When plasma cells go wrong: imaging features of Multiple
Myeloma and Solitary Bone Plasmacytoma
Poster No.:
C-1486
Congress:
ECR 2017
Type:
Educational Exhibit
Authors:
F. Matos, P. F. M. Azevedo, A. Figueiredo, A. P. Vedor, C.
Santiago, D. Silva; Viseu/PT
Keywords:
Hematologic, Bones, Conventional radiography, CT, MR, Staging,
Hematologic diseases
DOI:
10.1594/ecr2017/C-1486
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Page 1 of 22
Learning objectives
Understanding the range of imaging patterns of plasma cell dyscrasias, focusing on
Multiple Myeloma (MM) and solitary bone plasmacytoma (SBP) using conventional
radiographs, CT and MRI.
Background
•
General information - Plasma Cell Dyscrasias
Plasma cells are mature B lymphocytes destined to produce immunoglobulins (Ig).
When the refined control of this process is lost, plasma cell dyscrasias can lead to
overproduction of Ig.
MM accounts for approximately 10% of hematologic malignancies. Fatigue and bone pain
are common clinical presentations.
SBPs represent 2%-3% of plasma cell dyscrasias and they are characterized by the
formation of a single bone tumour. These malignant plasma cells are not commonly
present in the bone marrow itself or in the soft tissues surrounding the bone. SBPs may
reflect early MM according to the Durie-Salmon classification.
Durie-Salmon parameters:
•
•
•
•
blood hemoglobin
bone destruction on conventional radiography
blood calcium levels
urine and blood monoclonal immunoglobulin
Durie-Salmon Plus staging system (introduced in 2003 by the International Myeloma
Working Group) relies mainly on cross-sectional imaging findings and has substantially
different stages. This system, unlike the Durie-Salmon staging system, does not include
laboratory parameters such as hemoglobin and immunoglobin levels, apart from the use
of the serum creatinine level. The main innovation of this system was the inclusion of the
extent of diffuse bone marrow disease on MR or FDG PET/CT.
•
Solitary Bone Plasmacytoma
Page 2 of 22
Some patients can remain stable for decades, suggesting a relation to Monoclonal
Gammopathy of Undetermined Significance (MGUS).
The diagnosis requires:
1.
2.
3.
4.
5.
solitary lesion
biopsy showing plasma cells
no anemia, hypercalcemia or renal involvement suggesting multiple
myeloma.
negative clonal cells in bone marrow aspirate
negative skeletal survey , negative MR spine, pelvis, proximal femora/
humeri.
The most common location for SBP is the throracic vertebral body.
Because treatment of MM and SBP can differ significantly ( the latter involving essentially
radiotherapy), it is paramount to exclude a second lesion (which occurs in one third of
cases).
•
Multiple Myeloma
It represents a multifocal malignant proliferation of monoclonal cells within the bone
marrow.
Key clinical aspects
•
•
•
•
•
Bone pain occurs in 75% of cases
Bone marrow failure: anemia, infection
Renal insufficiency
M protein: blood and urine
Hypercalcemia
Main risk factors
•
•
•
•
•
•
Ionizing radiation
Chronic immune stimulation
HIV
Herpes virus 8
Autoimmune disease
Exposure to pesticides
Page 3 of 22
Incidence - Peak age: 60s to early 70s (SBPs have a lower age peak incidence).
•
Relevance
Over the past 10 years, a wider understanding of MM and SBP and the therapeutic
options available to patients with the disease have resulted in a significant increase in
their life expectancy. Imaging has contributted to this improvement by the use of modern
imaging methods, MR in particular. MR imaging bone marrow surveys in patients with
MM demonstrate the broad spectrum of involvement, the results of treatment, the areas
of potential complications and the sites of focal disease for safe bone biopsies.
Findings and procedure details
Multimodality approach - advantages and disadvantages
•
Conventional radiography
- Conventional radiography remains the primary imaging modality to look for bonedestructive lytic changes (Fig. 1; Fig. 2), fractures (Fig. 3) and collapse (Fig. 4) effects
in MM and SBP.
- It is good for evaluating cortical destruction but it might not detect small lytic lesions
(10-20% of lesions can be missed).
•
CT scan
- Main findings:
1.
2.
3.
4.
expansile lesions with associated soft tissue masses (Fig. 5; Fig. 6)
punched-out lytic lesions (Fig. 7)
compression fractures (Fig. 8)
rarely: involvement of intervertebral disc, adjacent vertebra
- Non enhanced CT is important to map the safest place for bone biopsy to establish the
diagnosis, which is of particular importance regarding the different types of treatment of
the plasma cell dyscrasias (Fig. 9).
- The bone cortex and trabeculae are depicted with greater detail in CT scans when
compared to plain film radiography.
Page 4 of 22
- CT scans also have the advantage of evaluating the soft tissues around the lesions
(compare Fig. 10 vs Fig. 11) .
- The main disadvantage of this imaging modality is that the imaging features of lytic
lesions persist even after a successful treatment.
- Contrast enhanced CT: no detectable enhancement.
•
MRI
- MRI is able to reflect the wide spectrum of tumoral spread. The presence of 7 focal
lesions measuring more than 5mm has been associated with a significant decrease in
the survival rate.
