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ACP Breast, skin, endocrine > L37 Endocrine pathology - Parathyroid, adrenal, MEN > Flashcards

L37 Endocrine pathology - Parathyroid, adrenal, MEN Flashcards

1
Q

_________ is the MC adult endocrine disorder.

A

Primary hyperparathyroidism

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2
Q

What are the causes of primary hyperparathyroidism? (3)

How to differentiate them, since they are histologically similar?

A
  1. Adenoma (80%) (1/4 of the parathyroid glands)
  2. Hyperplasia (15%) (multiple parathyroid glands)
  3. Carcinoma (5%) (metastatic features, capsular and vascular invasions)
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3
Q

Primary hyperparathyroidism is associated with a parathyroid neoplasia gene on 11q13 named _____________ and ____________ genes.

A

PRAD1 (cyclone D1);

MEN1 genes

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4
Q

Name some functional abnormalities of primary hyperparathyroidism. (5)

A

Hyperparathyroidism

  1. painful bones [increased action of osteoblasts]
  • may lead to brown tumor, radiologically undifferentiable with primary giant cell tumor of bone
  • osteitis fibrosa cystica
  1. Renal stones [increased renal absorption of Ca2+]
    - nephrolithiasis
    - nephrocalcinosis
    - polyuria + secondary polydipsia
  2. Abdominal groans
    - constipation, nausea, peptic ulcer, pancreatitis, gall stones
  3. Psychic moans
    - depression, lethargy, seizures
  4. Metastatic calcification
    - stomach, lungs, heart, vessels
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5
Q

Function of PTH?

A

PTH: increase Ca, decrease PO4-

  • activate osteoblast, increase renal absorption, increase conversion of vitamin D in kidney
  • increase phosphate excretion
  • increase GI reabsorption
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6
Q

Other than primary hyperparathyroidism, name other causes for hyperparathyroidism.

A
  1. Secondary
    - Chronic renal failure
    - Inadequate Ca intake
    - Vitamin D insufficiency
    - Steatorrhea
  2. Tertiary
    - Chronic renal failure
    - Hyperplasia > autonomous
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7
Q

Which of the following about parathyroid disease is correct?

A. Histologically, we can differentiate parathyroid adenoma, carcinoma and hyperplasia.

B. There is decreased adopts tissue in parathyroid disease

C. There is mild to moderate pleomorphism and atypic

D. There are a few clusters of oxyphil cells and clear cells

E. There is rare mitosis

A

All except A

Difficult

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8
Q

What are the causes for hypoparathyroidism?

A
  1. Autoimmune (MC)
  2. Surgery
  3. Congenital (DiGeorge syndrome)
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9
Q

What are the clinical presentations of hypoparathyroidism? (2)

A
  1. Tetany
  2. Parkinsonism (metastatic calcification at basal ganglia)
  • mental statsis, papilloedema, catatract, prolong QT, dental abnormailities
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10
Q

What are the causes for adrenal insufficiency?

A
  1. Primary
    - Congenital adrenal hypoplasia (CAH)
    - Autoimmune
    - Neoplastic
  2. Secondary
    - Long term use of steroids
  3. Acute: Waterhouse-Friderichsen Syndrome
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11
Q

What are the hormone from the adrenal cortex? (3)

What are the diseases caused by their excess respectively? (3)

A
  1. Glucocorticoid (cortiso;)
    - Cushing syndrome
  2. Mineralocorticoid (aldosterone)
    - Hyperaldosteronism
  3. Adrenocortical androgen
    - Adrenogenital syndrome
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12
Q

What are the causes for Cushing’s syndrome? (4)

A
  1. Exogenous
    - bilateral adrenal atrophy, sparing zones glomerulosa
  2. Pituitary (Cushing’s disease)
    - bilateral hyperplasia, thicken cortex (yellowish)
  3. Adrenal
    - adrenocortical neoplasm (adenoma/carcinoma)
  4. Ectopic ACTH: SCLC, MTC, (medullary thyroid carcinoma), carcinoid tumor, islet cell tumor of pancreas
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13
Q

How is adrenal adenoma or carcinoma differentiated?

A

By invasion, metastasis and size (larger in carcinoma)

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14
Q

Causes for hyperaldosteronism? (2)

A
  1. Primary
    - Conn’s syndrome
    = bilateral idiopathic adrenal hyperplasia (reduced renin)
  2. Secondary
    - decreased renal function, hypovolemia, pregnancy (increased renin)
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15
Q

Causes for adrenogenital syndrome? (2) Briefly describe

A
  1. Neoplasm
    - more likely androgen-secreting carcinoma
  2. Congenital adrenal hyperplasia (CAH): MC 21-hydoxylase deficiency;

increases ACTH due to reduced cortisol, bilateral hyperplasia

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16
Q

What are the clinical features for Cushing syndrome? Name 5.

