MEN- Station 1 Flashcards

STEM; 44 year male patient seen in the clinic, history of mild asthma & parathyroid surgery he had intermitted abdominal discomfort & pancreatic disease is being considered .

1
Q

STEM; 44 year male patient seen in the clinic, history of mild asthma & parathyroid surgery he had intermitted abdominal discomfort & pancreatic disease is being considered
….
Q1: Define parathyroid hyperplasia ?

A

Non-malignant pathology in which parathyroid cells proliferates and enlarges

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2
Q

Q2: how many parathyroid glands usually affected ?

A

Four

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3
Q

Q3: microscopic picture of parathyroid hyperplasia ?

A

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

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4
Q

Q4. define the 2 top reasons of parathyroid hyperplasia ?

A

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

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5
Q

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 ?

A

Insulinoma

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6
Q

Q6 : which part of the pancrease is usually affected ?

A

Beta cells of islets of Langerhans

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6
Q

Q7. How insulinoma is confirmed biochemically ?

A

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

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7
Q

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

A

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

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8
Q

Q9: define MEN ?

A

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

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9
Q

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

A

Pit. Adenoma

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10
Q

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

A

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

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11
Q

Q12: what is the two hit hypothesis ?

A

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

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12
Q

Q13: what is telomere ?

A

Region of repetitive nucleotide sequences at the end of the chromosome

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13
Q

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

A

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

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14
Q

Q-Genes of MEN syndrome?

A

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

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