Paraneoplastic Syndromes

Question 1

Several neoplasms, including ______, are associated with a paraneoplastic syndrome caused by ____ ____ ___, resulting in hypercalcemia and a decreased endogenous parathyroid hormone (PTH) level.

Answer 1

Several neoplasms, including NSCLC, are associated with a paraneoplastic syndrome caused by ectopic secretion of parathyroid hormone–related peptide (PTHrP), resulting in hypercalcemia and a decreased endogenous parathyroid hormone (PTH) level.

Question 2

A compression of the cervical sympathetic chain due to a Pancoast tumor causes ___ ____, which is characterized by __ ___ ___.

Answer 2

A compression of the cervical sympathetic chain due to a Pancoast tumor causes Horner syndrome, which is characterized by ptosis, miosis, and anhidrosis.

Question 3

A compression of the cervical sympathetic chain due to a ___ ____ causes Horner syndrome, which is characterized by ___ ____ ____.

Answer 3

A compression of the cervical sympathetic chain due to a Pancoast tumor causes Horner syndrome, which is characterized by ptosis, miosis, and anhidrosis.

Question 4

Define

Pancoast Tumor

Answer 4

A Pancoast tumor is a tumor of the apex of the lung.

It is a type of lung cancer defined primarily by its location situated at the top end of either the right or left lung. It typically spreads to nearby tissues such as the ribs and vertebrae. Most Pancoast tumors are non-small-cell lung cancers.

The growing tumor can cause compression of a brachiocephalic vein, subclavian artery, phrenic nerve, recurrent laryngeal nerve, vagus nerve, or, characteristically, compression of a sympathetic ganglion (the stellate ganglion), resulting in a range of symptoms known as Horner’s syndrome.

Question 5

Horner’s syndrome, also known as _____ is a combination of symptoms that arises when a group of nerves known as the___ ____ is damaged. The signs and symptoms occur on the______ it is a lesion of the ____ trunk. It is characterized by (name 4)

Answer 5

Horner’s syndrome, also known as oculosympathetic paresis, is a combination of symptoms that arises when a group of nerves known as the sympathetic trunk is damaged. The signs and symptoms occur on the same side (ipsilateral) as it is a lesion of the sympathetic trunk. It is characterized by miosis (a constricted pupil), partial ptosis (a weak, droopy eyelid), apparent anhydrosis (decreased sweating), with apparent enophthalmos (inset eyeball)

Question 6

Long-term exposure to silica results in

What condition and characterized by what?

Answer 6

Long-term exposure to silica results in silicosis, a restrictive lung disease,

characterized: by dyspnea, cough, fatigue, tachypnea, and chest pain.

Question 7

Renal resistance to ADH causes WHAT?

Answer 7

Renal resistance to ADH causes:

nephrogenic diabetes insipidus, which can cause polyuria and hypernatremia.

Question 8

Ectopic ADH production is also known as _______ and can occur in ____ and causes WHAT?

Answer 8

Ectopic ADH production is also known as syndrome of inappropriate secretion of ADH and can occur in SCLC and causes water retention (oliguria), hyponatremia, and fatigue.

Question 9

A solitary parathyroid adenoma can manifest with _____

Answer 9

A solitary parathyroid adenoma can manifest with hypercalcemia.

Question 10

Identify Condition & Cause

Answer 10

Non-Small Cell Lung Cancer

1. Ectopic secretion of parathyroid hormone–like protein
2. Neoplasms including NSCLC can cause hypercalcemia.
3. The neoplasms secrete PTHrP, which acts like PTH, leading to hypercalcemia and increased bone turnover.

Note:
This patient has hypercalcemia and centrally located mass in the lung on x-ray (red square on image). She most likely has non–small cell lung cancer (NSCLC), which may cause secondary hypercalcemia. The hypercalcemia is causing her constipation. The lung cancer diagnosis is supported by the patient’s smoking history and abnormal pulmonary exam.

Question 11

Indentify:

Answer 11

Compressionoflocoregionalstructuresmaycausearrayoffindings:

Also known as superior sulcus tumor. Carcinoma that occurs in the apex of lung may cause Pancoast syndrome byinvading/compressing local structures.

Recurrentlaryngealnerve hoarseness

Stellateganglion Horner syndrome (ipsilateralptosis,miosis,anhidrosis)

Superior vena cava SVCsyndrome

Brachiocephalic vein brachiocephalic syndrome (unilateralsymptoms)

Brachial plexus shoulder pain, sensori motor deficits (eg,atrophy of intrinsic muscles of the hand)

Phrenic nerve hemidiaphragm paralysis (hemidiaphragm elevation on CXR)

Question 12

SPHERE of complications:

Answer 12

SPHERE of complications: Superiorvenacava/thoracicoutletsyndromes,Pancoasttumor,Hornersyndrome,Endocrine(paraneoplastic),Recurrentlaryngealnervecompression(hoarseness),Effusions(pleuralorpericardial).

