Paraneoplastic Syndromes: Endocrinologic/Hematologic Flashcards
(108 cards)
1
Q
rapid correction of severe hypercalcemia
A
Calcitonin (2–8 U/kg SC every 6–12 h)
2
Q
treatment of symptomatic eosinophilia
A
steroids
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%)
3
Q
oncogenic osteomalacia is caused by benign tumors located at
A
extremities and head
5
Q
Most common paraneoplastic endocrine syndromes
A
hypercalcemia from overproduction of PTHrP
hyponatremia from excess vasopressin
Cushing’s syndrome from ectopic ACTH
5
Q
lab values in oncogenic osteomalacia
A
Low serum phosphate level
normal PTH
Normal serum calcium
low 1,25-dihydroxyvitamin D
6
Q
The transcription factor expressed at abnormally high levels in SCLC associated with ectopic ACTH
A
hASH 1
human achaete-scute homologue 1
6
Q
Growth hormone producing tumors
A
lung
pancreatic islet
6
Q
Testing for PE in cancer pts
A
CXR
ECG
ABG
Ventilation perfusion scan
7
Q
D dimer in diagnosis of paraneoplastic DVT
A
Not very predictive
elevations seen in pts older than 65 years without DVT
8
Q
Causes of tumor induced hypoglycemia in HCC
A
Reduced gluconeogenesis due to large mass
IGF2
9
Q
Tumors causing hyperthyroidism
A
Hydatidiform mole, embryonal tumors, struma ovarii
9
Q
Clinical manifestations of ectopic ACTH production
A
Easy bruising,skin fragility
hyperpigmentation
steroid psychosis
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%
10
Q
CRH producing tumors
A
Pancreatic islet, carcinoid, lung, prostate
10
Q
Tumor induced SIADH is aggravated by
A
free water intake oral or iv
11
Q
Tumors causing oncogenic osteomalacia
A
Hemangiopericytomas, osteoblastomas, fibromas, sarcomas, giant cell tumors, prostate, lung
12
Q
Cause of tumor induced eosinophilia
A
IL-5
13
Q
Prognosis in the presence of paraneoplastic thrombocytosis
A
poor
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
14
Q
Ectopic ACTH secretion accounts for _______ % of cushing syndrome
A
10-20%
14
Q
hereditary X-linked hypophosphatemia is due to
A
inactivating mutations in the PHEX gene
14
Q
When should you look for cancer in DVT pts?
A
Refractory clots
Unusal sites
Migratory or recurrent thrombophlebitis
15
Q
granulocytosis
A
Granulocyte count> 8000/µL
16
Solid tumors producing thrombocytosis
Lung cancer
Gastrointestinal cancer
Breast cancer
Ovarian cancer
Lymphoma
18
Tumors producing PGE2
Renal
lung
19
Rx of ectopic cushing
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)
19
# Jeopardy
IL-5
Cause of tumor induced eosinophilia
19
Percentage of pts with RCC,HCC and cerebellar hemangioblastoma having erythrocytosis
RCC 3%
HCC 10%
Cerebellar hemangioblastoma 15%
20
bisphosphonates can reduce calcium within
1-2 days
21
Tumors producing PTH
Lung
Ovary
21
protein causing thrombocytosis in solid malignancies
IL-6
21
Cancers associated with thrombophlebitis
Lung cancer
Pancreatic cancer
Gastrointestinal cancer
Breast cancer
Genitourinary cancer
Ovarian cancer
Prostate cancer
Lymphoma
24
Tumors producing Insulin like growth factor-2
Mesenchymal tumors, sarcomas, adrenal, hepatic, gastrointestinal, kidney, prostate
26
Tumors associated with excess PTHrP
Squamous cell (head and neck, lung, skin), breast, genitourinary, gastrointestinal
26
hCG alpha production is high in which cancers
Lung cancer
pancreatic islet cancer
27
Tumors producing eosinophilia
Lymphoma
Leukemia
Lung cancer
28
Dose of oral phosphorus
neutra phos 250mg tds
29
Cause for severe hypokalemia in ectopic ACTH secretion
excess minerelocorticoids due to ACTH stimulation of adrenal gland
high cortisol levels overwhelm 11ß -hydroxysteroid dehydrogenase type II enzyme
