A case report of a patient presenting with features suggestive of metastatic breast CA that turned out to be multiple myeloma

1. Myelomatosis: an unusual
presentation
Dr JG Nel
Dr AD Jafta
Prof VJ Louw

2. Case
• Me M. 68-year-old HIV positive woman
Presented with:
▫ backache
▫ paraparesis
▫ vertebral collapse
▫ palpable mass in her right breast

3. Case
Risk factors for breast cancer:
• Female over the age of 40
• No family history of breast CA
• No previous history of breast CA
• No h/o HRT
• No h/o smoking
• No history of nipple discharge or of breast
enlargement

4. Clinical examination of Breast mass
Localised to sup lat quadrant
6x5 cm
Not fixed to skin or pectoral
muscle
Irregular surface with ill defined
borders
Hard and tender on palpation
Associated axillary LA on the right

5. Clinical diagnosis
• Clinically infiltrating ductal breast carcinoma
• T4N1M1

6. Special investigations ordered
• U&E, LFT, CMP and FBC
• X-rays of chest and spine
• Abdominal ultrasound
• MRI of brain and spinal cord
• Whole body scintigraphy
• Histology of breast mass
• Bone marrow investigations

7. Laboratory investigations
U&E
Na 136
K 3.1
Cl 97
Urea 3.7
Creat 49
CMP
Alb 17
Ca 2.53
Ca corr 2.98
Mg 0.54
PO4 1.26

8. Laboratory investigations
Full blood count:
• WCC : 6.28
• Hb : 8.8
• Plt : 193
• Neutrophils : 3.24
• Lymphocytes : 2.43
Erythrocyte sedimentation rate
• ESR : 90mm in 1 hour

9. Peripheral smear
• Rouleaux
• Leuko-erythroblastic reaction

10. Radiological investigations
• Radiographs: lytic and sclerotic lesions of
vertebrae
• Abdominal US: multiple well circumscribed
lesions in both liver lobes
• MRI: Lesions suggestive of diffuse metastatic
changes in the thoracic and lumbar vertebrae
causing nerve impingement .
Metastatic involvement of sacrum

11. Scintigram
Multiple areas of irregular high uptake,
esp the spine, ribs, left SI-joint and the
left hip.

12. Bone marrow aspirate

13. Bone marrow aspirate
• cells in groups, ?plasmacytoid
appearance

14. Bone marrow biopsy
H&E
• bone and bone marrow
•distorted architecture

15. Bone marrow biopsy
H&E
•Normal cells displaced by cells in
clumps

16. Bone marrow biopsy
H&E
•foreign cells with medium to large
nuclei and abundant cytoplasm

17. Bone marrow biopsy
Reticulin
• marked reticulin fibrosis

18. Bone marrow biopsy
CD138
• tumour cells stained CD138 positive

19. Tru-cut biopsy
H&E
• Connective tissue infiltrated by tumour
cells
• Tumour cells have a plasmacytoid
appearance

20. Tru-cut biopsy
CD138
• tumour cells CD138 positive
•CD138
• Stains membranes
• Indicates plasmacytoid
differentiation

21. Summary of Histological findings on
breast and bone marrow
• Dense infiltrates of poorly differentiated cells
• Similar growth patterns
• CD 138 :positive
• Cytokeratin :negative
• Synaptophasin and chromogranin :negative
• Electron microscopy:
▫ No intercellular bridges
▫ Increased endoplasmic reticulum

22. Electrophoresis
Serum and Urine
Serum:
M band 12 g/l
Imfix IgG kappa
Urine:
BJ prot pos
0.6g/24hr
Imfix free kappa
B-2-Microglobulin : 4.2

23. Diagnostic criteria MM
M-component in serum and/or urine
PLUS
clonal plasma cells in the BM
and/or
a documented clonal plasmacytoma
BJH 20032003;121:749-757

24. Diagnostic criteria MM
• PLUS one or more of the following:
▫ Calcium (corr) > 2.65mmol/l
▫ Creatinine ≥ 177μmol/l
▫ Anemia (Hb < 10g/dl or 2g/dl < normal)
▫ Bone disease (lytic lesions or osteopenia)
 Provided they are attributable to the underlying
plasma cell disorder
BJH 2003;121:749-757
BJH 20032003;121:749-757

25. Prognostic factors
International Prognostic
index
Survival by IPI staging system
STAGE 1 β2M < 3.5
• ALB > 3.5
STAGE 2 β2M < 3.5
• ALB < 3.5
• or
• β2M 3.5 – 5.5
STAGE 3 β2M > 5.5

26. Final diagnosis
Diagnostic criteria met:
1. M peak in serum and urine
2. Plasma cell infiltrate of bone marrow
3. Plasmacytoma in breast
4. Corrected Calcium of :2.98
5. Hb :8.8
6. Lytic bone lesions
Prognostic indicators
1. B-2-microgobulin 4.2
2. Albumin 17 g/l
3. Age of 68
Stage 3 Multiple Myeloma (IPI score)

27. Extramedullary plasmacytomas
• Plasmacytomas: malignant tumours arising from
plasma cells
• solitary form and disseminated form (associated
with Multiple myeloma)
• In bone or extramedullary

28. Extramedullary plasmacytomas
• NB to distinguish between the solitary and
disseminated forms
• Disseminated form indistinguishable from
Multiple Myeloma
• Solitary form associated with mild behaviour
and a long survival

29. Extramedullary plasmacytomas
• Can occur
▫ Head and neck region –mainly in the upper
aerodigestive tract
▫ GI tract
▫ Urinary bladder
▫ CNS
▫ Thyroid
▫ Breast
▫ Testes
▫ Parotid gland
▫ Lymph nodes
▫ Skin

30. What happened after diagnosis
• The patient was radiated for the mass in her
back causing compression on her spinal cord
• Received dexamethasone pulses
• Passed away whilst in Hospital

31. The significance of the case
• 1st Case of Myeloma described in South Africa to
present with a plasmacytoma mimicking breast
CA
• 2nd Case described in an HIV positive patient.

32. Conclusion
• This case highlights the fact that even though
most neoplastic lesions of the breast are of an
epithelial nature, malignancies of mesenchymal
or lymphoproliferative origin must also be
considered, since it has implications for
management.