Question 13

Systemic lupus erythematosus is an autoimmune disease with ___, ____, and ____antibodies that is associated with (name 4)

Answer 13

Systemic lupus erythematosus is an autoimmune disease with anti-double-stranded DNA, anti-DNA, and anti-Smith antibodies that is

associated with skin rash, hair loss, fever, and anorexia.

Question 14

__ __ is an inflammatory disorder of synovial joints with __ formation that presents with (name 3 symptoms)

Answer 14

Rheumatoid arthritis is an inflammatory disorder of synovial joints with pannus formation that presents with symmetric morning stiffness, joint inflammation, and fatigue.

Question 15

Reactivation of a__ __ ___ lesion and hilar lymph nodes describes __ __, which would present with fever and cough.

Answer 15

Reactivation of a peripheral subpleural parenchymal lesion and hilar lymph nodes describes secondary tuberculosis, which would present with fever and cough.

Question 16

Autoantibodies to __ __at the neuromuscular junction are seen in __ ___, which can be associated with __ and presents with weakness that worsens with ____.

Answer 16

Autoantibodies to acetylcholine receptors at the neuromuscular junction are seen in myasthenia gravis, which can be associated with thymoma and presents with weakness that worsens with exertion.

Question 17

LES involves the production of (WHAT) at the neuromuscular junction. This reduces ___in the synaptic cleft and produces __ ___ weakness, decreased __ __ __, and ___with ptosis.

Ocular and cranial nerve involvement is much more common in __ ___, which can also present with muscle weakness and diplopia. However, ___ muscle weakness improves with muscle use, and ____ worsens with muscle use.

Answer 17

LES involves the production of antibodies against presynaptic voltage-gated (P/Q type) calcium channels at the neuromuscular junction. This reduces acetylcholine in the synaptic cleft and produces proximal muscle weakness, decreased deep tendon reflexes, and diplopia with ptosis.

Ocular and cranial nerve involvement is much more common in myasthenia gravis, which can also present with muscle weakness and diplopia. However, LES muscle weakness improves with muscle use, and myasthenia gravis worsens with muscle use.

Question 18

This patient likely has Lambert-Eaton syndrome (LES)

describe:

Answer 18

This patient likely has Lambert-Eaton syndrome (LES), a paraneoplastic syndrome, given his x-ray of the chest showing a central lung cancer, metastases, proximal muscle weakness, decreased deep tendon reflexes (that improve with activity), and diplopia.

Parasympathetic deficits, especially dry mouth and constipation, are common.

Exercise-induced facilitation of reflexes is a unique diagnostic finding for LES. Patients typically have neurologic symptoms prior to the diagnosis of lung cancer.

Small cell lung carcinoma is a neoplasm of neuroendocrine Kulchitsky cells that frequently produces paraneoplastic syndromes, including ectopic production of ACTH and ADH.

Question 19

Anisocoria

Answer 19

a condition characterized by an unequal size of the eyes’ pupils.

Question 20

Lambert-Eaton syndrome causes

Answer 20

Lambert-Eaton syndrome

causes muscle weakness in patients with small cell lung cancer. Patients have slowly progressive proximal lower extremity weakness, diminished reflexes, dry mouth, and occasionally ptosis.

The muscle weakness improves with exercise.

This patient lacks the lower extremity weakness characteristic of this disorder.

Question 21

Myasthenia gravis

Answer 21

Myasthenia gravis,

an autoimmune process involving the production of antibodies to the acetylcholine receptor, is associated with thymoma and often presents with ptosis.

Patients may also experience muscular weakness in the arms, legs, and in rare cases, muscles involved in respiration.

However, patients do not have miosis, and therefore it is unlikely to be the cause of this patient’s symptoms.

Question 22

Pancoast tumor is commonly a non–small cell lung cancer that may cause :

Answer 22

A Pancoast tumor is commonly a non–small cell lung cancer that may cause

the triad of Horner syndrome (ptosis, anhidrosis, and miosis) via disruption of the sympathetic plexus caused by the tumor’s position in the apex of the lung.

Question 23

This patient most likely has a Pancoast tumor, given her history of :

Answer 23

Pancoast tumor, given her history of fatigue and recent weight loss, ptosis, and miosis on the right side. A tumor at the apex of the lung, or superior sulcus, is known as a “Pancoast tumor.” Because of its location, it may compress the cervical sympathetic ganglia, resulting in loss of sympathetic function and causing Horner syndrome (ptosis, anhidrosis, and miosis).

It also may cause compression of the brachial plexus, spinal nerve roots, subclavian artery and/or vein, ribs, and muscles.

A Pancoast tumor is a non–small cell lung cancer seen in patients with a history of smoking.

Question 24

Bell palsy is a form of ?