30
Phex gene codes for
protease that inactivates FGF23
31
Incidence of SIADH in SCLC
50% of SCLC patients
32
diagnosis of tumor induced hypoglycemia
Low serum glucose levels
low insulin
symptoms of hypoglycemia
32
Symptoms with severe eosinophilia(\>5000/µL)
shortness of breath
wheezing
33
Difference btw ectopic ACTH secretion and pitutary ACTH
Ectopic ACTH doesnot respond to high dose dexamethasone suppression test
34
Trousseau's syndrome
peripheral venous thrombosis with visceral carcinoma
35
TIO
Tumor induced osteomalacia
37
Diff btw HHM and Primary hyperparathyroidism
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
Difference in clinical manifestation between ectopic ACTH and other causes of cushing syndrome
Ectopic ACTH:
Less weight gain and centripetal fat distribution
38
How long should warfarin be given for cancer pts with DVT
3-6 months
40
Humoral hypercalcemia of malignancy occurs in ________ % of cancer patients
41
Diagnosis of ectopic ACTH secretion
Urine free cortisol levels
plasma ACTH \>100pg/ml
41
# Jeopardy
IL-6
protein causing thrombocytosis in solid malignancies
42
Rx of proximal DVT in cancer pts with relative contraindication to anticoagulation
Placement of IVC filters(greenfield)
44
Tumors producing calcitonin
Lung, colon, breast, medullary thyroid carcinoma
45
Definitive test to differentiate pitutary ACTH excess with ectopic ACTH excess
Petrosal vein sampling after CRH stimulation
ratio \>3:1 between Petrosal and peripheral ACTH is suggestive of pitutary source
46
Ectopic hormones causing diarrhea
calcitonin
VIP
47
Tumor producing excess 1,25 dihydroxy VitD
Lymphomas
48
Relative contraindication to heparin anticoagulation for DVT in cancer patients
hemorrhagic brain metastases
pericardial effusion
49
Defence of body to tumor induced SIADH
decreased thirst
50
Dose of steroids in HHM
40-100mg Po QID
51
Steroids are useful for treatment of hypercalcemia due to
Lymphoma
leukemia
multiple myeloma
52
# Jeopardy
inactivating mutations in the PHEX gene
hereditary X-linked hypophosphatemia is due to
53
Why weight gain and fat redistribution is less in ectopic ACTH production?
exposed to steroids for short time
cancer induced cachexia and weight loss
54
Cancers causing granulocytosis as paraneoplastic syndrome
Lung cancer
Gastrointestinal cancer
Ovarian cancer
Genitourinary cancer
Hodgkin's disease
56
Treatment of tumor induced hypoglycemia
Treatment of underlying malignancy
frequent meals
IV glucose during fasting and sleep
glucagon
glucocorticoids
57
Paraneoplastic syndrome
Disorders that accompany benign or malignant tumors but are not directly related to mass effects or invasion
58
# Jeopardy
protease that inactivates FGF23
Phex gene codes for
59
Indication for heparin prophylaxis in cancer pts
Those who undergo major surgery
60
Proteins that sequester IGF2
ALS(acid labile subunit)
IGF binding protein 3
61
Ectopic hormones causing cushing syndrome
ACTH
CRH
GIP(gastric inhibitory peptide)
LH
hCG
Last three are associated with macronodular adrenal hyperplasia
62
Serum IGF2 levels in tumor induced hypoglycemia
May not be increased
As hypoglycemia is due to release of hormone from binding proteins
63
Women with hCG producing tumors
asymptomatic
64
Factors causing granulocytosis associated with solid tumors
G CSF
GM CSF
IL-6
66
ACTH producing tumors
Lung (small cell, bronchial carcinoid, adenocarcinoma, squamous), thymus, pancreatic islet, medullary thyroid carcinoma
67
Features that favor HHM, as opposed to primary hyperparathyroidism
Known malignancy, recent onset of hypercalcemia, and very high serum calcium levels
