Pathology & Tumour Flashcards

0
Q

Causes of vertebral plana?

A

EG
Infection - especially TB
Ewing’s sarcoma
Metastatic tumour

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1
Q

What are the three forms of Langerhan’s Cell Histiocytosis?

A
  1. EG (localised form) 70%
  2. Hand-Schuller-Christian disease (chronic recurring form) 20%
  3. Lettered-Siwe disease (fulminant form) 10%
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2
Q

Differential diagnosis of osteosarcoma?

A

Osteomyelitis
Ewings
Trauma
Myosotis ossification

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3
Q

Combined vascular malformation sand hypertrophy syndromes

A

Klippel-Trenaunay - capillary/lymphatic/venous

Parkes-Weber syndrome - AVN malformation

Maffuci- enchondroma todos with vascular malformation

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4
Q

DD of exquisitely tender lesion

A
Haemangioma 
Synovial sarcoma
Neuroma
Abscess
Glomus tumour
Fibrosarcoma
Acute inflammation
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5
Q

Macro and micro Characteristics of Tuberculosis

A

Centrally necrotic, caseous cavity surrounded by multi-uncleared giant cells (epitheloid) and lymphocytes
Calcified late
Thickened synovium, effusion and pannus
Increased vascularity - osteopaenia

Absence of - sclerosis and periosteal reaction

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7
Q

Two Differential diagnoses of TB

A

Brucellosis - unpasteurised milk - Mx tetracycline

Rheumatoid

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8
Q

Abx Treatment of TB

A

RIPE

Rifampicin & Isoniazid for 9 months
Pyramidazole for 2 months
Ethambutol- retrobulbar neuritis

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8
Q

Features of congenital syphilis?

A

Treponema pallidum crosses placenta in second half pregnancy
Sick and irritable infant
Hepatosplenomegaly
Joint swelling and pseudoparalysis at several weeks old.
Swelling and tenderness at ends of long bones

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9
Q

What is the organism of syphilis? What is pathology

A

Treponema pallidum - penicillin

Spirochete

Angitis of vasa vasorum or small arterioles. Endarteritis produces vessel wall necrosis and tissue infaction supplied by vessel.

Primary - chancre
Secondary - mucocutaneous lesions and lymphadenopathy
Tertiary - untreated cases. Antibiotics ineffective

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10
Q

Orthopaedic manifestations of syphilis

A

Tabes dorsalis -
Posterior column degeneration
Lightening pains in lower limbs
Sensory ataxia, joint instability and CHARCOT

Gummata -
Punched out lesions in medulla
Destructive lesions in cortex
Pathological fractures

Sabre tibia

Xray
Periostitis- can be onion peel
Metaphysitis-
Trabecular erosion in juxta-epiphyseal region
Lucent band near physis followed by frank destruction
Similar to leukaemia and neuroblastoma

Dd
Scurvy
Multifocal OM
Battered baby syndrome
Caffey's disease - infantile cortical hyperostosis
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11
Q

Define synovial chondromatosis

A

Monoarticular Benign proliferation disorder of the synovium
Chondroid metaplasia of synovium resulting in production of MULTIPLE INTRA-ARTICULAR LOOSE BODIES
Primary

Secondary - degenerative arthritis with cartilage breaking off and growing in joint

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12
Q

Xray feature of synovial chondromatosis and DD

A

Stippled calcification of soft tissues around a joint
unless lesions large and ossified

Degenerative changes if SC is secondary

DD
Tumeral calcinosis
Soft tissue chondroma
Hypercalcaemia

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13
Q

Genetic association for enchondroma (MCQ)

A

IDH1 and IDH2 mutations

Especially Ollier disease and Mafucci syndrome

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14
Q

Define a hamartoma

Name some examples

A

A hamartoma is a benign, focal malformation that resembles a neoplasm in the tissue of its origin. This is not a malignant tumor, and it grows at the same rate as the surrounding tissues. It is composed of tissue elements normally found at that site, but which are growing in a disorganized mass.