Answer 24

Bell palsy is a form of facial palsy characterized by malfunction of the facial nerve (cranial nerve VII), resulting in facial droop including both the muscles of the forehead and mouth toward the affected size.

The facial nerve does not control pupil size. This is an action of cranial nerve III.

Question 25

***Myasthenia gravis*** is associated

Answer 25

***Myasthenia gravis*** is associated with thymoma and often presents with ptosis, but not miosis. Patients may also experience muscular weakness in the arms, legs, and in rare cases, muscles involved in respiration.

Question 26

*Lambert-Eaton syndrome* is a paraneoplastic syndrome that causes

Answer 26

Lambert-Eaton syndrome is a paraneoplastic syndrome that causes slowly progressive proximal lower extremity weakness that improves with exertion. Other physical findings include diminished reflexes, dry mouth, and occasionally ptosis. It is most commonly identified in patients with small cell lung cancer.

Question 27

*Superior vena cava syndrome* presents with?

Answer 27

Superior vena cava syndrome presents with facial swelling, cyanosis, and dilation of the veins of the head, neck, and upper extremities. The most common causes are due to external compression or internal obstruction of the superior vena cava.

Question 28

*Botulism is due to*

Answer 28

Botulism is due to a toxin released by ingested Clostridium botulinum and presents with bilateral cranial nerve deficits and weakness that descends from upper to lower extremities

Question 29

***Renal cell carcinoma*** often presents as a WHAT syndrome? Example and this syndrome is common with WHAT?

Answer 29

Renal cell carcinoma often *presents as a paraneoplastic syndrome, eg, polycythemia, hypercalcemia, or fever.* *Paraneoplastic syndromes* are common with ***renal cell carcinoma***, *causing diverse complications* such as ***anemia, polycythemia, fevers, hypercalcemia, elevated hepatic alkaline phosphatase, or secondary amyloidosis.***

Question 30

The patient has weight loss, fevers, and a large right renal mass on CT (see image), all suggesting ___ \_\_\_ \_\_\_, which often presents with advanced disease. The classic triad of (name 3) can be present, but is seen in \<10% of cases. Patients more often present with constitutional or __ \_\_, and most are diagnosed when a CT scan shows __ \_\_\_.

Answer 30

The patient has weight loss, fevers, and a large right renal mass on CT (see image), all suggesting renal cell carcinoma, which often presents with advanced disease. The *classic triad* of ***hematuria, flank mass, and flank pain*** can be present, but is seen in \<10% of cases. Patients more often present with constitutional or ***nonrenal symptoms***, and most are diagnosed when a CT scan shows ***renal abnormalities.***

Question 31

Renal cell carcinoma commonly shows __ \_\_ findings, often due to secretion of __ \_\_. This most commonly causes __ in up to 80% of patients (due to __ \_\_\_ \_\_\_), but may also cause ___ (due to erythropoietin secretion), fevers, \_\_\_, elevated hepatic alkaline phosphatase, or ectopic\_\_ ___ (with Cushing syndrome), warranting the nickname “internist’s tumor.”

Answer 31

Renal cell carcinoma commonly shows ***paraneoplastic syndrome*** findings, often due to secretion of ***ectopic hormones.*** This most commonly causes ***anemia*** in up to 80% of patients (due to ***anemia of chronic disease***), but may also cause ***polycythemia*** (due to erythropoietin secretion), fevers, ***hypercalcemia***, elevated hepatic alkaline phosphatase, or ectopic ***ACTH secretion*** (with Cushing syndrome), warranting the nickname “internist’s tumor.”

Question 32

*Cerebral aneurysms and hepatic cysts* are seen in patients with

Answer 32

Cerebral aneurysms and hepatic cysts are *seen in patients with autosomal dominant polycystic kidney disease, which may present with an enlarged kidney*

Question 33

*Hypocalcemia* may be due to WHAT?

Answer 33

Hypocalcemia may be due to ***hypoparathyroidism, rickets, vitamin D deficiency, malnutrition, or drugs like loop diuretics.***

Question 34

*Dysuria* is most commonly seen in ?

Answer 34

Dysuria is most commonly seen *in lower urinary tract disorders like cystitis or urethritis.* ## Footnote ***Painful urination***

Question 35

Nephrotic-range proteinuria of ___ gm/24 hr is seen in several forms of glomerular disease such as (name 3)

Answer 35

Nephrotic-range proteinuria of ***\>3.5 gm/24 hr*** is seen in several forms of *glomerular disease* such as **diabetic nephropathy, minimal change disease,** and **membranous glomerulonephritis.**

Question 36

*Define and describe Condition*

Answer 36

***Cushing syndrome*** is caused by *high levels of circulating cortisol* and is characterized by weight gain, abdominal striae, and moon facies. Additional findings include *poor wound healing, truncal obesity, easy bruising, skin thinning, menstrual irregularities, hirsutism, proximal muscle weakness, secondary diabetes mellitus or glucose intolerance.*

Question 37

A 57-year-old woman comes to the clinic for recent weight gain. Her photos are shown.

