Paraneoplastic Syndromes: Endocrinologic/Hematologic Flashcards

1
Q

rapid correction of severe hypercalcemia

A

Calcitonin (2–8 U/kg SC every 6–12 h)

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2
Q

treatment of symptomatic eosinophilia

A

steroids

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3
Q

Causes of ectopic ACTH secretion with percentage

A

SCLC(>50%)

thymic carcinoid(15%)

islet cell tumors(10%)

bronchial carcinoid(10%)

other carcinoids(5%)

pheochromocytoma(2%)

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3
Q

oncogenic osteomalacia is caused by benign tumors located at

A

extremities and head

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5
Q

Most common paraneoplastic endocrine syndromes

A

hypercalcemia from overproduction of PTHrP

hyponatremia from excess vasopressin

Cushing’s syndrome from ectopic ACTH

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5
Q

lab values in oncogenic osteomalacia

A

Low serum phosphate level

normal PTH

Normal serum calcium

low 1,25-dihydroxyvitamin D

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6
Q

The transcription factor expressed at abnormally high levels in SCLC associated with ectopic ACTH

A

hASH 1

human achaete-scute homologue 1

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6
Q

Growth hormone producing tumors

A

lung

pancreatic islet

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6
Q

Testing for PE in cancer pts

A

CXR

ECG

ABG

Ventilation perfusion scan

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7
Q

D dimer in diagnosis of paraneoplastic DVT

A

Not very predictive

elevations seen in pts older than 65 years without DVT

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8
Q

Causes of tumor induced hypoglycemia in HCC

A

Reduced gluconeogenesis due to large mass

IGF2

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9
Q

Tumors causing hyperthyroidism

A

Hydatidiform mole, embryonal tumors, struma ovarii

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9
Q

Clinical manifestations of ectopic ACTH production

A

Easy bruising,skin fragility

hyperpigmentation

steroid psychosis

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9
Q

prevalence of granulocytosis in different cancers

A

Lung 40%

GIT 40%

Breast cancer 20%

brain tumors 30%

ovarian tumors 30%

hodgkin 20%

RCC 10%

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10
Q

CRH producing tumors

A

Pancreatic islet, carcinoid, lung, prostate

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10
Q

Tumor induced SIADH is aggravated by

A

free water intake oral or iv

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11
Q

Tumors causing oncogenic osteomalacia

A

Hemangiopericytomas, osteoblastomas, fibromas, sarcomas, giant cell tumors, prostate, lung

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12
Q

Cause of tumor induced eosinophilia

A

IL-5

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13
Q

Prognosis in the presence of paraneoplastic thrombocytosis

A

poor

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14
Q

Dose of bisphosphonates used for treatment of hypercalcemia

A

Pamidronate 60-90mg iv

Zoledronate 4-8mg iv

etidronate 7.5 mg/kg per day PO for 3–7 consecutive days

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14
Q

Ectopic ACTH secretion accounts for _______ % of cushing syndrome

A

10-20%

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14
Q

hereditary X-linked hypophosphatemia is due to

A

inactivating mutations in the PHEX gene

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14
Q

When should you look for cancer in DVT pts?

A

Refractory clots

Unusal sites

Migratory or recurrent thrombophlebitis

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15
Q

granulocytosis

A

Granulocyte count> 8000/µL

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16
Q

Solid tumors producing thrombocytosis

A

Lung cancer

Gastrointestinal cancer

Breast cancer

Ovarian cancer

Lymphoma

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18
Q

Tumors producing PGE2

A

Renal

lung

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19
Q

Rx of ectopic cushing

A

Treatment of primary tumor(rarely sufficient)

Unresectable tumor with good prognosis(carcinoid): adrenalectomy

Medical therapy:

Ketoconazole(300–600 mg PO bid)

metyrapone(250–500 mg PO every 6 h)

Mitotane(3–6 g PO in four divided doses)

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19
Q

Jeopardy

IL-5

A

Cause of tumor induced eosinophilia

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19
Q

Percentage of pts with RCC,HCC and cerebellar hemangioblastoma having erythrocytosis

A

RCC 3%

HCC 10%

Cerebellar hemangioblastoma 15%

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20
Q

bisphosphonates can reduce calcium within

A

1-2 days

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21
Q

Tumors producing PTH

A

Lung

Ovary

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21
Q

protein causing thrombocytosis in solid malignancies

A

IL-6

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21
Q

Cancers associated with thrombophlebitis

A

Lung cancer

Pancreatic cancer

Gastrointestinal cancer

Breast cancer

Genitourinary cancer

Ovarian cancer

Prostate cancer

Lymphoma

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24
Q

Tumors producing Insulin like growth factor-2

A

Mesenchymal tumors, sarcomas, adrenal, hepatic, gastrointestinal, kidney, prostate

