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Pathology > Endocrine > Flashcards

Endocrine Flashcards

1
Q

What genes are associated with MEN1 syndrome

A

MEN1
CDKN1B

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2
Q

What genes are associated with MEN2 syndrome

A

RET genes

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3
Q

What tumours are associated with MEN1 syndrome and what are the approximate lifetime incidence (from eviq)

A

Mostly benign tumours
Parathyroid adenoma 90-95%
Pituitary adenoma 30-40%
GDP-NET 90% (gastroduodenopancreatic)
Adrenocortical adenoma 30-40%
Bronchopulmonary NET (indolent) 20%
Thymine NET (usually malignant) 2%
Meningioma 8%

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4
Q

What tumours are associated with MEN2 syndrome

A

Medullary thyroid carcinoma (90% by age 75)
Phaeochromocytoma
Primary hyperparathyroidism

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5
Q

What is a common term used to describe well differentiated neuroendocrine tumours

A

Carcinoid

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6
Q

What is the microscopic appearance of well differentiated neuroendocrine tumours (Kurt’s notes)

A

Uniform cells, round nuclei
Salt and pepper granular chromatin in nucleus
Granular cytoplasm
Organoid architecture (cells arranged into nests, cords, rosettes or ribbons)
No necrosis

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7
Q

What is detected using a DOTA scan

A

Somatostatin receptors expressed by well differentiated neuroendocrine tumours

68Ga-DOTA-somatostatin analog-PET/CT is best.
Scintigraphy is considered inferior

FDG PET is often negative in grade 1 NETs, but may be positive in grade 2 and 3

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8
Q

What is the usual behaviour of well differentiated neuroendocrine tumours

A

Malignant, but slow growing and indolent. Early NETs have low rates of metastasis.

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9
Q

How are well differentiated neuroendocrine tumours graded

A

Based on Ki67 and mitotic rate
G1: ki67 <3% mitotic index <2
G2: 3-20%, 2-20
G3: >20%, >20

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10
Q

How are poorly differentiated neuroendocrine tumours graded

A

They are not, but they can either be small cell or large cell types

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11
Q

What are the microscopic features of poorly differentiated small cell neuroendocrine tumours

A

Small size: <3 lymphocytes
Fusiform (elongated/tapering at both ends) nuclei
Cells appear bland/sheet like growth (as opposed to being pleomorphic like carcinomas)
Fine granular chromatin
Scanty cytoplasm
Extensive necrosis
Very high mitotic rate, ki67 nearly 100%

See Kurt’s notes “GI neuroendocrine tumours” and handbook for surgical pathologists pg 151

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12
Q

What are is the microscopic architecture and cytology associated with neuroendocrine tumours

A

Architecture: ie organoid architecture such as nests, cords, rosettes
Cytology: monotonous/uniform cells. contain secretory granules, secrete peptides and amines locally.
See handbook for surgical pathologists page 169

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13
Q

What are the microscopic features of large cell neuroendocrine tumours

A

Large cells, round nuclei, prominent nucleoli, moderate cytoplasm.
Sheet-like to nested growth
Ki67 usually 60-80%

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14
Q

What is the common pathway that a poorly differentiated neuroendocrine tumour develops

A

Arise from a non-neuroendocrine tumour and develop neuroendocrine differentiation

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15
Q

What syndrome is associated with Gastrinomas, and what are the common resulting pathology

A

Zollinger-Ellison syndrome
Acid hypersecretion causes extensive peptic ulcers. Most commonly tumour in proximal duodenum

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16
Q

What are the symptoms of carcinoid syndrome and what is the cause. How is it tested for

A

Flushing, diarrhoea, bronchoconstriction
Due to serotonin and kallikrein secretion from neuroendocrine tumours, usually only if they are metastatic to liver.
Test serum 5HT or urinary 5-HIAA

17
Q

How do insulinomas usually present

A

Usually present when small/early due to hypoglycaemia

18
Q

Which layer of the GI tract do neuroendocrine tumours usually grow in

A

Either the submucosa, or muscle with normal overlying mucosa

19
Q

What are the common sites of neuroendocrine tumours in the GI tract

A

Small bowel most common: ileum

Appendix, rectum, stomach, pancreas

20
Q

Are the majority of well differentiated neuroendocrine tumours functioning or non-functioning

A

Non-functioning

21
Q

What percentage of adrenal adenomas are functional

A

15%

22
Q

What are the two most common products of functional adrenal adenomas

A

Aldosterone: causes hypertension and low potassium
Cortisol: (independent of ACTH control) causes Cushing syndrome; central obesity, poor healing, striae, hirtuitism

23
Q

What syndromes is adrenocortical carcinoma associated with

A

Mostly sporadic
Li Fraumeni
Lynch

24
Q

What are microscopic features of adrenal cortical carcinoma

A

Thick fibrous capsule
Necrosis
Vascular or capsular invasion
Increased mitotic activity
Solid or nested growth

25
Q

What tool is used to attempt to distinguish adrenal cortical adenoma from adrenal cortical carcinoma, what are it’s components

A

Modified Weiss criteria
Mitotic rate >6 per 50 HPF
Cytoplasm clear vs compact
Abnormal mitosis
Tumour necrosis
Invasion of capsule

26
Q

Aside from the Weiss criteria, what two things can be used to distinguish adrenal cortical adenoma and carcinoma

A

Ki67: >5% associated with carcinoma
Loss of reticulum framework between clusters of cells + one of tumour necrosis, high mitotic rate, venous invasion

27
Q

What is a myelolipoma

A

A benign mass, second most common adrenal neoplasm
Microscopically contains mature fat and bone marrow elements
Can be diagnosed on imaging due to high fat content

28
Q

What part of the adrenal do phaeochromocytoma arise from

A

The adrenal medulla

29
Q

What proportion of pheochromocytoma are considered malignant

A

All are. But rarely metastasize.

30
Q

What can pheochromocytoma produce, and what are the resulting symptoms

A

Catecholamines
-hypertension, headache, tachycardia, palpitations, sweating

31
Q

What are some typical microscopic features of pheochromocytoma

A

Nested architecture
Purple cytoplasm
Rich vascularity (frequently haemorrhage)

32
Q

What is the typical IHC pattern of pheochromocytoma

A

Chromogranin, synaptophysin diffuse positivity
S100
SOX10

Negative: cytokeratins, SF1, Inhibin

33
Q

What type of tumour are paragangliomas functionally and morphological related to

A

Pheochromocytoma

34
Q

Where can paragangliomas arise

A

Arise from extra-adrenal paraganglia
Head and neck: arise from parasympathetic nerves. Generally non functional and good prognosis
Sympathetic paragangliomas: arise from pre sympathetic and parasympathetic chains. Frequently functional and same risks as pheochromocytoma

35
Q

What is the IHC profile of angiosarcoma

A

cD31, CD34 positive (vascular markers)
MYC+ in radiation/lymphoedema associated tumours
HHV8- (kaposi is key differential)

Pathology (16 decks)

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