Answer 37

This patient has likely Cushing syndrome ***(elevated cortisol) based on weight gain*** and a photo showing ***purple streaks on the abdomen (striae), moon face, and facial acne***. Cushing syndrome may be *due to adrenal disease (adenoma, hyperplasia) or ACTH secretion by a pituitary adenoma (Cushing disease) or cancer (paraneoplastic syndrome).* *The increased adrenal hormonal synthesis increases cortisol and androgen levels. There also may be fat between the shoulders (“buffalo hump”), mood changes and androgen-caused virilizing symptoms such as acne and facial hair (hirsutism) in women.*

Question 38

Additional findings in ***high cortisol states*** include ?

Answer 38

Additional findings in high cortisol states include ## Footnote *poor wound healing, truncal obesity, easy bruising, skin thinning, menstrual irregularities, proximal muscle weakness, loss of bone density, fractures, secondary diabetes mellitus, and glucose intolerance.*

Question 39

Cold intolerance is a symptom of\_\_, which also presents with (name 4)

Answer 39

Cold intolerance is a symptom of ***hypothyroidism***, which also presents with ***hair loss, weight gain, constipation, and fatigue.***

Question 40

***Hypotension*** may be seen in (name 3). It also may be due to *overmedication* with\_\_\_ drugs.

Answer 40

*Hypotension* may be seen in ***adrenal insufficiency, volume depletion, or sepsis***. It also may be due to *overmedication* with ***hypertension drugs.***

Question 41

Nipple discharge is a symptom of elevated __ levels, usually secondary to a \_\_\_, a benign tumor of the pituitary gland

Answer 41

*Nipple discharg*e is a symptom of *elevated prolactin levels*, usually ***secondary to a prolactinoma***, a ***benign tumor of the pituitary gland***

Question 42

Hypoglycemia may be seen in patients taking insulin or __ \_\_ drugs like \_\_\_, in adrenal insufficiency, and in rare insulin-secreting tumors.

Answer 42

*Hypoglycemia* may be seen in patients taking ***insulin*** or *oral glucose-lowering drugs* like ***sulfonylureas***, in ***adrenal insufficiency***, and in *rare insulin-secreting tumors.*

Question 43

***Unexplained weight loss, jaundice, and a nontender mass in the RUQ*** are characteristic of a ___ \_\_\_\_. and most commonly occurs in the\_\_ \_\_\_, which arises from the __ and ____ pancreatic buds.

Answer 43

Unexplained weight loss, jaundice, and a nontender mass in the RUQ are characteristic of a ***pancreatic adenocarcinoma***. ***Pancreatic cance***r most commonly occurs in the ***pancreatic head,*** which arises from the ***ventral*** and ***dorsal*** pancreatic buds.

Question 44

The patient’s unexplained weight loss, jaundice, and a nontender mass in the right upper quadrant (RUQ) indicate likely __ \_\_ or, more uncommonly, __ \_\_ that has metastasized to the liver (excluded here by the lack of metastasis on CT). These tumors arise most commonly in the __ \_\_. Obstructive jaundice with a palpable and nontender gallbladder is ___ sign, a sign of ___ cancer. Less common additional signs include migratory thrombophlebitis (\_\_\_\_ syndrome).

Answer 44

The patient’s unexplained weight loss, jaundice, and a nontender mass in the right upper quadrant (RUQ) indicate likely ***pancreatic adenocarcinoma*** or, more uncommonly, ***gastric carcinoma*** that has metastasized to the liver (excluded here by the lack of metastasis on CT). These tumors arise most commonly in the ***pancreatic head.*** Obstructive jaundice with a palpable and nontender gallbladder is ***Courvoisier sign,*** a sign of ***pancreatic cancer.*** Less common additional signs include migratory thrombophlebitis (***Trousseau*** syndrome).

Question 45

The hepatic diverticulum (liver bud) is derived from the embryonic __ \_\_\_ that gives rise to the \_\_\_. Hepatocellular cancer can present with weight loss, jaundice, and an RUQ mass, but the absence of underlying liver disease makes this diagnosis unlikely.

Answer 45

The hepatic diverticulum (liver bud) is derived from the embryonic ***foregut endoderm*** that gives rise to the ***parenchyma of the liver***. Hepatocellular cancer can present with weight loss, jaundice, and an RUQ mass, but the absence of underlying liver disease makes this diagnosis unlikely.

Question 46

The midgut gives rise to ? Cancer in this region would not cause jaundice, instead usually causing __ \_\_\_.

