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Solitary Plasmacytoma of the Bone SPB

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Solitary Plasmacytoma of the Bone SPB

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    1. Solitary Plasmacytoma of the Bone (SPB) Michael Gu, MD May 9, 2003

    2. Case #1 30 y.o.A.A.F., healthy, presented with progressive bilateral LE weakness for 2 months PTA. MRI of the spine: T7 vertebral body lesion with epidural soft tissue mass. Positive for cord compression. Core biopsy: “atypical plasma cell” Lab: CBC, CMP normal. Ca++ 9. SPEP: Gamma 1.4; Restr Pk 0.3g/dl; IF: IgG lambda. UPEP: (-) for M-protein. IF (-). Quantitative immunoglobulin: normal range ?2-microglobulin 2.2; LDH 163

    4. Case #1 30 y.o.A.A.F., healthy, with progressive bilateral LE weakness for 2 months PTA. MRI of the spine: T7 vertebral body lesion with epidural soft tissue mass. Positive for cord compression. Lab: CBC, CMP normal. Calcium 9. SPEP: Gamma 1.4; Restr Pk 0.3g/dl; IF: IgG lambda. UPEP: (-) for M-protein. IF (-). Quantitative immunoglobulin: normal range. ?2-microglobulin 2.2; LDH 163. Core biopsy: “atypical plasma cell”

    5. Case #1 (continued) Bone marrow biopsy: 1% plasma cell. No evidence of plasma cell dysplasia. Further Imaging: Skeleton survey: no other lytic lesions CT scan of the chest/abdomen/pelvis: no other lesions Final diagnosis: Solitary plasmacytoma of the bone. Management: Local RT Lost follow up

    6. Case #2 48 y.o. healthy w.f. presented with left hip/thigh pain for 11 months PTA. Plain X-ray and CT scan: A large marrow-centered mass at left ilium with adjacent tissue invasion. Initial diagnosis: Metastatic malignancy/solitary plasmacytoma/chondrosaarcoma Lab: CBC, BMP normal SPEP: TP:7.7; Gamma: 2.5; Restr Pk: 2.2; IF: IgG Kappa UPEP: no Resr Pk; IF: IgG Kappa. ?2-microglobulin: 2.1 LDH: 152 Quantitative immunoglobulin: IgG 1580; IgA and IgM normal

    9. Case #2 48 y.o. healthy w.f. with left thigh/hip pain for 11 months. Plain X-ray and CT scan: A large marrow-centered mass at left ilium with adjacent tissue invasion. Initial diagnosis: Metastatic malignancy/chondrosaarcoma /solitary plasmacytoma Lab: CBC, BMP normal SPEP: TP:7.7; Gamma: 2.5; Restr Pk: 2.2; IF: IgG Kappa UPEP: (-) Restr Pk; IF: IgG Kappa. ?2-microglobulin: 2.1 LDH: 152 Quantitative immunoglobulin: IgG 1580; IgA and IgM normal.

    10. Case #2 (Continued) Exploratory surgery with biopsy: Pathology: diffuse sheets of slightly atypical plasma cell. 98% cells are CD 38+ and (+) cytoplasmic kappa light chain. Left total hip arthroplasty, partial excision of pelvis and acetabular reconstruction. Bone marrow biopsy: Plasma cell 5%. No diagnostic feature for plasma cell dyscrasia. Skeleton survey: ? Two small lytic lesions at right ilium CT scan of the chest/abdomen/pelvis: no other lytic lesions MRI of the spine: no marrow replacement or focal osseous lesion

    11. Case #2 (Continued) Final diagnosis: Solitary plasmacytoma of the bone Post-surgery management: Local adjuvant RT: total 4000cGy in 20 fractions Physical therapy.

    12. Questions Prognosis? Observation or adjuvant therapy?

    13. Solitary Plasmacytoma Definition: localized tumor containing monoclonal plasma cells Type: Solitary Plasmacytoma of the Bone (SPB) Extramedullary Plasmacytoma (EMP)

    14. Difference Between SPB and EMP

    15. SPB Incidence: 2-5% of plasma dysplasia disorders. Sex: Male:female = 3-4:1 (MM 1-1.5:1) Age: Median age 55 y/o (MM=69y/o) Initial site of involvement: thoracic spine > lumbar spine > pelvis > rib

    16. SPB (Continued) Symptoms: Pain: bone destruction Neurologic symptom: spinal cord / nerve compression Serum monoclonal protein: Positive in 24-72% of the cases The level is much lower than MM Immunoglobulin: The uninvolved immunoblobulin levels are preserved Standard therapy: Local radiotherapy Dose: 4000 cGy Field: a normal tissue margin (in spine lesion, = or >one uninvolved vertebra) Outcome and prognostic factors:

