Show and Tell
FIRM B - RED
Our team
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Dr. Clarke & Dr. Vargas
Shinoj & Arvind
Jacob & Muneeza
Chloe, Lauren & Njiye
History
57 yo women presented with 3 day h/o
- left sided chest pain.
diffuse, pressure like, pleuritic, radiating to the back,
4/10 on pain scale and non exertional.
- no shortness of breath
PMH: unremarkable
Labs
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BMP – unremarkable
CBC – unremarkable
EKG – normal
Troponin - < 0.012
Imaging
• Chest X ray:
“ Mass noted in the left lateral upper lobe of
the lung eroding into the fifth rib ”.
• CT angiogram:
“ Pleural-based mass in the posterior segment
of the left upper lobe with bony erosions
through the posterior fifth rib ” .
D/D
• Arising from the pleura - localized malignant
mesothelioma, solitary fibrous pleural tumour.
• Arising from lung parenchyma
- adenocarcinoma
• Arising from the bone
- Fibrous dysplasia, Paget’s disease
- Chondrosarcoma, Osteosarcoma
- Multiple myeloma, Plasmacytoma
Work up
• Plan was to get a tissue diagnosis, possibly a
bronchoscopy.
• Pulmonary service were consulted
• Pulmonary recs
- most likely adenocarcinoma.
- recommended IR guided biopsy as the mass
is not accessible to bronch due to its location.
Pathology report
• IR guided biopsy was performed.
• The biopsy result came back as
“ PLASMACYTOMA ”
Plasmacytoma
• Plasmacytoma is a discrete, solitary mass of
neoplastic monoclonal plasma cells.
• Types
Solitary bone plasmacytoma (SBP)
Soft tissue or non-osseous extramedullary
plasmacytoma (EMP)
Pathophysiology
• SBP : Arise from plasma cells of bone marrow
EMP: From plasma cells of mucosal surfaces
• SBP ~ 5% of plasma cell disorders
EMP ~ 3% of plasma cell disorders
• The median age of patients with SBP or EMP is
55 years. This median age is 10 years younger
than patients with multiple myeloma.
Presentation
• The most common symptom - pain at the site
of the skeletal lesion due to bone destruction
by the infiltrating plasma cell tumor.
• Vertebral involvement may also have evidence
of nerve root or spinal cord compression.
Labs
• Serum electrophoresis reveals a monoclonal
protein in the serum or urine in 24-72% of
patients, although levels are lower than in
multiple myeloma.
• Uninvolved immunoglobulin levels are usually
within the reference range.
• Peripheral blood cell count, renal function,
and calcium are within the reference range.
Imaging and histology
• Chest X ray: A lytic appearance with clear
margins and a narrow zone of transition to
healthy surrounding bone
• MRI: abnormal signal intensity (low on T1weighted imaging and high on T2-weighted)
• Histology: reveals infiltration of the bone by
monoclonal plasma cells.
SBP- Diagnostic criteria
1. Single area of bone destruction by clonal plasma cells
2. Bone marrow plasma cell infiltration not exceeding
5% of all nucleated cells.
3. Absence of osteolytic bone lesions or other tissue
involvement (no e/o myeloma).
4. Absence of anaemia, hypercalcemia, or renal
impairment attributable to myeloma.
5. Low, if present, concentrations of serum or urine
monoclonal protein.
6. Preserved levels of uninvolved immunoglobulins
Treatment
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Local radiotherapy is the treatment of choice.
Local control achieved in 88-100% of patients.
All patients have major symptom relief.
Local tumor recurrence rate of apprx 10%.
Monoclonal protein is markedly reduced after
radiotherapy in the majority of patients, but
protein disappearance is observed in only 2050% of patients.
Role of surgery & chemotherapy
• Surgery is contraindicated in the absence of
structural instability or neurologic
compromise
• Chemotherapy may be considered for patients
not responding to radiation therapy. Regimens
useful in multiple myeloma can be considered.
• No role exists for adjuvant chemotherapy in
solitary bone plasmacytoma.
Prognosis
 Solitary bone plasmacytoma (SBP) progresses to
multiple myeloma at a rate of 65-84% at 10 years
and 65-100% at 15 years.
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The median onset of conversion to multiple
myeloma is 2-5 years with a 10-year disease-free
survival rate of 15-46%.
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The overall median survival time is 10 years.
Treatment failure
• 3 patterns of treatment failure described :
1> development of multiple myeloma (54%)
2> local recurrence (11%), and
3> development of new bone lesions in the absence of
multiple myeloma (2%).
Prognostic factors for conversion
• Lesion size of at least 5 cm
• High M protein levels
• Persistence of M protein after treatment
• Elevated B2 microglobulin
• Spine lesions
• In a study by Wilder et al, 10-year myelomafree survival was 91% versus 29% in patients
whose M-protein did or did not resolve at 1
year following radiation therapy.
Role of b2 microglobulin
Southwest oncology group study
• Beta 2 microglobulin is used as a prognostic
marker in multiple myeloma.
• 322 patients with pretreatment serum b2m
values of less than 6 mcg/mL, median survival
was 36 months.
• 225 patients with a b2m level of greater than
or equal to 6 mcg/mL, median survival of 23
months (P less than .0001).
Back to my patient
• Skeletal survey: no lytic lesions.
• Serum immunofixation: normal
• Urine immunofixation: small monoclonal spike
of IgG kappa light chains
• Normal renal function.
• No anaemia, hypercalcemia.
• Scheduled for bone marrow biopsy next week.
If bone marrow shows less than 5% plasma
cells – Plasmacytoma.
• Radiation therapy is the next step.
• Finally, close follow up to look for progression
to multiple myeloma…………at my GMC !!!
Thank u !