Osteochondroma

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15
Q

Genetics for MHE

A

EXT1 & EXT2 proteins & genetic loci
Biosynthesis of heparin sulfate
Decreased expression leads to osteochondromas

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16
Q

What are the syndromes associated with non-ossifying fibromas

A

Familial multi-focal

Neurofibromatosis

Jaffe-Campanacci syndrome
Congenital with cafe-au-lait, mental retardation, non-skeletal abnorms - heart, eyes, gonads

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17
Q

Define fibrous dysplasia

A

Common developmental abnormality characterised by Hamartomatous proliferation of fibro-osseus tissue within bone

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18
Q

Genetics of fibrous dysplasia

A

Dominant activating mutations of GS alpha on chromosome 20q13
Produce a sustained adenylate cyclase- cAMP activation

High expressions of FGF-23
Cause of hypophosphataemia in McCune-Albright

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19
Q

Syndromes with fibrous dysplasia

A

McCune Albright - polyostotic FD, precocious puberty and Coast of Maine spots

Oncogenic osteomalacia- renal phosphate wasting due to FGF 23

Mazabraud syndrome - polyostotic FD, multiple intramuscular myxomas

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20
Q

Genetics of ABCs

A

Express TRE17/USP6 translocation

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21
Q

Chemotherapy for osteosarcoma

A

Adriamycin / doxorubicin - Blocks DNa/RNA synthesis via topoisomerase II

Cisplatinum - DNa disruption by covalent bonding

Methotrexate - inhibits dehydrofolate reductase

Ifosfamide - DNA alkylating agent

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22
Q

Genetics of osteopetrosis

A

Carbonic anhydrase II
TCIRG1 (ATP6i) gene mutation
Chloride channel 7

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23
Q

What are the seronegative arthropathies?

A
Reiter's
Ankylosing spondylitis
Psoriatic 
Enteropathic spondylitis
Lupus associated arthritis

HLA-B27

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24
Q

Cellular Characteristics of rheumatoid synovitis

A

High presence of T-cells in inflamed tissue
Paucity of lymphocytes at invasion front and in synovial fluid

B-cells produce RF - IgM to the Fc of IgG
T-cells regulate inflammatory response

RF complexes activates complement cascade

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25
Q

What are DMARDS?

A

Synthetics
Methotrexate - give with folic acid

Biological
Infliximab- TNF alpha antagonist. Monoclonal antibody

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26
Q

4 causes of back kneeing

A

Polio
Lax posterior capsule
PCL deficiency
Fracture Malunion - femur extension, tibia recurvatum

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27
Q

5 causes of hip pain in Pagets

A
Fracture
Osteoarthritis
Malignant transformation - 2nd Osteosarc 
Active disease 
Spinal stenosis
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28
Q

Causes of Erlenmeyer flask deformity

A
Gauchers
Osteopetrosis
Niemann-Pick disorder
MHE
Lead poisoning 
Cranio Metaphyseal dysplasia
Haemoglobinopathies- sickle / thalassaemia
Metaphyseal dysplasia -Pyle disease
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29
Q

Two benign tumours that can metastasise

A

GCT
Chondroblastoma

Same as only 2 epiphyseal

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30
Q

3 tumours more common in women

A

Fibrous dysplasia
Parosteal osteosarcoma
GCT

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31
Q

5 mimickers

A

Big Five Mice

Fibrous dysplasia
Metastatic bone disease
Infection
Cartilage lesions
EG
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32
Q

Small round blue cell tumours

A

Infants

  • neuroblastoma
  • nephroblastoma - Wilm’s
  • retinoblastoma

Kids

  • EG / LCH
  • Wilm’s

Adults

  • Ewings
  • MM
  • Lymphoma
  • Rhabdomyosarcoma
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33
Q

Classification of bone tumours

1) histological
2) grade
3) staging

A

Lichtenstein - osteogenic, chondrogenic etc

Grade
Low, intermediate, high
Morphological -
Nuclear anaplasia, hyperchromasia, pleomorphism

Enneking - malignant
1 Low grade - 98% 5 yr survival
2High Grade - 80% 5yr survival
3 Metastatic (G1 or G2) 30%
A Intra / B Extra -  compartmental
34
Q

Three conditions with Codman’s triangle

A

Osteosarcoma - high grade
Ewing’s sarcoma - high grade
Infection - osteomyelitis

35
Q

Why perform biopsy in tumour centre? (Evidence)

A

Mankin CORR 2006
Complication rates X5 if performed at non-tumour centre
Errors, amputation, survival rate

36
Q

4 types of biopsy

A

FNA
Core
Incisional
Excisional

37
Q

Tumours with Autumnal hues

A

PVNS

GCT

38
Q

Principles of treating a tumour patient

A
Diagnosis and staging
Discussion with patient and family
Multidisciplinary approach
Determine curative or palliative
Multimodality approach
Skeletal stability 

