Primary Malignant Bone Tumours

Question 1

Significant bone pain or a defined bony swelling in a person under 25

Answer 1

refer for urgent investigation with at least x-rays

Question 2

Signs of primary malignant bone tumours on x-ray

Answer 2

Agressive destruction
Cortical destruction
Raised periosteal reaction
New bone formation ( sclerosis as well as lysis)
Extension into surrounding soft tissue envelope

Question 3

What is the most common malignant primary bone tumour?

Answer 3

Osteosarcoma

Mostly seen in younger people (adolescence and early adulthood)
Often involving bones around the knee, proximal femur, proximal humerus, and pelvis
Metatstatic spread is haematogenous but can sometimes be lymphatic

10% of patients have pulmonary mets at diagnosis
Adjuvant chemotherapy can prolong survival

Question 4

Chondrosarcoma

Answer 4

Cartilage producing primary bone tumour
Less common and agressive than osteosarcoma
Older age group - mean age 45

Very large but slow to met
Found in pelvis or proximal femur
Prognosis dependent on histological grade
Not radiosensitive and unresponsive to adjuvant chemotherapy

Question 5

Fibrosarcoma and Malignant Fibrous Histiocytoma

Answer 5

Fibrous malignant primary bone tumours which occur in abnormal bone

(bone infarct, fibrous dysplasia, post irradiation, paget’s disease)

Fibrosarcoma mostly occurs in adolescents

Question 6

Ewing’s Sarcoma

Answer 6

Malignant tumour of primative cells in the marrow
2nd most prevalent and poorest prognosis

Most cases between 10-20 years old

Associated with fever, raised inflammatory markers, and a warm swelling - may be misdiagnosed as osteomyelitis due to this

Mostly radio/chemosensitive

Question 7

How are primary tumours treated?

Answer 7

Surgical removal of tumour and surrounding tissue
+
Adjuvant chemotherapy and radiotherapy if appropriate, plus neo-adjuvant therapy before surgery

Question 8

Lymphoma

Answer 8

Cancer of lymphocytic system - can occur as a primary bone tumour (non-Hodgkins lymphoma)
Pelvis/Femur

Question 9

Myeloma

Answer 9

Malignant proliferation of B cells from the marrow and can present as a solitary lesion - plasmacytoma

Patients 45-60 years
Weakness, back pain, bone pain, fatigue, weight loss, marrow suppression - anaemia and recurrent infection

Diagnosis by plasma protein electrophosesis - high level of paraprotein
And Bence Jones protein assay - morning urine collection

Solitary - radio
Multiple myeloma - chemo