Paraneoplastic syndromes

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  • Created by: MazzaW
  • Created on: 05-12-19 14:21

Pleural effusion

Sx: tachypnoea, SOB, cough

Signs: decreased chest expansion, mediastinal shift (often normal but may shift AWAY from effusion, usually apex shift rather than tracheal due to gravity), decreased vocal fremitus/resonance, dull to percussion, reduced air entry, may have bronchial breathing above air-fluid level (aegophony), whispering pectoriloquy, may have pleural rub above air-fluid level if pleura inflamed

Commonly occurs in lung cancer.

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SVC obstruction

May be due to extrinsic compression or invasion of SVC. Commonly occurs in small cell lung cancer, also in lymphoma/other lung Ca, can be non-neoplastic.

Sx: facial oedema, head fullness, raised non-pulsatile JVP, distension of chest wall veins (in SVC distribution), arm oedema, facial plethora, chest pain, cough. Sx may be exacerbated on bending forward/lying down. May be quick or gradual onset.

CXR signs: may be normal, superior mediastinal widening, pleural effusion, right-sided hilar mass, anterior mediastinal mass, cardiomegaly, bilateral diffuse infiltrates, calcified paratracheal nodes.

Rx: treat underlying disease (chemo)

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Hypercalcaemia

Most common electrolyte disturbance of malignancy. Often due to bony mets. Only a true paraneoplastic syndrome if related to PTHrP.

Sx: nausea, confusion, constipation, polyuria, polydipsia, dehydration, arrhythmias

PTHrP 100x more potent than PTH. Mostly produced by squamous cell lung Ca. Others include squamous cell renal/head+neck/oesophageal. Rarer in breast Ca (usually due to bony mets).

MUST DO serum corrected calcium if suspect this- metabolic emergency.

Ix: serum corrected calcium > 2.7, decreased serum chloride, hypercalciuria, decreased PTH, increased urinary phosphate

Rx: IV saline 1st, then IV bisphosphonates

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SIADH

Most common endocrine paraneoplastic syndrome. Usually from small cell lung cancer (also pancreatic Ca, prostate Ca, non-Hodgkin's lymphoma, CNS disease, pulmonary disease, some drugs)

Sx: often asymptomatic but may have CNS toxicity (headaches, fatigue, altered mental state, confusion, seizures).

Ix: exclude non-neoplastic causes, hyponatraemia, normal serum albumin + glucose, serum hypo-osmolality, urin osmolarity > serum osmolarity, high urinary sodium, non-suppressed ADH

Rx: fluid restrict, democlocycline for other Sx

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Cushing's syndrome

Ectopic ACTH production, usually from SCLC (50%), also from NSCLC, pancreatic Ca, thymic Ca, carcinoid tumours, phaeochromocytoma, medullary thyroid Ca

Sx: wt gain, central obesity, easy bruising, hirsutism, striae, impaired glucose metabolism, muscle wasting, osteoporosis, amenorrhoea, fatigue, weakness, hypokalaemia, metabolic alkalosis

Presentation: rapid onset, weakness secondary to proximal myopathy, hyperpigmentation, metabolic disturbances

Dx: clinical features, high serum/urinary cortisol, high ACTH, no response to high dose dexamethasone suppression test

Rx: specific anti-tumour Rx, if required decrease cortisol secretion (bilateral adrenalectomy, octreotide, ketoconazole, aminogluthethamide)

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Gonadotrophin secretion

Occurs in pituitary tumours, gestational trophoblastic tumours, germ cell tumours, hepatoblastomas, lung Ca

Ix: elevated beta-hCG (urine test as this is faster), gynaecomastia, testicular exam (especially in young patients), CXR/CT chest

Beta-hCG is secreted by lung tumours, GI tract tumours, adrenal tumours, genitourinary tumours and hepatoblastoma. Levels usually correlate with clinical signs

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Encephalomyopathies

Commonly in SCLC, also breast and ovarian Ca and non-Hodgkin's lymphoma

Perivascular inflammation and neuronal degeneration, may affect limbic system/brainstem/spinal cord. Loss of neurons in amygdala + hippocampus, insular cortex gliosis, lymphocyte cuffing of blood cells, microglial nodules.

Sx: slow subacute onset, progressive, loss of short term memory, hallucinations, seizures, personality changes

Ix: CSF (increased protein, IgG and lymphocytes- pleocytosis), anti-Hu Ab, MRI changes

Rx: anti-tumour therapy

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LEMS

Lambert-Eaton myasthenic syndrome.

Ab-mediated, usually with underlying malignancy (60% cases). Mostly in SCLC, also breast/thymic/GI tract Ca.

Sx: proximal muscle weakness, 30% have dysphagia, Sx improve briefly on starting exercise then get worse again

Dx: EMG

Rx: steroids, treat Ca, plasma exchange (generally effective)

Cholinesterase inhibitors usually ineffective (constrast with myasthenia gravis)

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