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26
Q

Tumors associated with excess PTHrP

A

Squamous cell (head and neck, lung, skin), breast, genitourinary, gastrointestinal

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26
Q

hCG alpha production is high in which cancers

A

Lung cancer

pancreatic islet cancer

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27
Q

Tumors producing eosinophilia

A

Lymphoma

Leukemia

Lung cancer

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28
Q

Dose of oral phosphorus

A

neutra phos 250mg tds

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29
Q

Cause for severe hypokalemia in ectopic ACTH secretion

A

excess minerelocorticoids due to ACTH stimulation of adrenal gland

high cortisol levels overwhelm 11ß -hydroxysteroid dehydrogenase type II enzyme

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30
Q

Phex gene codes for

A

protease that inactivates FGF23

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31
Q

Incidence of SIADH in SCLC

A

50% of SCLC patients

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32
Q

diagnosis of tumor induced hypoglycemia

A

Low serum glucose levels

low insulin

symptoms of hypoglycemia

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32
Q

Symptoms with severe eosinophilia(>5000/µL)

A

shortness of breath

wheezing

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33
Q

Difference btw ectopic ACTH secretion and pitutary ACTH

A

Ectopic ACTH doesnot respond to high dose dexamethasone suppression test

34
Q

Trousseau’s syndrome

A

peripheral venous thrombosis with visceral carcinoma

35
Q

TIO

A

Tumor induced osteomalacia

37
Q

Diff btw HHM and Primary hyperparathyroidism

A

PTH level

HHM: Metabolic alkalosis

Hyperparathyroidism: Hyperchloremic acidosis

PTHrP leads to a drop in urinary bicarbonate excretion rather than the increase in urinary bicarbonate produced by PTH

38
Q

Difference in clinical manifestation between ectopic ACTH and other causes of cushing syndrome

A

Ectopic ACTH:

Less weight gain and centripetal fat distribution

38
Q

How long should warfarin be given for cancer pts with DVT

A

3-6 months

40
Q

Humoral hypercalcemia of malignancy occurs in ________ % of cancer patients

A
41
Q

Diagnosis of ectopic ACTH secretion

A

Urine free cortisol levels

plasma ACTH >100pg/ml

41
Q

Jeopardy

IL-6

A

protein causing thrombocytosis in solid malignancies

42
Q

Rx of proximal DVT in cancer pts with relative contraindication to anticoagulation

A

Placement of IVC filters(greenfield)

44
Q

Tumors producing calcitonin

A

Lung, colon, breast, medullary thyroid carcinoma

45
Q

Definitive test to differentiate pitutary ACTH excess with ectopic ACTH excess

A

Petrosal vein sampling after CRH stimulation

ratio >3:1 between Petrosal and peripheral ACTH is suggestive of pitutary source

46
Q

Ectopic hormones causing diarrhea

A

calcitonin

VIP

47
Q

Tumor producing excess 1,25 dihydroxy VitD

A

Lymphomas

48
Q

Relative contraindication to heparin anticoagulation for DVT in cancer patients

A

hemorrhagic brain metastases

pericardial effusion

49
Q

Defence of body to tumor induced SIADH

A

decreased thirst

50
Q

Dose of steroids in HHM

A

40-100mg Po QID

51
Q

Steroids are useful for treatment of hypercalcemia due to

A

Lymphoma

leukemia

multiple myeloma

52
Q

Jeopardy

inactivating mutations in the PHEX gene

A

hereditary X-linked hypophosphatemia is due to

53
Q

Why weight gain and fat redistribution is less in ectopic ACTH production?

A

exposed to steroids for short time

cancer induced cachexia and weight loss

54
Q

Cancers causing granulocytosis as paraneoplastic syndrome

A

Lung cancer

Gastrointestinal cancer

Ovarian cancer

Genitourinary cancer

Hodgkin’s disease

56
Q

Treatment of tumor induced hypoglycemia

A

Treatment of underlying malignancy

frequent meals

IV glucose during fasting and sleep

glucagon

glucocorticoids

57
Q

Paraneoplastic syndrome

A

Disorders that accompany benign or malignant tumors but are not directly related to mass effects or invasion

58
Q

Jeopardy

protease that inactivates FGF23

A

Phex gene codes for

59
Q

Indication for heparin prophylaxis in cancer pts

A

Those who undergo major surgery

60
Q

Proteins that sequester IGF2

A

ALS(acid labile subunit)

IGF binding protein 3

61
Q

Ectopic hormones causing cushing syndrome

A

ACTH

CRH

GIP(gastric inhibitory peptide)

LH

hCG

Last three are associated with macronodular adrenal hyperplasia

62
Q

Serum IGF2 levels in tumor induced hypoglycemia

A

May not be increased

As hypoglycemia is due to release of hormone from binding proteins

63
Q

Women with hCG producing tumors

A

asymptomatic

64
Q

Factors causing granulocytosis associated with solid tumors

A

G CSF

GM CSF

IL-6

66
Q

ACTH producing tumors

A

Lung (small cell, bronchial carcinoid, adenocarcinoma, squamous), thymus, pancreatic islet, medullary thyroid carcinoma

