pathology 2 Flashcards
which condition causes:
-systolic murmur loudest at the back below left scapula
-rib notching on CXR
-radiofemoral delay
-can have syncope/ claudication
Aortic coarctation
Why do you see rib notching on CXR of aortic coarctation?
The intercostal vessels act as collaterals and are dilation
At which site is aortic coarctation most common
Site of ductus arteriosus
Groups likely to get aortic coarctation
Boys
Girls w/ turners
Mx for aortic coarctation
-angioplasty
-surgical resection
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)
what is the gene and chromosome mutation in MEN 1
MENIN gene
Chr 11
what are the tumours found in patients with MEN 2A
-Phaeochromocytoma
-Medullary thyroid carcinoma
-Hyperparathyroidism
what are the tumours found in patients with MEN 2b
-Phaeochromocytoma
-Medullary thyroid
-hyperparathyroid
+
marfanoid features
+
mucosal neuromas
what is the gene and chromosome mutation in MEN 2a/b
RET-oncogene
Chr 10
which cells of the thyroid produce the hormone that reduces calcium levels
Parafollicular cells/ C-cells produce calcitonin
what is the inhertience pattern for MEN
Autosomal dominant
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
T/F gardners syndrome is a variant of FAP
True
What are the gene/ chromosome affected in Gardners syndrome
APC gene
Chr 5
What is the inheritence pattern for Gardners?
Autosomal dominant
What are the features of Lynch syndrome
Colonic + endometrial cancer
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
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