Answer 46

The *midgut gives rise* to the ***intestine from the distal duodenum to the proximal transverse colon***. Cancer in this region would not cause jaundice, instead usually causing ***heme-positive stools.***

Question 47

The *ureteric bud* gives rise to the __ \_\_\_ (except for the nephrons). Renal cell carcinoma may show (name 4)

Answer 47

The ***ureteric bud*** gives rise to the ***adult kidneys*** (except for the nephrons). Renal cell carcinoma may show ***hematuria, flank pain, anemia, and weight loss.***

Question 48

***dorsal mesentery*** is a supportive structure that holds WHAT? During development, it evolves into the ___ supporting the \_\_\_and portions of the \_\_\_. It does not give rise to gastrointestinal organs, so it would not give rise to the organs of the upper abdomen that are the likely causes of this patient’s malignancy.

Answer 48

***dorsal mesentery*** is a supportive structure that ***holds the developing foregut to the posterior abdominal wall.*** During development, ***it evolves into the mesentery supporting the stomach and portions of the colon.****_It does not give rise to gastrointestinal organs,_* so it would not give rise to the organs of the upper abdomen that are the likely causes of this patient’s malignancy.

Question 49

This patient’s laboratory findings of ***hyponatremia***, *increased urine osmolarity, and decreased serum osmolarity* are suggestive of (WHAT)? due to increased __ \_\_, that is, the syndrome __ \_\_\_ ___ secretion. A common cause of \_\_\_secretion is small cell lung cancer, here suggested by the combination of (name 3)

Answer 49

This patient’s laboratory findings of *hyponatremia, increased urine osmolarity, and decreased serum osmolarity* are suggestive of ***increased free water retention due to increased ADH secretion***, that is, the ***syndrome of inappropriate ADH (SIADH) secretion.*** A common cause of ***SIADH*** secretion is small cell lung cancer, here suggested by the combination of ***hemoptysis, dyspnea, and weight loss.***

Question 50

*Severe symptomatic hyponatremia* (\_\_, \_\_) usually occurs when the __ \_\_is below ___ mEq/L, In these cases, the best treatment is giving __ (3\_\_) normal saline. The key to correcting severe hyponatremia is giving an intravenous solution that is more concentrated (\_\_ \_\_\_) than the \_\_. Here, the urine osmolarity is 550 mOsm/L. Three percent saline has an osmolarity of 1027 mOsm/L, which will correct the hyponatremia. Do not increase the serum Na+ concentration by more than\_\_ mEq/L per hour over the first __ hours (8-12 mEq/L in first 24 hours) to avoid\_\_ ___ syndrome, which may causer ***WHAT TYPE of injury.***

Answer 50

*Severe symptomatic hyponatremia* (***confusion, seizures***) usually occurs when the ***serum Na+*** is below ***120 mEq/L***, as it is here. In these cases, the best treatment is giving ***hypertonic (3%)*** normal saline. The key to correcting severe hyponatremia is giving an intravenous solution that is more concentrated ***(higher osmolarity) than the urine.*** Here, the urine osmolarity is 550 mOsm/L. Three percent saline has an osmolarity of 1027 mOsm/L, which will correct the hyponatremia. Do not increase the serum Na+ concentration by more than ***0.5 mEq/L per hour over the first 24 hours (8-12 mEq/L in first 24 hours***) to ***avoid osmotic demyelinating syndrome***, which may cause ***irreversible central nervous system neurologic injury***.

Question 51

Isotonic, iso-osmolar solutions used for __ \_\_\_are \_\_% normal saline (osmolarity of 308 mOsm/L) and\_\_ ___ solution (osmolarity of 273 mOsm/L).

Answer 51

***Isotonic***, iso-osmolar solutions used for v***olume resuscitation are 0.9% normal saline*** (osmolarity of 308 mOsm/L) and ***lactated Ringers solution*** (osmolarity of 273 mOsm/L).

Question 52

A hypotonic solution is \_\_% \_\_\_\_saline (osmolarity 154 mOsm/L), which is used as __ \_\_ in patients who cannot take fluids orally.

Answer 52

A ***hypotonic solution is 0.45% normal saline*** (osmolarity 154 mOsm/L), which is used as ***maintenance fluid*** in patients who cannot take fluids orally.

Question 53

\_\_% \_\_solution is used when the patient needs pure water.

Answer 53

***5% dextrose solution** is used when the patient needs pure water.*

Question 54

The most common cancers that metastasize to bone are those of the (name 6) breast and prostate cancers usually cause\_\_ __ characterized by __ \_\_ formation.

Answer 54

The *most common cancers that metastasize to bone* are those of the ***breast, prostate, lung, thyroid, testes, and kidney;*** ***breast and prostate cancers*** usually cause ***blastic lesions*** characterized by ***new bone formation.***

Question 55

The x-ray image shows ? Most likely diagnosis?

