Multiple Myeloma with an Unusual Distribution of Bony Involvement. Report of a Case

Clinical Cases 07.09.2007
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 44 years, male
Authors: S.M. Walsh D.G. Lohan P.A. McCarthy. Dept of Radiology, University College Hospital Galway, Ireland
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Details
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AI Report

Clinical History

Fatigue, malaise and right heel pain

Imaging Findings

44-year-old male with a one month history of fatigue, malaise and heel pain. The following haemaological abnormalities were identified at presentation: Low Haemoglobin of 7g/dl. Raised ESR of 150. Total globulins elevated at 80. IgA of 79. Serum electrophoresis revealed a localised band in the alpha region thus confirming IgA myeloma. Skeletal survey unusually showed no bony abnormality. Plain radiograph of the painful heel (Fig 1) demonstrated a well circumscribed radiolucent lesion within the calcaneum measuring 2cm x 2cm in diameter. This radiological appearance looked suspicious for that of a myelomatous deposit. Percutaneous biopsy of this lesion confirmed the presence of abundant neoplastic plasma cells within the calcaneum. Progression of bone pain in both feet revealed multiple punched out lytic lesions in a predominantly periarticular distribution within the bones of the tarsus and the metatarsals (Figs 2 and 3). This is a characteristic radiological manifestation of the osteolysis associated with multiple myeloma. Radiograph of the forearm (Fig 4) also demonstrated another prominent feature of multiple myeloma – that of endosteal scalloping. This is due to erosion of the inner border of the cortex of the proximal radius caused by myelomatous deposits. A solitary lytic lesion was also seen in the proximal right tibia (Fig 5).Plain radiographs of the wrist (Fig 6) also showed involvement of these areas by periarticular diffuse myelomatosis. Disease in the axial skeleton was never demonstrated.

Discussion

Multiple myeloma or plasma cell myeloma is the most common primary malignant process in adults, representing around 1% of all malignancies and approximately 10% of the total number of haematologic malignancies[1]. 97% of cases occur in adults over the age of 40 with a slight male preponderance[2]. It is one of a spectrum of conditions arising from dysfunction of the plasma cell. A series of genetic changes leads to formation of a clonal neoplasm of plasma cells of B lymphocyte origin. Uncontrolled plasma cell proliferation occurs without any stimulus by an antigen. This results in excessive quantities of globulins or their subunits being produced. In addition, changes in the microenvironment of the bone marrow enhances tumour growth and overproduction of monoclonal immunoglobulins and prevents the immune system from controlling disease progression[4]. In the vast majority of cases plasma cell infiltration first appears in the axial skeleton and therefore the first radiological manifestations of multiple myeloma become apparent in the vertebrae, ribs, skull and pelvis[3]. The rapidly expanding population of malignant plasma cells compromises the integrity of bones by physically replacing and eroding the native architecture of the bony trabeculae. The malignant clones also inhibit bone formation and stimulate bone resorption by invading the marrow, adhering to marrow stromal cells and stimulating production of osteoclast-activating factors[5]. Clinically, the most common presenting symptom is that of bone pain. Fatigue and lethargy are also frequently encountered and result from the underlying malignancy as well as anaemia. Patients may also experience increased incidence of infection due to suppression of normal immunoglobulin synthesis. As well as causing symptoms resulting from bone pain and destruction up to 30% of patients suffer from the effects of elevated serum calcium (more often in advanced disease). The most characteristic and frequently seen pattern of bone destruction in multiple myeloma is the presence of either solitary or multiple areas of osteolysis and is seen in over 90% of patients. In the majority of patients with the common osteolytic form of multiple myeloma the axial skeleton is the first site of disease involvement. Eventual spread to the extremities may occur but usually only after disease has been well established in sites such as vertebrae and ribs. Rarely, multiple myeloma with exclusive involvement of the appendicular skeleton has been described but in almost all of these cases it has been in association with concurrent disease of central haemopoeitic sites such as myelofibrosis[3]. This patient has no such synchronous disease process. To the best of our knowledge, a distribution of multiple myeloma such as this is indeed unusual and represents yet another facet of the many manifestations of this widely prevalent condition.

Differential Diagnosis List

Mutiple Myeloma with sparing of the axial skeleton.

Final Diagnosis

Mutiple Myeloma with sparing of the axial skeleton.

Liscense

Figures

Fig 1. Calcaneum

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Fig 1. Calcaneum

Fig 2. Tarsus

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Fig 2. Tarsus

Fig 4. Forearm

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Fig 4. Forearm

Fig 5. Proximal Tibia.

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Fig 5. Proximal Tibia.

Fig 3. Metatarsals

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Fig 3. Metatarsals

Fig 6. Wrist.

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Fig 6. Wrist.