Solitary bone plasmacytomas have a male to female ratio of 2:1.1 Extramedullary plasmacytomas occur in men about 75% of the time. The average age of patients is between 40 and 80 years old, and has a median age of 55. It is a rare disease.
Etiology:
There are not many risk factors for plasmacytoma. The couple risk factors are2:
Ionizing radiation
Pesticides
Human immunodeficiency virus (HIV) infection
Herpes infection
Signs & Symptoms:
Pain and tenderness in the affected site are the most common signs and symptoms for plasmacytoma.3
Diagnostic Procedures:
MRI, PET and blood test are used for diagnosis purpose.3
Histology:
Plasma cells are associated with proliferation and accumulation of immunoglobulin-secreting cells derived from B-call lymphocytes.4
Lymph node drainage:
There is no specific data pertaining to the lymph node drainage for plasmacytoma.
Metastatic spread:
Most commonly detected in multiple vertebral bodies, pelvic bones, or bone marrow.4 It can also arise in the upper aerodigestive tract, nasal cavity, nasopharynx, paranasal sinuses, larynx, tonsils, which can lead to disease in the lung, lymph nodes, spleen, and gastrointestinal tract.
Grading:
Grade I: A marchalko type in which the plasma cells are indistinguishable from normal cells, with excentric cartwheel nuclei, a perinuclear, and basophilic cytoplasm. Occasional mitotic figures can be seen.5
Grade II: Asynchronous type in which there is marked discrepancy of maturation between nucleus and cytoplasm. At least 50% of all cells have enlarged nuclei with prominent nucleoli. The cytoplasmic:nuclear ratio is low, but a small perinuclear hof usually is present.
Grade III: A plasmablastic type with large nuclei and very prominent, centrally located nucleoli. The cytoplasm is restricted to a fairly narrow rim. The perinuclear hof is inconspicuous or absent.
Staging:
No universally accepted staging system exists. Of the plasma cell neoplasms, a staging system exists only for multiple myeloma.6
Radiation side effects:
General side effects include fatigue and erythema. Other side effects depend on the site of treatment. For example, pelvic radiation can suppress bone marrow.7
Prognosis:
In 11-30% of patients with extramedullary plasmacytoma, their disease will evolve into multiple myeloma within 10 years. In these patients the overall survival rate at 10 years is 70%.
In 50-60% of patients with solitary bone plasmacytoma, their disease will turn into multiple myeloma. In these cases the median survival span is 10 years.
A decrease in survival rate is seen with:
Severe anemia
Hypercalcemia
Blood urea nitrogen greater the 40 mg per dL
Elevated M protein
Hypoalbuminemia
High tumor cell burden.[4]
Treatments:
The standard of treatment for plasmacytoma is radiation therapy.[4]
It can be used to control local tumors, or for palliation if the chemotherapy has not controlled bone pain. It can also be used to stop the possibility of fractures in weight-bearing bones, and avoid spinal cord and nerve root compression.[8]
The fields should include the entire bone plus a 2-3 centimeter (cm) margin. For extramedullary plasmacytomas the primary lymph nodes should also be included as the treatment is taken to 50 Gy in 5 weeks of treatment.[4]
Surgery: Without radiation therapy there is a high recurrence rate.[8]
TD 5/5:
The necessary organ tolerance doses (TD) that will cause a minimum 5% complication rate within 5 years are dependent on the exact location of the lesions, but typically include the following9: Organ TD5/5 Spinal cord 4500cGy Bone Marrow 250cGy Lung 3000cGy Liver 2500cGy Colon 4500cGy Small intestine 4000cGy Stomach 4500cGy Esophagus 6000cGy
Additional images:
Reprinted from Principles and Practice of Radiation Oncology, 5th edition.10 Radiation treatment plan for a solitary plasmacytoma of the right iliac bone involving the acetabulum down to the ischial tuberosity, with associated soft tissue mass, 40 Gy in 20 fractions, anterior-posterior/posterior-anterior fields with 18 megavolts (MV) photons. Image A: Digital reconstructed radiograph displaying gross tumor volume (GTV), clinical target volume (CTV), and planning target volume (PTV). Image B: Isodose distribution in the axial perspective.
Grade II: Asynchronous type in which there is marked discrepancy of maturation between nucleus and cytoplasm. At least 50% of all cells have enlarged nuclei with prominent nucleoli. The cytoplasmic:nuclear ratio is low, but a small perinuclear hof usually is present.
Grade III: A plasmablastic type with large nuclei and very prominent, centrally located nucleoli. The cytoplasm is restricted to a fairly narrow rim. The perinuclear hof is inconspicuous or absent.
- In 11-30% of patients with extramedullary plasmacytoma, their disease will evolve into multiple myeloma within 10 years. In these patients the overall survival rate at 10 years is 70%.
- In 50-60% of patients with solitary bone plasmacytoma, their disease will turn into multiple myeloma. In these cases the median survival span is 10 years.
A decrease in survival rate is seen with:Organ TD5/5
Spinal cord 4500cGy
Bone Marrow 250cGy
Lung 3000cGy
Liver 2500cGy
Colon 4500cGy
Small intestine 4000cGy
Stomach 4500cGy
Esophagus 6000cGy
Reprinted from Principles and Practice of Radiation Oncology, 5th edition.10
Radiation treatment plan for a solitary plasmacytoma of the right iliac bone involving the acetabulum down to the ischial tuberosity, with associated soft tissue mass, 40 Gy in 20 fractions, anterior-posterior/posterior-anterior fields with 18 megavolts (MV) photons.
Image A: Digital reconstructed radiograph displaying gross tumor volume (GTV), clinical target volume (CTV), and planning target volume (PTV).
Image B: Isodose distribution in the axial perspective.
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