Answer 55

The x-ray image shows ***several nodular, hyperdense sclerotic (blastic) lesions in the patient's spine*** (see arrows in image), typical of *metastatic cancer, especially in an older adult with concurrent weight loss.* In evaluations of bone tumors, metastases to bone are far more common than primary bone tumors. The most c***ommon cancers that metastasize to bone are cancers of the breast, lung, thyroid, testes, kidney, and prostate.*** They ***can cause lytic lesions showing bone destruction (lucent areas) in multiple myeloma as well as in lung, thyroid, and kidney cancers***. Other cancers may instead cause ***blastic lesions with new bone formation, common in prostate and breast cancer.*** Some show mixed lesions (eg, in breast cancer). ***Given the blastic lesions, the patient most likely has*** ***_prostatic adenocarcinoma._***

Question 56

\_\_ __ is a *spondylitis* that typically affects the lower thoracic and lumbar regions; it is caused by __ \_\_\_.

Answer 56

***Pott disease*** is a *spondylitis that typically affects the lower thoracic and lumbar regions;* it is caused by ***Mycobacterium tuberculosis.***

Question 57

\_\_ ___ \_\_\_ is associated with *hyperparathyroidism* and characterized by __ \_\_\_ of bone.

Answer 57

***Osteitis fibrosa cystica*** is associated with *hyperparathyroidism* and characterized by ***fibrous replacement of bone.***

Question 58

\_\_\_ is a rare bone tumor in adults, but it is the most common primary bone malignancy in __ and \_\_\_\_

Answer 58

***Osteosarcoma*** is a rare bone tumor in adults, but it is the most ***common primary bone malignancy in children and adolescents.***

Question 59

\_\_ __ \_\_\_ is characterized by *accelerated bone turnover* leading to increased __ \_\_\_ and\_\_\_, which produce distinguishing *“\_\_\_” patterns.*

Answer 59

***Paget disease of bone*** is characterized by a*ccelerated bone turnover* leading to ***increased bone formation*** and ***resorption***, which produce distinguishing ***“mosaic” patterns.***

Question 60

This patient has a *smoking history and right hilar mass with chronic cough* suggestive of __ \_\_. He also has *hematuria and flank pain suggestive* of __ \_\_. Putting these together, he likely has a ***paraneoplastic syndrome of*** a __ \_\_secreting (WHAT), leading to hypercalcemia and secondary\_\_\_. Recall that ***hypercalcemia may*** cause a \_\_,\_\_,\_\_and *psychiatric overtones*” presentation, representing (What 4 Conditions)

Answer 60

This patient has a smoking history and right hilar mass with chronic cough suggestive of ***lung cancer.*** He also has hematuria and flank pain suggestive of ***kidney stones***. Putting these together, he likely has a paraneoplastic syndrome of a l***ung cancer secreting parathyroid hormone (PTH)-related peptide (PTHrP)***, leading to hypercalcemia and ***secondary nephrolithiasis.*** Recall that hypercalcemia may cause ***a “stones, thrones, groans, and psychiatric overtones”*** presentation, representing ***renal stones, nephrogenic diabetes insipidus (polyuria), constipation, and confusion.***

Question 61

*Hypercalcemia Disease Mechanism*

Answer 61

Many malignancies cause paraneoplastic syndromes by secreting hormones like ***ADH or peptides like PTHrP***. The *latter causes* ***hypercalcemia***, *acting just like the normal PTH*. ***Hypercalcemia*** leads to ***increased urine calcium (hypercalciuria),*** which *leads to* the ***formation of calcium oxalate and phosphate stones.***

Question 62

***High vitamin D*** levels may be seen in\_\_, which can cause elevated __ \_\_\_ (by production of vitamin D), \_\_,\_\_ and *dyspnea*. However, it is most commonly seen in __ \_\_ \_\_, and there is often\_\_ __ \_\_ with coarse reticular opacities on chest x-ray. In ***sarcoidosis*** the levels of \_\_-\_\_ ___ increase

Answer 62

*High vitamin D levels* may be seen in ***sarcoidosis,*** which can cause ***elevated serum calcium*** (by production of vitamin D), ***kidney stones, cough, and dyspnea.*** However, it is most commonly seen in ***young Black women***, and there is often ***bilateral hilar adenopathy*** with coarse reticular opacities on chest x-ray. In sarcoidosis the levels of ***angiotensin-converting enzyme increase***

Question 63

(Pulse is *72/min and blood pressure is 130/78 mm Hg* without orthostatic changes. Laboratory tests show a *serum sodium level of 116 mEq/L and urine osmolality of 520 mOsm/kg.* Severe ***hyponatremia*** (\_\_ mEq/L) may present with ___ or an altered mental state. *SIADH* presents with ___ with elevated __ \_\_\_ yet with a normal total body volume and may commonly be due to tumors or drugs such as (Name 4 drug groups)

Answer 63

*Severe hyponatremia* (***\<120 mEq/L***) may present with ***seizures*** or an altered mental state. SIADH presents with ***hyponatremia*** with ***elevated urine osmolality*** yet with a normal total body volume and may commonly be due to tumors or drugs such as ***selective serotonin reuptake inhibitor, antidepressants, antipsychotics, and glyburide.***