68
tumors producing VIP
Pancreas, pheochromocytoma, esophagus
69
action of FGF23
inhibits renal tubular reabsorption of phosphate
inhibits renal conversion of 25-hydroxyvitamin D to 1,25-dihydroxyvitamin D
70
Vassopressin secretion due to adaptive circulatory mechanisms is seen in
HF
cirrhosis
hypotension
71
Ectopic sources of ACTH which respond to high dose dexamethasone test
bronchial and other carcinoids
Also show ACTH responsiveness to adrenal blockade with metyrapone
72
Dose of warfarin prophylaxis in cancer pts
1 mg/day
73
The prevalence of thrombocytosis in different cancers
Lung and GIT 40%
20% of patients with breast, endometrial, and ovarian cancers
Lymphoma 10%
75
Renin secreting tumors
Hypertension
Juxtaglomerular tumors, kidney, lung, pancreas, ovary
76
\_\_\_\_\_\_\_\_ % of pts with thrombocytosis have an underlying diagnosis of cancer
35
78
Cause of gynacomastia in hCG secreting tumors
high hCG►increased steroidogenesis and aromatase activity in leydig cells of testis►increased estrogen
79
warfarin prophylaxis in cancer patients
Breast cancer patients undergoing chemotherapy
Pts with implanted catheters
80
thrombocytosis
Platelet count\> 4,00,000/µL
81
Rx of DVT or PE in cancer pts
UFH/LMWH for 5 days
Warfarin started within 1 or 2 days
Maintain INR btw 2-3
83
GHRH producing tumors
Pancreatic islet, bronchial and other carcinoids
85
Tumors causing SIADH
Lung (squamous, small cell), gastrointestinal, genitourinary, ovary
86
Precocious puberty in boys or gynacomastia in men
measurement of hCG
consideration of a testicular tumor or another source of ectopic hCG production
88
Clinical features of oncogenic osteomalacia
Muscle weakness
bone pain
osteomalacia
89
Factor responsible for oncogenic osteomalacia
Phosphatonin [fibroblast growth factor 23 (FGF23)]
90
treatment of oncogenic osteomalacia
removal of tumor
supplementation with phosphate and vitamin D
91
Ectopic factors responsible for hypercalcemia of malignancy
Parathyroid hormone-related protein (PTHrP)
1,25 dihydroxyvitamin D
Parathyroid hormone (PTH)
Prostaglandin E2 (PGE2)
last two are rare causes
92
target Phosphorus levels in HHM
\>3 mg/dl
93
Metabolic manifestations of ectopic ACTH production
fluid retention
hypertension
hypokalemia
metabolic alkalosis
glucose intolerance
95
Rx of SIADH
Water restriction
Vaptans: conivaptan
oral: 20-120mg bd
IV: 10-40mg
Demeclocycline :
150–300 mg orally three to four times daily
96
Granulocytosis is more likely to be seen in ________ stages of disease
Advanced
97
High dose dexamethasone suppression test
8mg of dexamethasone PO supresses 8am serum cortisol(
98
Tests to diagnose DVT
Impedence plethysmography
b/l compression USG of leg veins
99
Role of octreotide in oncogenic osteomalacia
Reduces phosphate wasting in some patients with tumors that express somatostatin receptor subtype 2
101
Tumor producing insulin
Cervix (small cell carcinoma)
102
What to do in equivocal V/P scans in suspected PE pts?
USG to screen for DVT
If present,start anticoagulation
If absent,do pulmonary angiogram
103
# Jeopardy
measurement of hCG
consideration of a testicular tumor or another source of ectopic hCG production
Precocious puberty in boys or gynacomastia in men
104
Oncogenic osteomalacia is similar to
hereditary X-linked hypophosphatemia
105
\_\_\_\_\_\_\_ % of pts with APLAS have cancers
20%
106
Tumors causing male feminisation
tumors secreting hCG
Testis (embryonal, seminomas), germinomas, choriocarcinoma, lung, hepatic, pancreatic islet
107
Nonparaneoplastic causes of granulocytosis
Infection
tumor necrosis
Glucocorticoid administration