- This imaging modality can detect the presence of areas with decreased amount of fat
and increased signal intensity within the bone marrow on T1-weighted sequences (Fig.
12; Fig. 13).
- In MM, abnormalities are identified as hypointensities on T1, hyperintensities on STIR
images and enhancement on gadolinium-enhanced images (Fig. 14).
- STIR and T2 reflect the most sensitive sequences for the depiction of the typical changes
that occur in MM (Fig. 14; Fig. 15).
- Note that all of the changes described above are not specific, therefore other processes
characterized by infiltration, such as leukemia, lymphoma and metastasis are part of the
list of differential diagnosis in MM.
- Bone takes 5 years to recover the normal sign after treatment which is a disadvantage
of this modality.
- If nerve root or spinal root compression is suspected, MR should be the exam of choice.
Whole-body MR:
•
Recommended for SBP or monoclonal gammapathy and normal
radiographs OR < 5 lesions.
Page 5 of 22
•
•
•
In SBP, MRI is used to determine the location, size and local compressive
effects (Fig. 13);
STIR sequences shown to have highest level of sensitivity and reliability best for initial assessment of MM or SBP (Fig. 14; Fig. 15)
For exact staging and grading, protocol should include unenhanced and
enhanced sequences.
Images for this section:
Page 6 of 22
Fig. 1: Multiple Myeloma. Punched-out lesions in the skull. This pattern is typical of
multiple myeloma. Also known as "swiss cheese-pattern".
© - Viseu/PT
Fig. 2: Multiple Myeloma. Right umerus radiograph shows a sharply circumscribed lytic
lesion (red arrow).
© - Viseu/PT
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Page 8 of 22
Fig. 3: Multiple Myeloma. Left umerus radiograph shows pathologic fracture due to
mielomatous infiltration across the bone.
© - Viseu/PT
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Page 10 of 22
Fig. 4: L5 Plasmacytoma - lateral view radiograph - collapse of the vertebral body due
to tumour infiltration.
© - Viseu/PT
Fig. 5: MM. Lesion on the large left sphenoid wing (red arrow), with spread to the middle
fossa of the skull. The orbital component causes deviation of its extrinsic musculature
and the endocranial component causes deformation of the tip of the left temporal lobe.
Page 11 of 22
© - Viseu/PT
Fig. 6: MM. CT scan. Axial view. Lytic lesion centered on the right frontal bone, with
soft tissue density and endocranial (epidural) expression to the contiguous parenchyma.
Page 12 of 22
However, it should be noted that the bulkier component of the lesion is epicranial, which
causes bulging of the scalp.
© - Viseu/PT
Fig. 7: MM. CT reconstruction: Severe lytic lesions across the right portion of the skull
© - Viseu/PT
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Fig. 8: L5 Plasmacytoma - sagittal view - CT scan shows collapse of the vertebral body.
© - Viseu/PT
Page 14 of 22
Fig. 9: SBP. CT scan - axial view. CT guided biopsy directed at the vertebral body of L2.
© - Viseu/PT
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Fig. 10: SBP. Lateral view of the knee. Radiograph shows a lytic lesion in the distal
metaphysis of the femur. Biopsy revealed it corresponded to a SBP.
© - Viseu/PT
Fig. 11: SBP. Coronal view CT scan showing a lytic lesion located at the level of the
medial condyle of the femur, causing cortical disruption. There is no evidence of invasion
of the adjacent soft tissues.
© - Viseu/PT
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Fig. 12: SBP. MR of the knee plasmacytoma of the depicted in Fig. 10 and Fig. 11. a)
sagittal view: Spin Echo T1-weighted image; b) sagittal view: Gradient Echo T2. Depiction
of a relatively well defined lesion in the medial condyle of the femur showing hypointensity
in SE T1 and marked hyperintensity in GRE T2.
© - Viseu/PT
Fig. 13: SBP. MR imaging. The images show alteration of the L2 vertebral body
signal, with extension to the pedicles, characterized by (left to right): hypointensity on
T1, hyperintensity on T2 / SPAIR, with homogenous enhancement after administration
of gadolinium (right), corresponding to a vertebral plasmacytoma. Associally, there is
depression of the L2 vertebral platforms, conditioning the discrete retreat of the posterior
wall, in the context of a pathological fracture.
© - Viseu/PT
Page 18 of 22
Fig. 14: MR of a patient with MM. Left to right: T1-weighted; T2-weighted;
gadolinium-enhanced T1-weighted; STIR. Signal alteration shows hypointensity on T1,
hyperintensity on T2/STIR, enhancement on gadolinium-enhanced image. "Salt and
pepper pattern" is better appreciated on STIR.
© - Viseu/PT
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Page 20 of 22
Fig. 15: MM. A closer look at the STIR sequence of the patient of Fig. 14 shows an
expansile lesion with associated soft tissue mass in D2 representing a focus of plasma
cell infiltration with compression of the cord.
© - Viseu/PT
Page 21 of 22
Conclusion
- Important steps have been taken over the last decade regarding imaging techniques
and treatment options of plasma cell dyscrasias.
- Recognizing patterns and understanding the behavior of plasma cells and the
repercussions in imaging is major to assess the extension and behaviour of the lesions.
Personal information
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