    17. Criteria of the Diagnosis of SPB Single bone lesion Complete radiographic skeletal survey MRI scan of the axial skeleton (skull, spine, pelvis, proximal femora and humeri) Clonal plasmacytosis Biopsy of the tumor Flow cytometry or immunohistochemistry Normal bone marrow Morphology Lack of clonal plasma cells or aneuploidy on flow cytometry Absent or low, serum or urinary levels of monoclonal protein If present at diagnosis, should disappear within 6-12 months of therapy Preserved levels of uninvolved immunoglobulins No anemia, hypercalcemia, or renal impairment attributable to myeloma

    18. OUTCOME

    19. Summary of the outcome with RT

    20. Pattern of Progression Local relapse (<10%) New focal bone lesions(<10%) Multiple Myeloma( >80%)

    21. Prognostic Factors

    22. Solitary Bone Plasmacytoma: Outcome and Prognostic Factors Following Radiotherapy 1965-1996. 57 previously untreated patient with SPB Treatment: Megavoltage radiation Median dose 50 Gy (30-70 Gy) Median fraction size 2 Gy (1.3-5.0)

    23. Results Local control : 96% Post RT myeloma protein level: Disappeared from serum: 9/33 (27%) Disappearance of B-J protein: 2/7 (29%) Evolution of MM: 29 Pts (53%) Median time for progression: 1.8 year Median survival: 11 years

    24. Prognostic Factors Dose of Radiotherapy:

    25. Prognostic Factors (Continued) Age, site of the disease and pretreatment paraprotien level: no effect

    26. Prognostic Factors (Continued)

    27. Prognostic Factors (Continued)

    28. Prognostic Factors (Continued) Pretreatment imaging modality:

    29. Prognostic Factors (Continued) low level of uninvolved IG: All 3 patients had early progression to MM Patient with ? uninvolved IG should not be considered to have SPB. *Size of the Lesion: Lesion >5 cm is an adverse prognostic factor. (Holland, et al 1991) 82 % of the conversion Pts had lesion >5 cm (median 7 cm) 28% of the Unconversion Pts had lesion>5 cm (median 3.75 cm)

    30. Solitary Plasmacytoma of Bone: Mayo Clinic Experience 1950-1982. 46 cases with median follow up 90 months. RT alone. Dose 20 Gy -70 Gy ( median 39.75 Gy) MM progression rate 54%. Median time to progression: 18 months. Survival:

    31. Prognostic Factors

    32. Summary of Prognostic Factors Post-radiotherapy M protein level Pretreatment imaging modality Uninvolved immunoglobulin level Original tumor size

    33. How to prevent disease progression and improve survival? Increase the sensitivity of diagnostic tool to fill out “ MM in evolution”: MRI, PET, Flow cytometry, plasma cell labeling index Strictly follow diagnostic criteria Adjuvant therapy for “high risk” patients

    34. The Role of Adjuvant Chemotherapy in SPB

    35. The role of radiation therapy in the treatment of solitary plasmacytomas 1960-1985. Total 30 patients (SPB 17; EMP 13) Median follow up: 12.8 years ( 39 mo - 25 y) Criteria for SPB: ….+ BM plasma cell <10; IgG <3.5; Ig A<2.

    36. Improved Outcome in Solitary Bone Plasmacytomata with Combined Treatment

    38. Results: 15/28 (54%) patients with RT alone progressed to MM 3/25 (12%) patients with RT+chemo progressed to MM No significant adverse effects. No leukemia.

    39. Solitary Plasmacytoma of the Spine long-Term Clinical Course 1959-1979. 19 patients with SPB of the spine. 8/19 patients had RT + chemotherapy 4/8 (50%) progressed 4/8 developed leukemia (3/4 had progressed disease)

    40. Plasmacytoma Treatment Results and Conversion to Myeloma 1961-1988. 46 patients (32 SPB; 14 EMP) Adjuvant chemo and conversion to MM: Chemotherapy did not prevent the conversion to MM. Chemotherapy may delay the time to conversion. The survival time after conversion was the same (14.5 mo)

    41. The Disadvantage of Adjuvant Chemotherapy in SPB The benefit is still uncertain. May over-treat patients who are cured by RT. The chance to induce resistant subclones. Adverse effects such as leukemia.

    42. American society of Clinical Oncology Clinical Practice Guidelines: The Role of Bisphosphonates in Multiple Myeloma Starting bisphosphonates for patients with solitary plasmacytoma is not suggested.

    43. Summary Solitary plasmacytoma is a rare disease which consists of SPB and EMP. EMP has a better outcome then SPB. Local RT is still the standard treatment. With that, SBP has long-term overall survival (9.9Y). However 2/3 of the patients will progress to MM in 10 years. Most of the conversions occurred in first 4 years. The size of the tumor, the post-radiotherapy M-protein level and level of uninvolved IG may predict the outcome.

    44. Summary (continued) Strictly following diagnostic criteria and using sensitive screening tools to exclude MM may increase the specificity for diagnosis of “pure” SPB. It may improve the outcome. The role of adjuvant chemotherapy in the prevention of MM conversion is still unclear. Generally, it is not recommended at the time.

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