Multimodal approach

  1. Pharmacological - analgesia, bisphosph
  2. Surgery - limb salvage vs amputation
  3. Chemotherapy
  4. Radiotherapy
39
Q

Criteria for limb salvage

A

Survival rate > amputation
Function > amputation plus prosthesis
Vascular, innervated and stable limb

40
Q

4 surgical margins

A

Intralesional- 100% recurrence
Marginal - through reactive zone - 25-50% recurrence
Wide - through normal tissue - <10% recurrence
Radical - entire compartment - ligaments, CT and muscle

41
Q

Main tumours that chemo used effectively

A

Osteosarcoma
ewings
De differentiated chondrosarcoma
Malignant fibrous Histiocytoma

Neo-adjuvant 8-12weeks preop
Adjuvant - 6-12 months

42
Q

Mode of action of radiotherapy

A

Direct DnA damage

Indirect via Free radical
Keep <50 Gray to avoid tissue healing problems

43
Q

Tumours that radiotherapy effective

A

Ewings
Lymphoma
MM/ Plasmacytoma

Soft tissue sarcoma - no difference in survival but debulks for surgery

44
Q

How to score fracture risk in tumour

A

Mirel’s - CORR 1989

Pain - mild, mod, funct
Size - 2/3
Site - UL, LL, troch
Type - Blastic, mixed, lyric

Score min 4, max 12
7 - 4% Observe and Rx
8 - 15% +/- FIX
9 - 33% FIX
10 - 100% FIX
At 3 months
45
Q

What is diagnosis in known metastatic lung Ca patient who is now confused, weak, dehydrated and n&v?

A

Metastatic hypercalcaemia

Diuretics and fluid hydration

46
Q

How do you determine spine stability in metastatic bone disease?

A

Kostuik criteria

Divides Denis columns into 6
Instability is 3 or more

47
Q

Prognosis of MBD

A

Months

Lung - 6
Kidney - 6

Breast - 24

Thyroid - 40

Prostate - 48

48
Q

Differential for GCT

A

Brown tumour

49
Q

Histological buzzword of chordoma

A

Physaliferous cells

50
Q

Histology of adamantioma

A

Nests of epithelial cells in a fibrous stroma

51
Q

Which tumours are storiform?

A

NOF
Malignant fibrous Histiocytoma

Osteosarcoma
Leimyosarcoma
Haemangioperocytoma
Neurogenic sarcoma

52
Q

Which tumours have coffee bean nuclei?

A

EG / LCH - plus tennis racquet Birbeck granules

Malignant fibrous Histiocytoma

Chondroblastoma

53
Q

Which tumour has clock face nuclei?

A

MM / Plasmacytoma

54
Q

Which tumours have giant cells

A
UBC
ABC
NOF
Ostefibrous dysplasia 
EG / LCH
MFH

Chondroblastoma
Chondromyxoidfibroma
Clear-call chondrosarcoma

55
Q

Which tumour has Chinese letters histology

A

Fibrous dysplasia

Immature fibrous tissue surrounded by islands of irregularly poorly mineralised woven bone trabeculae

56
Q

What is a Codman’s tumour?

A

Chondroblastoma

Benign chondroid tumour characterised by its epiphyseal location.
Similar to GCT but chondroid matrix and occurs in skeletally immature
Mets to lungs - like GCT
Curettage and bone graft as locally aggressive

57
Q

What is the histological differentiation between enchondroma and chondrosarcoma

A

Bone encasement pattern vs permeation pattern

58
Q

What tumour has chicken wire / lace-like calcification

A

Chondroblastoma

59
Q

Main Differential of a NOF - non-ossifying fibroma?

A

Chondro-myxoid fibroma

Rare, benign chondroid tumour
Metaphyseal 
Lytic, eccentric and lobulated
Thinking and cortical expansion 
SHARP, scalloped, sclerotic rim 

Surgical curettage and bone graft

DD for CMF is -
ABC
Chondromyxoid sarcoma
Osteomyelitis

60
Q

What Metaphyseal lesion has a sharp, scalloped sclerotic rim?

