Pathology & Tumour Flashcards

(84 cards)

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
Cellular Characteristics of rheumatoid synovitis
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
25
What are DMARDS?
Synthetics Methotrexate - give with folic acid Biological Infliximab- TNF alpha antagonist. Monoclonal antibody
26
4 causes of back kneeing
Polio Lax posterior capsule PCL deficiency Fracture Malunion - femur extension, tibia recurvatum
27
5 causes of hip pain in Pagets
``` Fracture Osteoarthritis Malignant transformation - 2nd Osteosarc Active disease Spinal stenosis ```
28
Causes of Erlenmeyer flask deformity
``` Gauchers Osteopetrosis Niemann-Pick disorder MHE Lead poisoning Cranio Metaphyseal dysplasia Haemoglobinopathies- sickle / thalassaemia Metaphyseal dysplasia -Pyle disease ```
29
Two benign tumours that can metastasise
GCT Chondroblastoma Same as only 2 epiphyseal
30
3 tumours more common in women
Fibrous dysplasia Parosteal osteosarcoma GCT
31
5 mimickers
Big Five Mice ``` Fibrous dysplasia Metastatic bone disease Infection Cartilage lesions EG ```
32
Small round blue cell tumours
Infants - neuroblastoma - nephroblastoma - Wilm's - retinoblastoma Kids - EG / LCH - Wilm's Adults - Ewings - MM - Lymphoma - Rhabdomyosarcoma
33
Classification of bone tumours 1) histological 2) grade 3) staging
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
Three conditions with Codman's triangle
Osteosarcoma - high grade Ewing's sarcoma - high grade Infection - osteomyelitis
35
Why perform biopsy in tumour centre? (Evidence)
Mankin CORR 2006 Complication rates X5 if performed at non-tumour centre Errors, amputation, survival rate
36
4 types of biopsy
FNA Core Incisional Excisional
37
Tumours with Autumnal hues
PVNS | GCT
38
Principles of treating a tumour patient
``` 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
Criteria for limb salvage
Survival rate > amputation Function > amputation plus prosthesis Vascular, innervated and stable limb
40
4 surgical margins
Intralesional- 100% recurrence Marginal - through reactive zone - 25-50% recurrence Wide - through normal tissue - <10% recurrence Radical - entire compartment - ligaments, CT and muscle
41
Main tumours that chemo used effectively
Osteosarcoma ewings De differentiated chondrosarcoma Malignant fibrous Histiocytoma Neo-adjuvant 8-12weeks preop Adjuvant - 6-12 months
42
Mode of action of radiotherapy
Direct DnA damage Indirect via Free radical Keep <50 Gray to avoid tissue healing problems
43
Tumours that radiotherapy effective
Ewings Lymphoma MM/ Plasmacytoma Soft tissue sarcoma - no difference in survival but debulks for surgery
44
How to score fracture risk in tumour
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
What is diagnosis in known metastatic lung Ca patient who is now confused, weak, dehydrated and n&v?
Metastatic hypercalcaemia Diuretics and fluid hydration
46
How do you determine spine stability in metastatic bone disease?
Kostuik criteria Divides Denis columns into 6 Instability is 3 or more
47
Prognosis of MBD
Months Lung - 6 Kidney - 6 Breast - 24 Thyroid - 40 Prostate - 48
48
Differential for GCT
Brown tumour
49
Histological buzzword of chordoma
Physaliferous cells
50
Histology of adamantioma
Nests of epithelial cells in a fibrous stroma
51
Which tumours are storiform?
NOF Malignant fibrous Histiocytoma Osteosarcoma Leimyosarcoma Haemangioperocytoma Neurogenic sarcoma
52
Which tumours have coffee bean nuclei?
EG / LCH - plus tennis racquet Birbeck granules Malignant fibrous Histiocytoma Chondroblastoma
53
Which tumour has clock face nuclei?
