Pathology: Malignant Bone Tumours

Question 1

what age group are primary bone tumours common in

Answer 1

younger

Question 2

what do bony lesions in older population usually relate to

Answer 2

metastases from epithelial malignancies

Question 3

describe the pain that metastatic cancer affecting the bone produces

Answer 3

severe and usually worse at night systemic symptoms may present

Question 4

when do 1y bone tumours present

Answer 4

late - tend to have already metastasised some have an ill defined bony swelling

Question 5

what is seen on X ray

Answer 5

cortical destruction, periosteal reaction, new bone formation and extension into surrounding soft tissue

Question 6

what is a periosteal reaction

Answer 6

formation of new bone in response to periosteum (injury to)

Question 7

most common form of primary bone tumour

Answer 7

osteosarcoma

Question 8

what mutation are most osteosarcomas associated with

Answer 8

mutations in Retinoblastoma gene (tumour suppressor protein)

Question 9

where do most osteosarcomas occur

Answer 9

60% bones around knee also proximal femur, proximal humerus and pelvis

Question 10

what can prolong survival with osteosarcomas

Answer 10

chemotherapy

Question 11

chondrosarcoma

Answer 11

cartilage producing primary bone tumour

Question 12

what age group does chondrosarcoma tend to present in

Answer 12

older (45)

Question 13

where do chondrosarcoma tend to occur

Answer 13

pelvis and proximal femur

Question 14

describe the characteristics of chondrosarcoma

Answer 14

large and slow to metastasise compared to osteosarcomas which are fairly aggressive

Question 15

what is the prognosis of chondrosarcoma dependent on

Answer 15

histological grade - majority low grade

Question 16

what is the effect of radio and chemo therapy on chondrosarcoma

Answer 16

not effective

Question 17

who is Ewings sarcoma most commonly seen in

Answer 17

teenagers , highly malignant

Question 18

where do Ewings sarcomas tend to occur

Answer 18

along long bones

Question 19

what is the radiological appearance of Ewings sarcoma

Answer 19

onion skin

Question 20

what group do ewings sarcoma belong to

Answer 20

small round blue cell tumours - wide DD

Question 21

what translocation is Ewings sarcoma associated with

Answer 21

t11:22 translocation involving EWS gene on chromosome 22

Question 22

what CF is Ewings sarcoma assoicated with

Answer 22

fever, raised inflammatory markers and warm swelling (may be misdiagnosed as osteomyelitis)

Question 23

is ewings sarcoma radio or chemo sensitive

Answer 23

both

Question 24

outline the treatment of primary bone tumours

Answer 24

usually surgery to remove the tumour and surrounding tissue to prevent recurrence

amputations and limb salvage surgery also used

Question 25

what are the staging investigations for primary bone tumours

Answer 25

bone scan and CT

chest MRI and CT are useful to determine the local extent of tumour and involvement of muscle and vessels etc

Question 26

what must be done prior to surgery

Answer 26

biopsy for histological diagnosis grading

Question 27

what must be done after surgery

Answer 27

joint involved needs reconstruction with special joint replacements

Question 28

when are radio and chemo used

Answer 28

when appropriate neo- adjuvant chemo (prior to surgery) can improve survival

Question 29

what is lymphoma called when it presents as a primary bone tumour

Answer 29

non hodgkins lymphoma

Question 30

where does 1y lymphoma of the bone tend to affect

Answer 30

pelvis or femur

Question 31

how does one treat 1y lymphoma of the bone

Answer 31

surgical resection

Question 32

what CF may be present with metastatic lymphoma

Answer 32

lymphadenopathy or splenomegaly - treat with chemo/radio

Question 33

where does myeloma arise from

Answer 33

marrow

Question 34

what is a solitary lesion of myeloma referred to as

Answer 34

plasmacytoma

Question 35

what are multiple lesions of myeloma referred to as

Answer 35

multiple myeloma

Question 36

how does myeloma present

Answer 36

malignant clonal proliferation of abnormal plasma cells, these produce abnormal proteins called PARAPROTEINS (defective Immunoglobulin) that are deposited throughout many organ sites

Question 37

what is deposition of abnormal proteins known as

Answer 37

amyloidosis

Question 38

what is the amyloidosis known as in myeloma

Answer 38

AL amyloid - protein represents immunoglobulin light chain

Question 39

what age are the patients with myeloma typically

Answer 39

45-65

Question 40

how do patients with multiple myeloma typically present

Answer 40

weakness, back pain, bone pain, fatigue and weight loss

marrow suppression - anaemia and recurrent infection

pathological fracture

Question 41

how is myeloma diagnosed

Answer 41

identification of the abnormal proteins either by plasma protein electrophoresis and/or early morning urine collection for Bence Jones protein assay

Question 42

what will the plasma protein electrophoresis show in myeloma

Answer 42

high level of paraprotein

Question 43

treatment of myeloma

Answer 43

solitary plasmacytoma - radio

multiple myeloma - chemo

Question 44

name the primary malignant tumours that commonly metastasise to bone (in order of frequency)

Answer 44

breast

prostate

lung

renal

thyroid

Question 45

what may the pain of bone metastases frequently be diagnosed as

Answer 45

muscle strain

Question 46

how are fractures or impending fractures treated

Answer 46

stabilisation using long rods if destruction of the joint replacement may be better

Question 47

The most common form of primary bone tumour, producing abnormal bone. Most cases are seen in younger age groups (adolescence and early adulthood) with 60% involving the bones around the knee.

Answer 47

osteosarcoma

Question 48

A malignant tumour of unknown histogenesis characterised by the t(11;22) translocation.

Answer 48

ewings sarcoma

Question 49

A malignant tumour that only rarely occurs in bone and usually only if the bone is abnormal (Paget’s etc.).

Answer 49

fibrosarcoma