A

Chondromyxoidfibroma

DD - NOF, myxoid chondrosarcoma

Myxoid chondrosarcoma -
Clear permeations of surrounding bone and HYPERCELLULARITY THROUGHOUT

61
Q

What are he types of chondrosarcoma

A
Intramedullary
Clear cell
Mesenchymal 
Dedifferentiated 
Secondary
- Osteochondromas
- MHE
- enchondromatosis
62
Q

What is a clear -cell chondrosarcoma

A

The malignant form of a chondroblastoma

63
Q

How are chondrosarcomas graded

A
Differentiated (survival %)
Well (90)
Intermediate (60)
Poor (30)
Dedifferentiated (10)
64
Q

Differentials for a chondrosarcoma

A

Secondary Osteosarcoma
Osteomyelitis
MBD
Malignant fibrous Histiocytoma

65
Q

Differential diagnosis for chordoma

A

Fibrosarcoma
MBD
Chondrosarcoma

66
Q

What is the spectrum of diseases known as histiocytosis x

A

Cell of origin is Langerhan’s cell. Dendritic APC found everywhere especially skin and bones

Eosinophilic granuloma

Hand-Schuller-Christian disease - exophthalmos, diabetes insipidus, bone destruction

Letterer-Siwe hepatosplenomegaly, lymphadenopathy, anaemia, acute infections. Fatal

67
Q

Classic cause of vertebral plana

A

EG

68
Q

What so the pattern of tumours in Olliers / Maffuci disease

A

Multiple enchondromas

Unilateral in long bones, bilateral in hands

69
Q

Prognosticators for Ewings

A
90% tumour necrosis with Neo-adj chemo
Metastatic disease - lung bad, bone terrible
Wide excision 
Pelvic - bad
Increased LDH or ALP bad
70
Q

What is the other name for Trevor’s disease?

A

Dysplasia Epiphysealis Hemimelica

Medial physis overgrowth.
Knee or ankle most common. Valgus

71
Q

What’s the chromosomal translocation of synovial cell sarcoma?

A

X;18

72
Q

What distinguishes PVNS from Synovial cell sarcoma on xray ?

A

SCC is calcified in 50%

73
Q

How are simple UBCs classified?

A

Neer classification

2cm from growth plate - INACTIVE

74
Q

Stanley’s trilogy for transmural bone lesion with large soft tissue component

A

Ewings
Lymphoma
Infection

Also - all small round blue cell

75
Q

What are the different types of osteosarcoma and what are their grades

A

Intramedullary - HIGH
Parosteal - LOW
Periosteal - INTERMEDIATE
Tangiectatic

Secondary - HIgH

  • Pagets
  • Radiation
76
Q

Genetic associations of osteosarcoma

A

RB-1 retinoblastoma gene - 13q/recessive - TSG

P53 gene - 17p/dominant TSG

Retinoblastoma patients have increased risk of OS x1000
Also - Pagets, radiation, Rothmund-Thompson syndrome

77
Q

Describe radiological appearance of osteoid osteoma

A

Well circumscribed intra-cortical lesion with a radiolucent nidus

78
Q

Indications for surgery in spinal metastatic bone disease.

A

Persistent severe pain
Instability
Progressive neurology
Tissue biopsy

79
Q

Radiological findings of Hyperparathyroidism

A

Osteoporosis

Subperisoteal resorption in Middle phalanges of fingers, particularly MF and IF

Erosions at symphysis pubis, distal clavicles, vertebral end plates

Brown tumours

Chondrocalcinosis

80
Q

What are the 6 principles of chronic osteomyelitis management?

A

Surgical debridement of all devitalised tissues
Dead space management
Skeletal stabilisation
Soft tissue coverage
Appropriate antibiotics - local or systemic
Optimise host factors

81
Q

Sclerotic lesions of bone - VINDICATE

A
VINDICATE
Vascular - hamangioma
Infection
Neoplasm- OO, osteosarc
Drugs - Fluoride, Vit D
IDiopathic
Congenital - bone islands, poikiloisis, petrosis
Autoimmune
Trauma
Endocrine - HPTH, Pagets
82
Q

Sclerotic lesions of bone - VINDICATE

A
VINDICATE
Vascular - haemangioma
Infection
Neoplasm- OO, osteosarc
Drugs - Fluoride, Vit D
Idiopathic
Congenital - bone islands, poikiloisis, petrosis
Autoimmune
Trauma
Endocrine - HPTH, Pagets
83
Q

Risk factors for OA

A

Systemic - age, ethnic origin, sex, bone density, hormones, nutrition, genetics, obesity
LOCAL - Trauma, instability, malalignment, laxity, weakness

Factors for 2nd OA
Acromegaly, Hyper PTH, Pagets
Gout, Pseudo, chondrocalcinosis
Trauma, AVN, EDS, Septis, Charcot, DDH
Wilsons, Amyloid, haemoglobinopathies