MM / Plasmacytoma
54
Which tumours have giant cells
``` UBC ABC NOF Ostefibrous dysplasia EG / LCH MFH ``` Chondroblastoma Chondromyxoidfibroma Clear-call chondrosarcoma
55
Which tumour has Chinese letters histology
Fibrous dysplasia Immature fibrous tissue surrounded by islands of irregularly poorly mineralised woven bone trabeculae
56
What is a Codman's tumour?
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
What is the histological differentiation between enchondroma and chondrosarcoma
Bone encasement pattern vs permeation pattern
58
What tumour has chicken wire / lace-like calcification
Chondroblastoma
59
Main Differential of a NOF - non-ossifying fibroma?
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
What Metaphyseal lesion has a sharp, scalloped sclerotic rim?
Chondromyxoidfibroma DD - NOF, myxoid chondrosarcoma Myxoid chondrosarcoma - Clear permeations of surrounding bone and HYPERCELLULARITY THROUGHOUT
61
What are he types of chondrosarcoma
``` Intramedullary Clear cell Mesenchymal Dedifferentiated Secondary - Osteochondromas - MHE - enchondromatosis ```
62
What is a clear -cell chondrosarcoma
The malignant form of a chondroblastoma
63
How are chondrosarcomas graded
``` Differentiated (survival %) Well (90) Intermediate (60) Poor (30) Dedifferentiated (10) ```
64
Differentials for a chondrosarcoma
Secondary Osteosarcoma Osteomyelitis MBD Malignant fibrous Histiocytoma
65
Differential diagnosis for chordoma
Fibrosarcoma MBD Chondrosarcoma
66
What is the spectrum of diseases known as histiocytosis x
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
Classic cause of vertebral plana
EG
68
What so the pattern of tumours in Olliers / Maffuci disease
Multiple enchondromas Unilateral in long bones, bilateral in hands
69
Prognosticators for Ewings
``` 90% tumour necrosis with Neo-adj chemo Metastatic disease - lung bad, bone terrible Wide excision Pelvic - bad Increased LDH or ALP bad ```
70
What is the other name for Trevor's disease?
Dysplasia Epiphysealis Hemimelica Medial physis overgrowth. Knee or ankle most common. Valgus
71
What's the chromosomal translocation of synovial cell sarcoma?
X;18
72
What distinguishes PVNS from Synovial cell sarcoma on xray ?
SCC is calcified in 50%
73
How are simple UBCs classified?
Neer classification 2cm from growth plate - INACTIVE
74
Stanley's trilogy for transmural bone lesion with large soft tissue component
Ewings Lymphoma Infection Also - all small round blue cell
75
What are the different types of osteosarcoma and what are their grades
Intramedullary - HIGH Parosteal - LOW Periosteal - INTERMEDIATE Tangiectatic Secondary - HIgH - Pagets - Radiation
76
Genetic associations of osteosarcoma
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
Describe radiological appearance of osteoid osteoma
Well circumscribed intra-cortical lesion with a radiolucent nidus
78
Indications for surgery in spinal metastatic bone disease.
Persistent severe pain Instability Progressive neurology Tissue biopsy
79
Radiological findings of Hyperparathyroidism
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
What are the 6 principles of chronic osteomyelitis management?
Surgical debridement of all devitalised tissues Dead space management Skeletal stabilisation Soft tissue coverage Appropriate antibiotics - local or systemic Optimise host factors
81
Sclerotic lesions of bone - VINDICATE
``` VINDICATE Vascular - hamangioma Infection Neoplasm- OO, osteosarc Drugs - Fluoride, Vit D IDiopathic Congenital - bone islands, poikiloisis, petrosis Autoimmune Trauma Endocrine - HPTH, Pagets ```
82
Sclerotic lesions of bone - VINDICATE
``` VINDICATE Vascular - haemangioma Infection Neoplasm- OO, osteosarc Drugs - Fluoride, Vit D Idiopathic Congenital - bone islands, poikiloisis, petrosis Autoimmune Trauma Endocrine - HPTH, Pagets ```
83
Risk factors for OA
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 ```