67
Q

Features that favor HHM, as opposed to primary hyperparathyroidism

A

Known malignancy, recent onset of hypercalcemia, and very high serum calcium levels

68
Q

tumors producing VIP

A

Pancreas, pheochromocytoma, esophagus

69
Q

action of FGF23

A

inhibits renal tubular reabsorption of phosphate

inhibits renal conversion of 25-hydroxyvitamin D to 1,25-dihydroxyvitamin D

70
Q

Vassopressin secretion due to adaptive circulatory mechanisms is seen in

A

HF

cirrhosis

hypotension

71
Q

Ectopic sources of ACTH which respond to high dose dexamethasone test

A

bronchial and other carcinoids

Also show ACTH responsiveness to adrenal blockade with metyrapone

72
Q

Dose of warfarin prophylaxis in cancer pts

A

1 mg/day

73
Q

The prevalence of thrombocytosis in different cancers

A

Lung and GIT 40%

20% of patients with breast, endometrial, and ovarian cancers

Lymphoma 10%

75
Q

Renin secreting tumors

A

Hypertension

Juxtaglomerular tumors, kidney, lung, pancreas, ovary

76
Q

________ % of pts with thrombocytosis have an underlying diagnosis of cancer

A

35

78
Q

Cause of gynacomastia in hCG secreting tumors

A

high hCG►increased steroidogenesis and aromatase activity in leydig cells of testis►increased estrogen

79
Q

warfarin prophylaxis in cancer patients

A

Breast cancer patients undergoing chemotherapy

Pts with implanted catheters

80
Q

thrombocytosis

A

Platelet count> 4,00,000/µL

81
Q

Rx of DVT or PE in cancer pts

A

UFH/LMWH for 5 days

Warfarin started within 1 or 2 days

Maintain INR btw 2-3

83
Q

GHRH producing tumors

A

Pancreatic islet, bronchial and other carcinoids

85
Q

Tumors causing SIADH

A

Lung (squamous, small cell), gastrointestinal, genitourinary, ovary

86
Q

Precocious puberty in boys or gynacomastia in men

A

measurement of hCG

consideration of a testicular tumor or another source of ectopic hCG production

88
Q

Clinical features of oncogenic osteomalacia

A

Muscle weakness

bone pain

osteomalacia

89
Q

Factor responsible for oncogenic osteomalacia

A

Phosphatonin [fibroblast growth factor 23 (FGF23)]

90
Q

treatment of oncogenic osteomalacia

A

removal of tumor

supplementation with phosphate and vitamin D

91
Q

Ectopic factors responsible for hypercalcemia of malignancy

A

Parathyroid hormone-related protein (PTHrP)

1,25 dihydroxyvitamin D

Parathyroid hormone (PTH)

Prostaglandin E2 (PGE2)

last two are rare causes

92
Q

target Phosphorus levels in HHM

A

>3 mg/dl

93
Q

Metabolic manifestations of ectopic ACTH production

A

fluid retention

hypertension

hypokalemia

metabolic alkalosis

glucose intolerance

95
Q

Rx of SIADH

A

Water restriction

Vaptans: conivaptan

oral: 20-120mg bd

IV: 10-40mg

Demeclocycline :

150–300 mg orally three to four times daily

96
Q

Granulocytosis is more likely to be seen in ________ stages of disease

A

Advanced

97
Q

High dose dexamethasone suppression test

A

8mg of dexamethasone PO supresses 8am serum cortisol(

98
Q

Tests to diagnose DVT

A

Impedence plethysmography

b/l compression USG of leg veins

99
Q

Role of octreotide in oncogenic osteomalacia

A

Reduces phosphate wasting in some patients with tumors that express somatostatin receptor subtype 2

101
Q

Tumor producing insulin

A

Cervix (small cell carcinoma)

102
Q

What to do in equivocal V/P scans in suspected PE pts?

A

USG to screen for DVT

If present,start anticoagulation

If absent,do pulmonary angiogram

103
Q

Jeopardy

measurement of hCG

consideration of a testicular tumor or another source of ectopic hCG production

A

Precocious puberty in boys or gynacomastia in men

104
Q

Oncogenic osteomalacia is similar to

A

hereditary X-linked hypophosphatemia

105
Q

_______ % of pts with APLAS have cancers

A

20%

106
Q

Tumors causing male feminisation

A

tumors secreting hCG

Testis (embryonal, seminomas), germinomas, choriocarcinoma, lung, hepatic, pancreatic islet

107
Q

Nonparaneoplastic causes of granulocytosis

A

Infection

tumor necrosis

Glucocorticoid administration