CLINICAL DATA:
An elderly man with generalized weakness.
Elevated serum calcium level.
Skull radiographs showing numerous, discrete, well circumsribed small, lytic round lesions in the skull and mandible.
DIAGNOSIS: Multiple Myeloma
Discussion:
- a hematologic disorder involving overproduction of a monoclonal immunoglobulin by neoplastic plasma cells. Tumor cells proliferate in the bone marrow and overexpress RANK ligand, which causes increased osteoclast activity and, ultimately, bone resorption.
- most common primary malignant bone tumor in patients older than 40 years of age.
- primarily affects older people (only 2% of patients are younger than 40 years of age).
- At least 30% cancellous bone loss is required to visualize an intramedullary destructive process with radiographs.
- a disease of older patients. The disease can present with diffuse demineralization, which may be indistinguishable from the pattern found in patients with simple senile osteoporosis.
- 2 sclerotic forms of myeloma:
- A rare form, known as POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes), that may demonstrate sclerotic lesions on radiographs, but this condition is responsible for fewer than 1% of myeloma cases.
- Mixed lytic and sclerotic lesions.
- Radiographs of treated myeloma lesions also may rarely show areas of abnormal bone architecture with sclerosis. Usually, little reactive bone sclerosis or periosteal reaction is seen (1)
- If associated with bone marrow plasmacytosis and elevated blood gamma-globulins, the diagnosis of myeloma is certain.
- False-positive examinations are encountered when multiple lytic lesions are found. In these patients, perform additional studies because the most likely source of this pattern is metastatic disease, not myeloma.
- Diffuse osteopenia that is found on radiographs is often a source of false-negative examinations because a substantial amount of cancellous bone must be destroyed before an intramedullary lesion becomes visible radiographically.
- A sclerotic form of multiple myeloma, sclerosing myelomatosis, which is the most severe variant. It is uncommon and associated with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome.
1. Radiographs
– typically presents as well-defined intramedullary lytic lesions, which are characteristically described as having a “punched out” appearance. The lesions can vary in size and can be focal or diffuse.
– commonly affects the axial skeleton (vertebrae are the most common sites of involvement), but it can affect the proximal appendicular skeleton,
– it rarely affects the distal appendicular skeleton.
– less commonly present with diffuse osteopenia, often with vertebral compression fracture(s).
2. MRI or PET/CT
– multifocal or diffuse infiltration of bone marrow, and an intramedullary soft-tissue mass is often visualized.
- Differential diagnoses (for multiple focal lytic lesions)
- Metastases
- Leukemia
- Primary lymphoma of bone.
- Treatment typically involves chemotherapy and possibly autologous hematopoietic stem cell transplant.
- Bisphosphonates are often used to prevent pathologic fractures.
References:
1. Mulligan ME. Myeloma update. Semin Musculoskelet Radiol. Sep 2007;11(3):231-9.
2. Angtuaco EJ, Fassas AB, Walker R, Sethi R, Barlogie B. Multiple myeloma: Clinical review and diagnostic imaging. Radiology. 2004;231(1):11-23.
3. Kyle RA, Gertz MA, Witzig TE, et al. Review of 1027 patients with newly diagnosed multiple myeloma. Mayo Clin Proc. 2003;78(1):21-33.
4. Rothschild BM, Hershkovitz I, Dutour O. Clues potentially distinguishing lytic lesions of multiple myeloma from those of metastatic carcinoma. Am J Phys Anthropol. 1998;105(2):241-250.
5. Siegel RL, Miller KD, Jemal A. Cancer statistics, 2015. CA Cancer J Clin. 2015;65(1):5-29.
6. Dr. Neil Harrison, University of Pennsylvania Department of Radiology.