Definition
- primary malignant tumour caused by malignant proliferation of plasma cells
- Also known as Kahler’s disease
Incidence
- Most common primary malignant tumour of bone
- 27-44% of biopsied bone tumours
- 1% of all malignant disease
- Average age is 60
- Rare in patients less than 40 years old
- M>F by 2:1
Localization
- Spine is commonest, particularly lower thoracic & lumbar areas
- Marrow rich flat bones of pelvis, skull, ribs, clavicle & scapula are frequently involved
- In long bones femur & humerus are much more commonly affected than distal appendicular skeleton
Pathology
Gross
- Multiple permeative lesions which are filled by gelatinous, red, soft masses of neoplastic plasma cells, “current jelly” in appearance lesion is usually multicentric
Microscopic
- Sheets of closely packed cells with basophilic cytoplasm cells are usually uniform
- few mitotic figures nuclei have clock face appearance
- surrounding cytoplasm has perinuclear clear zone or halo (which is taken up by Golgi apparatus)
- Occasionally only amyloid may be seen in specimens
Immunohistochemistry
- CD56, natural killer antigen, is frequently seen on myeloma cells but not reactive plasma cells
Signs & symptoms
- Pain
- cardinal initial symptom
- Initially intermittent & becomes continuous
- acute sudden exacerbation may represent pathological fracture
- Paraplegia may occur with vertebral collapse
Other presentations:
- Anaemia
- Infection
- Renal failure
- Hypercalcaemia
- Weight loss
- Amyloidosis
- Neurological findings
- Peripheral polyneuropathy
Investigations
Laboratory
- FBC
- Anaemia – often normochromic normocytic
- Thrombocytopaenia
- ESR
- elevated
- Electrolytes
- Hypercalcaemia in 20-50% of patients
- Normal phosphate
- Hyperuricaemia
- Serum proteins
- Hyperglobulinaemia with reversal of albumin-globulin ratio
- Beta –2 microglobulin
- elevation is poor prognostic finding
- Urine
- Bence Jones proteins
- Bone marrow aspiration
- diagnostic
Xray
- Diffuse osteoporosis
- first sign of myeloma
- Usually most evident within spine
- Goes on to result in pathologic vertebral collapse, which may cause vertebra plana or so called wrinkled vertebra
- Osteolytic defects
- Punched out, sharply circumscribed
- No surrounding sclerosis
- Most frequently seen in bones with haematopoietic potential
- ie skull, pelvis, long bones, clavicles & ribs
- In long bones diaphysis is most often involved
- In vertebra pedicles are often preserved
- In skull this is referred to as raindrop skull
- Myelomatous deposits in skull are relatively uniform in size If lesions vary lot in size they are more likely to be secondaries
- ribs may have “ballooned out” appearance
- Sclerotic lesions
- Less than 3% of patients have sclerosis on their X-rays
- Can have solitary ivory vertebra
- sclerotic form of disease is associated with peripheral demyelinating symmetrical polyneuropathy
- With sclerotic lesions differential diagnosis includes:
- Osteoblastic metastasis
- Mastocytosis
- Lymphoma
- Myelosclerosis
Bone Scan
- Bone scans are cold except at site of pathological fractures
- This is because of deficiency of osteoblastic activity at site of lesion
Differential diagnosis
- Metastatic carcinoma
- Lymphoma
- Hyperparathyroidism
Treatment
- Supportive treatment
- including rehydration & management of hypercalcaemia may be necessary before definitive treatment
- Radiotherapy
- treatment of choice for localized disease, after excisional biopsy if possible
- Chemotherapy
- Prophylactic long bone fixation, or tumour prosthesis if required around joints
Prognosis
- 90% dead within 3 years
- Most deaths are due to renal failure
Plasmacytoma (solitary myeloma)
- This is forerunner of disseminated myeloma
- Commonest site is vertebral column
- overall survival rate was 74% at 5 years & 45% at 10 years