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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
What type of renal cancer is associated with Von Hippel Lindau?
Bilateral clear cell RCC
26
What type of genital cancer is common in Von Hippel Lindau?
Papillary cystadenocarcinoma of the epididymis
27
Think Von Hippel Lindau Note: Cerebellar/spinal cord tumors (hemangioblastomas) and bilateral renal tumors (clear cell carcinomas).
28
What type of pancreatic tumor is associated with Von Hippel Lindau?
Serous cystadenoma Note: 75% of VHL pts have pancreatic cysts, but serous cystadenoma is the most common pancreatic tumor.
29
PGL syndrome
Paraganglioma and pheochromocytoma syndrome (multiple head and neck paragangliomas +/- pheochromocytoma)
30
Facial angiofribroma, seizures, mental retardation...
Think tuberous sclerosis Note: This is the classic clinical triad.
31
Clinical triad for tuberous sclerosis
- Facial angiofibromas - Seizures - Mental retardation
32
Facial angiofibromas (often seen in tuberous sclerosis)
33
Tuberous sclerosis Note: Cortical tubers (A) and subependymal nodules (B).
34
Think subependymal giant cell astrocytoma (SEGA) in a pt with tuberous sclerosis Note: You can see cortical tubers peripherally.
35
What renal lesions are common in tuberous sclerosis?
Multiple renal angiomyolipomas (80%)
36
What cardiac lesions are associated with tuberous sclerosis?
Cardiac rhabdomyosarcoma
37
What pulmonary findings are common in tuberous sclerosis?
Lymphangiolyomyomatosis (LAM)
38
Imaging features of tuberous sclerosis
39
What is the most common pituitary tumor in MEN I?
Prolactinoma
40
Flushing and diarrhea in a pt with MRN IIa or IIb?
Calcitonin-producting thyroid cancer (MEN II syndromes are usually associated with medullary thyroid cancer)
41
Familial medullary thyroid (FMTC)
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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