pathology 2 Flashcards by Anjali Yapa

which condition causes:
-systolic murmur loudest at the back below left scapula
-rib notching on CXR
-radiofemoral delay
-can have syncope/ claudication

Aortic coarctation

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Why do you see rib notching on CXR of aortic coarctation?

The intercostal vessels act as collaterals and are dilation

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At which site is aortic coarctation most common

Site of ductus arteriosus

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Groups likely to get aortic coarctation

Boys
Girls w/ turners

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Mx for aortic coarctation

-angioplasty
-surgical resection

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what are the tumours found in patients with MEN 1

3 P’s + thyroid + adrenal adenoma

-Pituitary (secretes ACTH, GH, prolactin)
-Parathyroid
-pancreatic (islet cells/ ZEllison)

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what is the gene and chromosome mutation in MEN 1

MENIN gene
Chr 11

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what are the tumours found in patients with MEN 2A

-Phaeochromocytoma
-Medullary thyroid carcinoma
-Hyperparathyroidism

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what are the tumours found in patients with MEN 2b

-Phaeochromocytoma
-Medullary thyroid
-hyperparathyroid
+
marfanoid features
+
mucosal neuromas

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what is the gene and chromosome mutation in MEN 2a/b

RET-oncogene
Chr 10

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which cells of the thyroid produce the hormone that reduces calcium levels

Parafollicular cells/ C-cells produce calcitonin

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what is the inhertience pattern for MEN

Autosomal dominant

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What are the colonic and extracolonic features seen in Gardners syndrome

Colonic:
colonic polyps

Extra:
jaw/ skull osteomas
epidermal/ sebaceous cysts
desmoid tumours
Thyroid cancer

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T/F gardners syndrome is a variant of FAP

True

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What are the gene/ chromosome affected in Gardners syndrome

APC gene
Chr 5

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What is the inheritence pattern for Gardners?

Autosomal dominant

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What are the features of Lynch syndrome

Colonic + endometrial cancer

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What is the criteria used to identify high risk patients for Lynch syndrome

Amsterdam criteria:

-needs 3 or more members of the family affected with 1 being a first degree relative of the other 2

-2 successive generations affected

-1 or more colon cancers diagnosed under 50 years

-FAP has been excluded

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What is the inheritence pattern for Lynch?

Autosomal dominant

What are the characteristic malignancies in Li-Farumani syndrome

Leukaemias
Sarcomas
breast cancers
brain Ca
adrenalCa

What gene mutation is seen in Li-Farumani syndrome?

p53 tumour suppressor gene

What is the inheritence pattern for Li-Farumani syndrome?

Autosomal dominant

What is the management of AAA according to its size

<2cm nothing
<5cm surveillance imaging
5.5-6cm surgery

What are the indications for surgical management of AAA

-size 5.5-6cm and asymptomatic
-symptomatic
-ruptured AAA

What age does screening for AAA start

65 yrs

What characteristics of the aneurysm are required for an EVAR

-long neck -straight iliac vessels -healthy groin vessels

What is resected in a right hemicolectomy

caecum + ascending colon +/- right half of transverse colon ileocolic + right colic + right branch of middle colic vessels

Complications following AAA repair

-ITU stay -cardiac: reperfusion injury, complications from cross clamp -renal injury/ AKI -infections -aorto-enteric fistula -embolism (gut/ feet)

What are the common sites of rupture of a AAA and which is more common?

anteroperitoneal retroperitoneal (more common)

What is resected in a left hemicolectomy

descending colon +/- left half of transverse left colic + left branch of middle colic + IMA

What is resected in a anterior resection

high rectum

What is resected in a Hartmann's

sigmoid + rectum + colostomy + close rectal stump

How would you treat a DALM

panproctocolectomy

What is the diagnosis for PC: cyclical pyrexia, cough, haemoptysis, weightloss, asymptomatic lymphadenopathy

Hodgkins lymphoma

What is the staging for hodgkins

Ann Arbor 1-single lymph node region 2- multiple nodes on same side of diaphragm 3- nodes on both sides of diaphragm 4-extranodal sites

what are the subtypes of hodgkins

Nodular sclerosing Hodgkin lymphoma (NSHL) Mixed-cellularity Hodgkin lymphoma (MCHL) Lymphocyte-depleted Hodgkin lymphoma (LDHL) Lymphocyte-rich classical Hodgkin lymphoma (LRHL)

Tx of Hodgkins

both chemo and radiotherapy

Which oncovirus causes both Burkitss and Hodgkins?

EBV

What are the cellular changes in blood that are seen following a splenectomy (in time order)

granulocytosis thrombocytopenia target cells + siderocytes + reticulocytes lymphocytosis monocytosis

What is the rule of thirds for carcinoid syndrome

1/3 multiple cancers 1/3 second tumour 1/3 small bowel 1/3 metastasize

Where do carcinoid tumours originate

neuroendocrine cells usually in the gut (ileum/ appendix)

What do carcinoid tumours secrete

serotonin

T/F: carcinoid tumours on the appendix can present with carcinoid syndrome even without mets to the liver/ lung

False tumours need to metastasize outsid eof the gut to cause the carcinoid syndrome

Investigations for carcinoid syndrome

-5-HIAA in a 24-hour urine collection -Somatostatin receptor scintigraphy -CT scan -Blood testing for chromogranin A

Mx of carcinoid syndrome

octreotide surgery

Clinical features of carcinoid syndrome

flushing palpitations diarrhoea asthma pul valve stenosis + tricuspid regurg insidious onset