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Core > Endocrine: Syndromes > Flashcards

Endocrine: Syndromes Flashcards

1
Q

What are the types of multiple endocrine neoplasia syndromes?

A
  • MEN I
  • MEN IIa
  • MEN IIb
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2
Q

MEN I is associated with…

A
  • Pituitary adenoma
  • Parathyroid hyperplasia (90%)
  • Pancreatic tumor (usually gastrinoma)

Note: 3 P’s of MEN I.

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3
Q

What is the most common pancreatic tumor in MEN I?

A

Gastrinoma

Note: Look for Zollinger-Ellison syndrome.

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4
Q

Severe, persistent peptic ulcer disease in a pt with parathyroid hyperplasia…

A

Think MEN I

Note: 3 Ps of MEN I are pituitary adenoma, parathyroid hyperplasia, and pancreatic tumor (usually gastrinoma causing Zollinger-Ellison syndrome).

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5
Q

MEN IIa is associated with…

A
  • Medullary thyroid cancer (100%)
  • Parathyroid hyperplasia
  • Pheochromocytoma (33%)

Note: 3 A’s of MEN IIa are Adrenal (pheo), Aggressive thyroid cancer (medullary), and A lot of calcium (parathyroid hyperplasia).

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6
Q

What is the most common finding in MEN I?

A

Parathyroid hyperplasia (90%)

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7
Q

What is the most common finding in MEN IIa?

A

Medullary thyroid cancer (100%)

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8
Q

What is the most common finding in MEN IIb?

A

Medullary thyroid cancer (80%)

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9
Q

Pheochromocytomas are most frequently found in pts with which type of MEN?

A

MEN IIb (50%)

Note: Also found in MEN IIa (33%).

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10
Q

MEN IIb is associated with…

A
  • Medullary thyroid cancer (80%)
  • Pheochromocytoma (50%)
  • Mucosal neuroma
  • Marfanoid body habitus

Note: MEN IIb is a wannaBe, its exactly like IIa (pheochromocytomas and medullary thyroid cancer), but instead of the parathyroid hyperplasia of IIa it has Body habitus of marfans and ganglioneuroma.

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11
Q

Which MEN syndrome is associated with a marfanoid body habitus?

A

MEN IIb

Note: B for marfanoid Body habitus.

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12
Q

Which MEN syndrome is associated with Zollinger-Ellison syndrome?

A

MEN I

Note: The P’s Pancreatic tumor is most often a gastrinoma.

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13
Q
A

Mucosal neuromas, commonly seen in pts with MEN IIb

Note: Look for the other MEN IIb associations of medullary thyroid cancer and pheochromocytoma.

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14
Q

Which MEN syndrome is associated with pituitary adenomas?

A

MEN I

Note: The 3 P’s of MEN I (Pituitary adenoma, Parathyroid hyperplasia, and Pancreatic tumor/gastrinoma).

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15
Q

Which MEN syndrome is associated with hypercalcemia due to parathyroid hyperplasia?

A

MEN I and MEN IIa

Note: 3 P’s of MEN I (parathyroid hyperplasia) and A’s of MEN IIa (A lot of calcium).

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16
Q

What is the most common primary location for a carcinoid tumor?

A

The distal ileum

Note: GI carcinoid (70%) is more common than pulmonary carcinoid.

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17
Q

Flushing, diarrhea, and right heart failure…

A

Think GI carcinoid that has metastasized to the liver

Note: The serotonin from liver metastases causes right-sided heart dysfunction (unlike the left-sided heart dysfunction caused by serotinin from primary pulmonary carcinoid).

18
Q

What percentage of pts with a carcinoid tumor develop carcinoid syndrome?

A

10%

Note: Carcinoid syndrome is overrepresented on tests.

19
Q

Abdominal tumor and elevated urine 5-HIAA (5-hydroxyindoleacetic acid)…

A

Think carcinoid tumor

20
Q

What is the best nuclear imaging test for a carcinoid tumor?

A

Indium 111-Octreotide scan

Note: Gallium is positive but very nonspecific. MIBG is only positive in 15% of cases.

21
Q

Most common cardiac complication of a GI carcinoid tumor

A

Tricuspid regurgitation (and other right heart issues)

Note: Only occurs after the GI carcinoid tumor has metastasized to the liver (otherwise the liver will metabolize the serotonin that carcinoid tumors produce).

22
Q

GI carcinoid tumors are associated with what other tumors?

A

Other GI tumors (e.g. adenocarcinoma)

23
Q

What is the most common tumor seen in Von Hippel Lindau?

A

Hemangioblastoma

Note: These are usually in the retina, cerebellum, and/or spinal cord.

24
Q

Von Hippel Lindau is associated with…

A
  • Hemangioblastomas (in the retina, cerebellum, spinal cord)
  • Bilateral renal clear cell carcinoma (70%)
  • Papillary cystadenoma of the epididymis (55%)
  • Endolymphatic sac tumor (10%)
  • Pheochromocytoma (10%)
  • Pancreatic cysts and serous cystadenomas
25
Q

What type of renal cancer is associated with Von Hippel Lindau?

A

Bilateral clear cell RCC

26
Q

What type of genital cancer is common in Von Hippel Lindau?

A

Papillary cystadenocarcinoma of the epididymis

27
Q
A

Think Von Hippel Lindau

Note: Cerebellar/spinal cord tumors (hemangioblastomas) and bilateral renal tumors (clear cell carcinomas).

28
Q

What type of pancreatic tumor is associated with Von Hippel Lindau?

A

Serous cystadenoma

Note: 75% of VHL pts have pancreatic cysts, but serous cystadenoma is the most common pancreatic tumor.

29
Q

PGL syndrome

A

Paraganglioma and pheochromocytoma syndrome (multiple head and neck paragangliomas +/- pheochromocytoma)

30
Q

Facial angiofribroma, seizures, mental retardation…

A

Think tuberous sclerosis

Note: This is the classic clinical triad.

31
Q

Clinical triad for tuberous sclerosis

A
  • Facial angiofibromas
  • Seizures
  • Mental retardation
32
Q
A

Facial angiofibromas (often seen in tuberous sclerosis)

33
Q
A

Tuberous sclerosis

Note: Cortical tubers (A) and subependymal nodules (B).

34
Q
A

Think subependymal giant cell astrocytoma (SEGA) in a pt with tuberous sclerosis

Note: You can see cortical tubers peripherally.

35
Q

What renal lesions are common in tuberous sclerosis?

A

Multiple renal angiomyolipomas (80%)

36
Q

What cardiac lesions are associated with tuberous sclerosis?

A

Cardiac rhabdomyosarcoma

37
Q

What pulmonary findings are common in tuberous sclerosis?

A

Lymphangiolyomyomatosis (LAM)

38
Q

Imaging features of tuberous sclerosis

39
Q

What is the most common pituitary tumor in MEN I?

A

Prolactinoma

40
Q

Flushing and diarrhea in a pt with MRN IIa or IIb?

A

Calcitonin-producting thyroid cancer (MEN II syndromes are usually associated with medullary thyroid cancer)

41
Q

Familial medullary thyroid (FMTC)

A

A hereditary endocrine neoplasia syndrome (can be considered a subtype of MEN II) that is associated with thyroid cancers at a young age

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