Show and Tell

FIRM B - RED

Our team

• 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

• 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.

• TypesSolitary 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 T1-weighted 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 cells2. 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

• 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 20-50% 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.

The median onset of conversion to multiple myeloma is 2-5 years with a 10-year disease-free survival rate of 15-46%.

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 myeloma-free survival was 91% versus 29% in patients whose M-protein did or did not resolve at 1 year following radiation therapy.

Role of b2 microglobulinSouthwest 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 !