Question 64

(Pulse is 72/min and blood pressure is 130/78 mm Hg without orthostatic changes. Laboratory tests show a **serum sodium level of 116 mEq/L** and ***urine osmolality of 520 mOsm/kg.)*** The patient presents with seizure, altered mental state, severe hyponatremia (\_\_\_\_ mEq/L), and concentrated urine (\_\_\_\_\_ mOsm/kg), which in the setting of a normal volume status suggests a diagnosis of ***WHAT?***

Answer 64

The patient presents with seizure, altered mental state, ***severe hyponatremia (\<120 mEq/L),*** and ***concentrated urine (\>300 mOsm/kg),*** which in the setting of a normal volume status suggests a ***diagnosis of syndrome of inappropriate ADH secretion (SIADH)***. While volume depletion and edematous states like congestive heart failure can also cause hyponatremia with concentrated urine due to high ADH levels, these causes usually lead to more modest hyponatremia (\>125 mEq/L), which do not cause the symptoms of severe hyponatremia like altered mental state (see table).

Question 65

SIADH is most often due to either abnormal __ \_\_\_from malignancy (classically __ \_\_\_ __ \_\_, but also __ or other gastrointestinal malignancies) or from the posterior pituitary gland. Abnormal high ADH release from the pituitary occurs with many drugs (Name 4 drug groups) as well as from central nervous system (CNS)/psychiatric disorders like CNS tumors, psychosis, and epilepsy. High, unregulated ADH levels cause excess free water retention by the kidney, which leads to \_\_-\_\_\_ and ___ from excess total body water. Despite the name, most hyponatremia is not due to a lack of sodium, but to an \_\_of \_\_.

Answer 65

SIADH is most often due to either abnormal ***ADH release from malignancy*** (classically ***small cell lung cancer,*** but also ***pancreatic*** or other gastrointestinal malignancies) or from the posterior pituitary gland. Abnormal high ADH release from the pituitary occurs with many drugs (s***elective serotonin reuptake inhibitor, antidepressants, antipsychotics, glyburide***) as well as from central nervous system (CNS)/psychiatric disorders like CNS tumors, psychosis, and epilepsy. High, unregulated ADH levels cause excess free water retention by the kidney, which leads to ***hypo-osmolarity*** and ***hyponatremia*** from excess total body water. Despite the name, most hyponatremia is not due to a lack of sodium, but to an ***excess of water.***

Question 66

Excessive fluid intake would show?

Answer 66

*would show low urine osmolality as the kidney excretes excess water*

Question 67

*Volume depletion* and *furosemide* would lead to ?

Answer 67

clinical signs of volume depletion. The ***urine osmolality would be high*** (ex: urine osmolality of 520 mOsm/kg.)

Question 68

*Lithium therapy* would show

Answer 68

*hypernatremia with polyuria and a very low urine osmolality (nephrogenic diabetes insipidus)*.

Question 69

*Angiotensin-converting enzyme inhibitors* are uncommon causes of \_\_\_, as they mostly affect __ \_\_ (blood pressure), not __ \_\_\_.

Answer 69

*Angiotensin-converting enzyme inhibitors* are uncommon causes of ***hyponatremia***, as they mostly affect ***vascular tone (blood pressure)***, not ***water metabolism.***

Question 70

increasing bilateral leg weakness over the past 3 weeks. After he walks for a short period, the strength seems to return to his legs. dry mouth and that he chokes when trying to drink a glass of water quickly. mild proximal muscle weakness in the legs. After being asked to walk up and down the hallway several times, the muscle strength is improved, and the reflexes are 2 to 3

Answer 70

***Small cell lung carcinoma*** ***LEMS*** is characterized by muscle weakness that diminishes with use (increased muscle strength with use). ***Small cell lung cancer*** is associated with ***LEMS*** as a paraneoplastic syndrome. The *mechanistic cause* of LEMS involves ***antibodies that inhibit the presynaptic calcium channels at the neuromuscular junction.***

Question 71

This patient presents with muscle weakness and hyporeflexia that improves with use. These symptoms are typical of \_\_\_-\_\_\_ \_\_\_. Patients may also have autonomic symptoms ***(name 3)***. \_\_\_ is more common in ***myasthenia gravis***, which also shows proximal muscle weakness but lacks the __ \_\_ \_\_\_of strength and reflexes seen here.

Answer 71

***Lambert-Eaton myasthenic syndrome (LEMS)*** ***autonomic symptoms like dry mouth, erectile dysfunction, or sluggish pupillary response to*** ***light***. *Ptosis* is more *common in myasthenia gravis*, which also shows proximal muscle weakness but ***lacks the exercise-induced improvement of strength*** and reflexes seen here.

