones.

As a rule, doctors treat plasmacytomas through the combination of radiotherapy and chemotherapy, when they want to implement an even more powerful treatment against the neoplasm.

As anticipated, in the presence of plasmacytoma, the use of chemotherapy is reserved for a few circumstances.

Curiosity

The chemotherapy drugs for the treatment of plasmacytoma are the same chemotherapy drugs used for the treatment of multiple myeloma.

After the Treatment: What happens?

Anyone who has developed a plasmacytoma and has undergone all the necessary treatment must follow a follow-up program at the end of the latter, which includes laboratory tests on blood and urine, and imaging tests at regular intervals. in order to monitor the response to therapy over the long term.

Prognosis

Radiation therapy ensures excellent control of the plasmacytoma only at the local level ; in fact, it does not prevent the tumor in question from recurring or, worse, from progressing to multiple myeloma after a few years.

Radiation therapy, therefore, allows to better treat the area of ​​proliferation that characterizes the plasmacytoma, but does not allow to eliminate the possibility of developing a similar tumor or getting multiple myeloma.

To know the details of the prognosis in case of plasmacytoma (response to radiotherapy, average survival rate, percentage of plasmacytomas evolving into multiple myeloma, etc.), it is advisable to consult the table below.

Type of PlasmacytomaResponse to RadiotherapyAverage Survival RatePercentage of cases progressing in Multiple Myeloma
Solitary bone plasmacytoma88-96%17-156 months (= just over a year - around 13 years)46-56%
Extramedullary solitary plasmacytoma83-96%69-124 months (= almost 6 years - just over 10 years)8-36%