paraneoplastic syndromes

Question 1

what causes paraneoplastic syndromes?

Answer 1

• malignancy secreted hormones
• cytokines / host immune response
• cross reaction of tumour antibodies
• depletion of normal factors

Question 2

which tumours can produce cushings syndrome and how?

Answer 2

small cell lung cancer by production of ACTH

also pancreas, lymphomas, prostate

Question 3

which tumours can produce hyperparathyroidims and how?

Answer 3

non small cell lung cancer - squamous cell cancer

also oesophagus, skin, cervix, breast and kidney

by production of PTHrP

Question 4

which tumours can produce syndrome of inappropriate ADH and how?

Answer 4

small cell lung cancer
also lymphoma, pancreas and prostate

by secretion of ADH

Question 5

which tumours secreted activated factor X and what effect does this have?

Answer 5

ovary, pancreas, breast and prostate

prothrombotic tendency (Trousseau syndrome)

Question 6

which tumours are known to lead to significant cachexia and why?

Answer 6

lung (usually NSCLC)
CRC

due to release of cytokines

Question 7

which tumours secrete erythropoietin and what affect can this have?

Answer 7

kidney

leads to polycythemia

Question 8

which hormone is secreted by the following tumours and what affect does it have?

• gastrinoma
• germ cell tumours
• glucagonoma
• insulinoma
• ovarian/ testicular cancers

Answer 8

• gastrin leads to peptic ulcer
• hCG - leads to gynaecomastic
• glucagon - muscle wasting and erythema
• insulin - hypoglycaemia
• oestrogens - gynaecomastia

Question 9

which tumour secretes serotonin? what does this lead to?

Answer 9

neuroendocrine tumours and carcinoid tumours

leads to carcinoid syndrome

Question 10

what is the hormone secreted in pheochromocytoma and what effect does it have?

Answer 10

adrenaline and NA

HTN, flushing, headache, palpitations

Question 11

where do carcinoid syndromes originate from?

Answer 11

Enterochromaffin cells from GIT, pancreas, lung

Question 12

describe pathophysiology behind carcinoid tumours?

Answer 12

tumours produce serotonin

(if GIT tumour then usually 5HT is degraded by liver in first pass effect, however if there are liver mets impairing function OR 5HT is being made outside the portal system then 5HT can build up)

serotonin can cause vasomotor flushing, diarrhoea, wheezing, fever and abdominal pain

Question 13

how is carcinoid syndrome diagnosed?

Answer 13

24 hour urinary collection of 5HIAA

Question 14

what are the chronic complications of carcinoid syndrome?

Answer 14

tricuspid incompetence
cirrhosis
arthropathy
pellagra

Question 15

what are the neurological manafestations of paraneoplastic syndromes?

Answer 15

Abs can be made by tumour cells that cross react with neuronal or muscle tissue
e.g.

Lambert eaton myasthenic syndrome

peripheral neuropathy due to axonal degeneration and demyelination

encephalomyelitis - symptoms depend on region of brain e.g. cerebellar symptoms

Question 16

which cancers are commonly indicated in producing neurological paraneoplastic syndromes?

Answer 16

lung, pancreas, breast, prostate, ovary and lymphoma

Question 17

does treating cancer always reduce the paraneoplastic syndromes?

Answer 17

if they are due to hormone secretion by tumour cells - yes

however auto antibodies are also unaffected and thus syndrome can continue post treatment of cancer

Question 18

what is lambert eaton myasthenic syndrome?

Answer 18

Ab to presynaptic Ca Channels resulting in proximal muscle weakness which improves with exercise.
new onset myasthenia like ptosis should prompt consideration of underlying malignancy

Question 19

list some cutaneous manifestations of paraneoplastic syndromes

Answer 19

pruritis - lymphoma, leukaemia, CNS tumour

acanthosis nigricans - oesophagastric cancer

pemphigus - blisters to skin and mucus membranes - lymphoma, thymus, kaposki sarcoma

vitiligo - malignant melanoma - immune response to melanocytes

dermatomyositis

erythema gyratum - wood grain appearance

Question 20

what malignancy is dermatomyositis associated with?

Answer 20

in 40 % of cases there is cancer

commonly oesophagogastric, lung, CRC, ovarian and breast

Question 21

what cancer is erythema gyratum associated with?

Answer 21

lung , breast, cervix and GI

strongly suggestive of malignancy

Question 22

what are the haematological manifestations of paraneoplastic syndromes?

Answer 22

anaemia

• normocytic normochromic of chronic disease
• microcytic - chronic blood loss
• haemolytic anaemia - leukaemia

leucocytosis - from cytokine release

eosinophilia - hodgekins

prothrombotic tendency - trousseau’s

Question 23

what are the GI manifestations of paraneoplastic sydnromeS?

Answer 23

weight loss
anorexia
nausea
fever and night sweats

due to cytokine release by host response to cancer

chemotherapy adds to this.

Question 24

how is the kidney involved as a paraneoplastic syndrome?

Answer 24

immune complexes can deposit in the kidneys and cause nephritic/ nephrotic syndrome

Question 25

what is hypertrophic osteoarthropathy ?

Answer 25

periosteal bone formation
arthritis
finger clubbing

caused by squamous cell lung cancer.