Question 72

About 60% of patients with LEMS have an underlying malignancy causing the symptoms, which is why LEMS is known as a\_\_\_ syndrome. The most common malignancy associated with LEMS is s\_\_ __ \_\_ \_\_\_. Antibodies to\_\_ __ \_\_\_ at the __ \_\_ play a part in the pathophysiology of the disease.

Answer 72

About 60% of patients with LEMS have an underlying malignancy causing the symptoms, which is why LEMS is known as a ***paraneoplastic syndrome***. The most common malignancy associated with LEMS is ***small cell lung carcinoma***. Antibodies to ***presynaptic calcium channels*** at the ***neuromuscular junction*** play a part in the pathophysiology of the disease.

Question 73

Trousseau syndrome?

Answer 73

Trousseau syndrome is an acquired blood clotting disorder that results in migratory thrombophlebitis ( inflammation of a vein due to a blood clot).

Question 74

Nonbacterial thrombotic endocarditis ( NBTE) is?

Answer 74

Nonbacterial thrombotic endocarditis ( NBTE) is a form of endocarditis in which small sterile vegetations are deposited on the valve leaflets. These excrescences, composed of fibrin, platelets, and red blood cells, resemble thrombi. Formerly known as marantic endocarditis, which comes from the Greek marantikos, meaning "wasting away"

Question 75

Autoimmune paraneoplastic diseases linked with thymoma include:

Answer 75

***Myasthenia gravis*** (the most common autoimmune paraneoplastic disease linked with thymoma). ***Thymoma-associated hypogammaglobulinemia*** (Good syndrome). ***Thymoma-associated autoimmun***e pure red cell aplasia.

Question 76

***Calcitriol*** is the active form of

Answer 76

Calcitriol is the ***active form of vitamin D, normally made in the kidney.*** It is also known ***as 1,25-dihydroxycholecalciferol.*** It is a *hormone which binds to and activates the vitamin D receptor in the nucleus of the cell*, which *then increases the expression of many genes.* Calcitriol increases blood calcium (Ca2+) mainly by increasing the uptake of calcium from the intestines.

Question 77

***Calcitriol*** It can be given as a medication for the treatment of

Answer 77

It can be given as a medication for the treatment of *low blood calcium and hyperparathyroidism due to kidney disease, low blood calcium due to hypoparathyroidism, osteoporosis, osteomalacia, and familial hypophosphatemia*

Question 78

***Erythropoietin*** , also known as ?

Answer 78

***Erythropoietin*** , also known as erythropoetin, haematopoietin, or haemopoietin, - is a g***lycoprotein cytokine secreted mainly by the kidney in response to cellular hypoxia***; - it ***stimulates red blood cell production (erythropoiesis) in the bone marrow.*** - Low levels of ***EPO (around 10 mU/mL)*** are constantly secreted sufficient to compensate for normal red blood cell turnover.

Question 79

***Pheochromocytomas*** are a type of ?

Answer 79

Pheochromocytomas are a type of paraganglioma, rare, usually noncancerous (benign) tumor that develops in an adrenal gland -They are catecholamine-secreting tumors derived from chromaffin cells. They typically demonstrate a nesting (Zellballen) pattern on microscopy. This pattern is composed of well-defined clusters of tumor cells containing eosinophilic cytoplasm separated by a fibrovascular stroma.

Question 80

Pheochromocytoma Clin Feat:

Answer 80

Question 81

A hemangioblastoma ?

Answer 81

A ***hemangioblastoma*** is a benign, highly vascular tumor (blood vessel tumor) of the cerebellum, spinal cord, or retina, consisting of proliferated blood vessel cells and angioblasts. ## Footnote Those arising in the cerebellum (cerebellar hemangioblastoma) may be cystic and associated with von hippel-lindau disease. Called also ***angioblastoma.*** cerebellar hemangioblastoma of the cerebellum, often cystic; an autosomal dominant form is associated with ***von Hippel-Lindau disease.***

Question 82

Identify condition and define

Answer 82

***Acanthosis nigricans*** A skin pigmentation problem characterised by dark, velvety, and thick patches of skin usually formed in the skin folds and creases Associated with underlying malignancies: ***Gastric Adenocarcinoma and viceral malignancies***

Question 83

***Hypertrophic osteoarthropathy*** (HOA) is WHAT?

Answer 83

Hypertrophic osteoarthropathy (HOA) is a s***yndrome of clubbing of the digits, periostitis of the long (tubular) bones, and arthritis***

Question 84

*Heliotrope ras*h is a characteristic symptom of?

Answer 84

*Heliotrope rash is a characteristic symptom of* ***Dermatomyositis.*** ***Dermatomyositis*** is idiopathic inflammatory myositis which causes persistent inflammation on the muscle and skin. The heliotrope rash is developed *on the upper eyelids and become inflamed.* Usually the condition is *associated with violaceous erythema of the upper eyelids, in addition edema and telangiectasia also progress in the heliotrope rash.*