Paraneoplastic syndromes

Question 1

Paraneoplastic syndromes three physiological causes

Answer 1

Tumour production of substances directly or indirectly cause distant symptoms
Depletetion normal substances -> paraneoplastic manifestation
Host response to tumour that results -> syndrome (auto-immunological)

Question 2

Range of paraneoplastic syndroems

Answer 2

Endocrinologic
Haematologic
Gastrointestinal
Renal
Cutaneous
Neurologic

Question 3

What causes cushings paraneoplastic syndroem

Answer 3

ACTH release - ACTH
Precursors - MSH, b-lipotroin, endorphins, encephalins
-> cushings syndrome

Question 4

Cancer types causing ectopic ACTH

Answer 4

SCLC
NSCLC
Pancreatic
Thymic
Carcinoid tumours
Phaechromocytoma
Medullary carcinoma of thyroid

Question 5

Presentation of ectopic ACTH syndrome

Answer 5

Rapid onset
Marked weakbess secondary to proximal myopatjy
Hyper-pigmentation
Metabolic disturbance eg hyperglycaemia, hypokalaemic alkalosis

Question 6

Diagnosis of ectopic ACTH

Answer 6

Clinical features - hyperpigmentation, myopahty, hypokalaemia, metabolic alkalosis, high 24hr urinary cortisol, high plasma ACTH/precursors, no response to high dose dex suppression or corticotropin releasing homrone stimulation

Question 7

treatment for ectopic ACTH syndrome

Answer 7

Specific anti-tumour treatment
Decrease cortisol secretion surgically - bilateral adrenalectomy
efically - ocreotide, ketoconazole, aminogluthethamide

Question 8

Most common endocrine paraneoplastic syndrome

Answer 8

Innapropriate Anti diuretic hormone - aginine vasopressin

Question 9

Causes of innappropriate SIADH secretion

Answer 9

CNS - infections, vasculitis, stroke, head injury, tumours, Guillain Barre syndrome, acute intermittent prophyria, psychological stress

Question 10

Causes of innapropriate secretion drugs

Answer 10

Drugs - vincristine, cyclophosphamide, morphine, chlorpropramide, thiazides, clofibrate, carbamazepine, cisplatin

Question 11

Pulmonary causes of innapropriate anitdieuresis

Answer 11

Pulmonary - infections, tumours, positive pressure ventialtion, pneumothorax, asthma, CF,

Question 12

Cancers causng innapropriate antidiuresis

Answer 12

SCLC, pancreatic, prostate, NHL, HD

Question 13

Presentation of innappropriate antidiuresis

Answer 13

CNS toxicity = fatigue, headaches, progress altered mental state, confusion and seizures
Often asymptomatic
Exclde CNS disease, pulmonary disease, drug induced

Question 14

Treatment for inappropriate antidiuresis

Answer 14

Fluid restriction (0.5-1.0L/day)
Democlocycline

Question 15

Lab criteria for SIADH

Answer 15

Hyponatremia Na<130mmol/L
Serum hypo-osmolarity <275 mosm/kg
Urinary osmolarity>serum
Urinary sodium >25 mmol/L
Non suppressed ADH

Question 16

What tumours can secrete gonadotrophins

Answer 16

Pituitary tumours
Gestational trophoblastic tumours
Germ cell tumours
Hepatoblastomas in children and lung tumours

Question 17

Investigaitons for gynaecomastia in a male

Answer 17

Testicular exam
CXR/CT scans

Question 18

Exxtragonadal tumurs secreting beta hCG

Answer 18

Lung
Adrenal
Hepatoma
GI tract tumours
Genitourinary tumours

Question 19

What type of cancers cell wise more likey -> hypercalcemia

Answer 19

Squamous cell -> PTH-rP

Question 20

What is hypercalcemia in breast cancer most commonly from

Answer 20

Bone mets
Rarely from PTH-rP

Question 21

Presentation of hypercalcemia

Answer 21

NAUSEA
Polyuria
Polydipsia
Dehydration
Cardiac arrhythmias
Constipation

Question 22

Diagnosis of hypercalcemia

Answer 22

Serum coorected calcium >2.7 mmol/L
Serum chloride low
Hypercaluria
High urinary phosphate
Low or undetectable palasma PTH hormone

Question 23

When is peak effect of IV bisphophonate in calcium

Answer 23

Severeal days after given

Question 24

What is a encephalomyopathy

Answer 24

Petivascular inflammation and selective neuronal degeneration at several levels of nervous system

Question 25

What sites can encephalomyopathies affect

Answer 25

Limbic system Brainstem and spinal cord Loss of neurons in amygdala, hippocampus and insular cortex gliosis Lymphocyte cuffing of blood vessels Microglial nodules

Question 26

What cancers cause ecephalomyopathies

Answer 26

75% - SCLC Breast, ovary, NHL

Question 27

Presentation of encephalomyopathies

Answer 27

Slow, subacute onset Progressive Loss of short term memory hallucinations Fit/seizure Personality changes

Question 28

Diangosis of encephalomyopathies

Answer 28

CSF - raised protein/IgG level, pelocytosis Serum: anti-Hu antibody MRI

Question 29

Treatment for encepahlomyopathies

Answer 29

Anti-tumour theraoy

Question 30

Prognosis of encephalomyopathies

Answer 30

Can persist after cancer treated due to anitbodies still present in immune system

Question 31

Ovarian cancer encepahlomyopathy symptoms

Answer 31

Anti-Hu antibodies attack cerebellum Ataxic gait - may be in wheel chair

Question 32

What is lambert eaton myasthenic syndrome

Answer 32

Disorder of NMJ Reduced pre-synaptic calcium dependent acetylcholine release 60% of patients with LEMS have cancer

Question 33

Cancers causing LEMS

Answer 33

SCLC - 60-70% Breast, thymus, GIT cacners

Question 34

Presenation of LEMS

Answer 34

Proximal muscle weakness Sparing of bulbar muscles (unlike Myasthenia gravis) 30% dysphagia

Question 35

Diagnosis of LEMS

Answer 35

EMG - normal condction velocity Low amplitude compund mucel action potential nears normal after exercise

Question 36

Treatment for LEMS

Answer 36

Treat cancer Corticosteroids PLasma exchange - high resposne rate Cholinesterase inhibitors usually ineffective

Question 37

Cancers ass w dermatomyositis

Answer 37

NSCLC, SCLC, breat, ovary, GIT cancers

Question 38

Presentation of dermatomyosutus

Answer 38

Proximal myopathy SKin changes Cardiopulmonary conditions, arthralgias, retinopathy

Question 39

Diagnosis of dermatomyosits/polymyositis

Answer 39

Serum: high CK, LDH, aldolase Muscle biopsy - mysotisia and inflammatory degeneration EMG - fibrillation, insertion irritability, short polyphasic motor units

Question 40

Treatment for dermatomyositis

Answer 40

Search for and treat tumoru - 40% have cancer Corticosteroids Azathioprine Inconsistent course often independent of tumour

Question 41

Waht cancer can acanthosis nigricans be ass with

Answer 41

Distal third oesophagus or stomach Teenagers - insulin resistance

Question 42

What is erythema giratum repens

Answer 42

Seen w cervical cancer and bowel ccancer

Question 43

Haematological manifestiations in cancer

Answer 43

Erythrocytosis - EPO Anaemia Granulocytosis/granulocytopenia Thrombocytpsis/thromboxytopenia Thrombophlebitis Coagulopathies and diseeminated IV coagulation Nonbacterial thrombotic endocarditis