A
  • Baldness and facial hirsutism in females
  • Buffalo hump
  • Hypertension
  • Skin thinning
  • Easy bruising, poor wound healing
  • Acne
  • Moon face, plethoric cheeks
  • Osteoporosis
  • Increased abdominal fat
  • Abdominal striae
  • Avascular necrosis of femoral head
  • Muscle weakness
17
Q

What can be seen histologically in adrenal hyperplasia?

A

Diffusely enlarged glands due to hyperplastic cortex

18
Q

_____________ is a 10% tumor in which it is

10% malignant,
10% bilateral,
10% familial and
10% extra-adrenal;

it is adrenaline secreting adrenal medullar disorder.

A

Pheochromocytoma

19
Q

Pheochromocytoma
A. Arises in adrenal meulla
B. Causes hypertension
C. Diagnosed by urine catecholamines and metabolites
D. Hemorrhagic, necrotic and cystic in large lesions
E. Capsular and vascular invasion is seen only in malignancies

A

E is incorrect
- may be seen in benign lesions

Others

  • circumscribed, yellow, polygons to spindle cells
  • Zellballen pattern - small nests of tumor cells with rich vascular network
20
Q

What is the diagnostic criteria for malignant pheochromocytoma?

What can be seen in histological slides?

A

Distant metastasis (only criteria)

  • Zellballen pattern - small nests of tumor cells with rich vascular network
21
Q

What is the MC tumor and malignancy in childhood?

It is frequently in adrenal medulla but can be anywhere in SNS.

A

Neuroblastoma

22
Q

What is the pathology of neuroblastoma? (3)

A

SBRCT
- Small, blue, round cell tumors

(Small round cells that stain blue on routine H&E stained sections;
~Ewing sarcoma and medulloblastoma, lymphoma, rhabdomyosarcoma )

23
Q

Prognosis of neuroblastoma is based on?

A

Depends on histology and staging

24
Q

Multiple endocrine neoplasia syndromes

A. May manifest in affected organs as hyperplasia, adenoma or caricinoma

B. Is more aggressive compared to sporadic cases

C. Has high recurrence compared to sporadic cases

D. Has later onset than sporadic cases (older)

E. Has synchronous or metachonous involvement of multiple endocrine glands

F. Has multi-focal involvement within a gland

A

All except D

Younger onset

25
Q

What is the mode of inheritance of MEN syndromes?

A

Autosomal dominant

26
Q

What gene mutation is involved in MEN1? (1)

What organs are affected? (3)

A
  • 11q13, MEN1 tumor suppressor gene

3Ps

  1. Parathyroid
    - primary hyperparathyroodism due to hyperplasia
  2. Pancreas
    - Zollinger-Ellison syndrome, hypoglycemia
    - functional, aggressive, leading cause of death
  3. Pituitary
    - Prolactinoma, somatrotrophin-secreting tumors
27
Q

What gene mutation is involved in MEN2 syndrome?

A

Activating mutation of RET

- Strong genotype-phenotype correlation

28
Q

What are the different types of MEN2 syndromes? Describe.

A

MEN2A and 2B

29
Q

What organs are affected in MEN 2A syndrome? (3)

A

MEN2A

  1. Thyroid
    - MTC= C-cell hyperplasia, multi-focal
  2. Adrenal medulla
    - pheochromocytoma
  3. Parathyroid
    - hyperplasia
30
Q

What organs are affected in MEN 2B syndrome (William syndrome)? (3)

A
  1. Thyroid
    - MTC
  2. Adrenal medulla
    - pheochromocytoma
  3. Extra-endocrine
    - ganglioneuroma/ neuroma of mucosal sites
    - Marfanoid habitus (a constellation of symptoms resembling those of Marfan syndrome, including long limbs, with an arm span that exceeds the height of the individual, sometimes with a high arch in the palate, arachnodactyly.)

*No hyperparathyroidism

31
Q

What are the main differences between MEN2A and B?

A

MEN2B has no hyperparathyroism

and has extra-endocrine involvement

32
Q

What is FMTC in MEN2 syndrome?

A

Familial medullary thyroid carcinoma

- just medullary carcinoma

33
Q

Neuroendocrine tumors are graded based on? (2)

A

Mitoses and/or Ki67 index
(increases with grading)

(Also a reference for cell division (mitosis))

34
Q

PanNET (neuroendocrine tumours) grading from G__ to ____?

A

G1-G3
G1 Low grade
G2 Intermediate grade
G3 High grade

35
Q

What are the different tumors in different grades in Pancreas & GIT NET?

A

Pancreas & GIT

G1 (low grade)
- Carcinoid tumor

G3 (high grade)
- Neuroendocrine carcinoma

36
Q

What are the different tumors in different grades in Lung NET?

A

Lungs

G1 (low grade)
- Typical carcinoid tumor

G2 (intermediate grade)
- Atypical carcinoid tumor

G3 (high grade)

  • Small cell carcinoma
  • Large cell neuroendocrine carcinoma

ACP Breast, skin, endocrine (4 decks)

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