MEN- Station 1

Question 1

Q1: Define parathyroid hyperplasia ?

Answer 1

Non-malignant pathology in which parathyroid cells proliferates and enlarges

Question 2

Q2: how many parathyroid glands usually affected ?

Answer 2

Four

Question 3

Q3: microscopic picture of parathyroid hyperplasia ?

Answer 3

Chief cell (Most commonly in diffuse or multinodular pattern)/ Water clear cells (less common) / Island of oxyphils and delicate fibrous strands

Question 4

Q4. define the 2 top reasons of parathyroid hyperplasia ?

Answer 4

Renal Failure and Men(type 1 , 2a) Syndr.

Question 5

Q5. The patient collapsed in the clinic his glucose level can’t be recorded immediately, later confirmed to be 1.2 mmol/ liter by blood sample , History showed repeated attacks of sweating ad fainting, what pancreatic neoplastic process may cause this clinical picture ?
Insulinoma

Answer 5

Insulinoma

Question 6

Q6 : which part of the pancrease is usually affected ?

Answer 6

Beta cells of islets of Langerhans

Question 6

Q7. How insulinoma is confirmed biochemically ?

Answer 6

By High levels of insulin / high insulin – glucose ratio / presence or proinsulin and high c-peptide

Question 7

Q8: apart from insulinoma what other pathology can create this condition ?

Answer 7

Isulin Like growth factor from non-pancreatic tumor / Ectopic insulin production by fibrosarcoma / diffuse liver disease.

Question 8

Q9: define MEN ?

Answer 8

Multiple Endocrine neoplasia Group of related conditions inhertided as AD trait chch by hyperplesia or neoplasia of involved organs

Question 9

Q10. what other pathology would you suspect in this patient ?

Answer 9

Pit. Adenoma

Question 10

Q11: what are the gene mutations that happens in insulinoma ?

Answer 10

MEN-1 gene / Loss-of-function mutations in tumor suppressor genes like PTEN TSC2 / Inactivating mutations in 2 genes (ATRX – DAXX)

Question 11

Q12: what is the two hit hypothesis ?

Answer 11

Tumor supp gene is recessive in nature in order for particular cell to be carcinogenic needs mutation of both genes

Question 12

Q13: what is telomere ?

Answer 12

Region of repetitive nucleotide sequences at the end of the chromosome

Question 13

Q14: what are the genes responsible for cancer in general ?

Answer 13

Tumor supp genes (P53 – SPC1/2) – Oncogenes (K-Ras) – Protooncogenes

Question 14

Q-Genes of MEN syndrome?

Answer 14

MEN type 1  MEN-1 gene
Type 2,3  RET Oncogene – CDKN1pg??