Radiological manifestations of malignant hyperparathyroidism, plasmacytoma and multiple myeloma

Clinical Cases 08.03.2006
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 66 years, female
Authors: JSH. Gaskin, N. Gopalakrishnan, DI. Gaskin
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Details
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AI Report

Clinical History

A fit and healthy female patient was exercising when she has severe pain in her left arm. X-rays of the humerus, skull and chest as well as CT scans and related blood chemistry comfirmed the diagnosis of pathological fracture, malignant hyperparathyroidism, solitary osseous plasmacytoma and multiple myeloma.

Imaging Findings

The patient was asymptomatic and was exercising when she experienced severe pain in her left arm. X-rays of the humerus (Fig 1) showed a mid-shaft transverse fracture through an osteolytic lesion. Sub-cortical bone resorption (brown tumors) was also noted (Fig 2). X-rays of the skull showed multiple osteolytic lesions (Fig 3). X-rays of the fingers showed sub-periosteal bone resorption (Fig 5). Chest X-rays showed a 3-4 cm diameter soft tissue mass projected over the anterior end of the right 3rd rib and into the chest cavity with multiple lytic lesions in the ribs (Fig 4). This chest lesion was seen on Computer Tomography (CT) (Fig 6) and noted to be involving the 3rd rib (Fig 7). A solitary metastasis was also noted in the liver on CT scan (Fig 8). It was confirmed to be a plasmacytoma involving the 3rd rib on CT guided biopsy and histology. Biochemical investigations showed hypercalcaemia (Ca 2.7mmol/l), hypophosphataemia (PO4 0.44mmol/l), elevated alkaline phosphatase (ALP 182u/l) and a low parathormone (5 pg/ml). Protein electrophoresis revealed light chain monoclonal immunoglobulins and bence-jones proteins. The humeral fracture was fixed with a retrograde intra-medullary nail and the patient underwent a course of radiotherapy.

Discussion

Malignant hyperparathyroidism occurs due to production of parathyroid- related proteins(PTHrP) by some tumours, particularly of pulmonary origin. The PTHrP mimics PTH resulting in hypercalcaemia and a low PTH. Radiographic signs of hyperparathyroidism result. These include sub-cortical bone resorption also called brown tumors(1) or osteitis fibrosa et cystica. This was evident in the humeral diaphysis adjacent to the pathological fracture. Sub-periosteal bone resorption also occurs, especially in the hands(2). Another sign is the ‘pepper-pot skull’ due to multiple osteolytic lesions. This is also seen in multiple myeloma. Plasmacytomas are plasma cell tumours. They can be of bony origin i.e. osseous plasmacytomas, and are then usually solitary, or originate from soft tissues i.e. extramedullary plasmacytomas (EMP). Their prognosis depends on their ability to progress to multiple myeloma. Progression is more likely in solitary osseous plasmacytomas (SOP) which thus have the worse prognosis. Knowling in his series found that 16% of patients with SOP who were treated with radiotherapy were progression free at 10 years in comparison to 71% of EMP patients(3). Bolek in a retrospective review of 37 patients concluded that radiotherapy was an effective local treatment for solitary plasmacytomas[4]. In our case the chest lesion originated from the 3rd rib and CT guided biopsy confirmed a plasmacytoma. Bence-jones proteins and light chain monoclonal immunoglobulin confirmed multiple myeloma. This case shows how relatively advance disease can present incidentally (in this case as a pathological fracture) and how plasmacytoma can produce malignant hyperparathyroidism and progress to multiple myeloma.

Differential Diagnosis List

Final Diagnosis

Liscense

Figures

X-ray of the humerus showing a mid-shaft pathological fracture

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X-ray of the humerus showing a mid-shaft pathological fracture

X-ray of the humerus showing sub-cortical bone resorption (brown tumours or osteoclastomas)

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X-ray of the humerus showing sub-cortical bone resorption (brown tumours or osteoclastomas)

X-rays of the fingers showing sub-periosteal bone resorption

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X-rays of the fingers showing sub-periosteal bone resorption

X-ray of the skull showing multiple osteolytic lesions (pepper-pot skull)

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X-ray of the skull showing multiple osteolytic lesions (pepper-pot skull)

CT scan of the liver showing solitary metastasis

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CT scan of the liver showing solitary metastasis

X-ray of the chest showing soft tissue mass projecting from the 3rd rib (osseous plasmacytoma) and multiple lytic lesions of the ribs (multiple myeloma)

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X-ray of the chest showing soft tissue mass projecting from the 3rd rib (osseous plasmacytoma) and multiple lytic lesions of the ribs (multiple myeloma)

CT scan of the chest showing a pleural based mass in the anterior right hemi-thorax destroying the adjacent 3rd rib

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CT scan of the chest showing a pleural based mass in the anterior right hemi-thorax destroying the adjacent 3rd rib

CT scan of the chest showing a pleural based mass in the anterior right hemi-thorax

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CT scan of the chest showing a pleural based mass in the anterior right hemi-thorax