Important cancers Flashcards
Inheritance pattern of MEN
autosomal dominant
What glands are implicated in MEN and what adaptation do they undergo
Hyperplasia of several endocrine glands
List the 3 variants of MEN
Men I
Men II or IIa
Men IIb or III
What glands are implicated in MEN I
Parathyroid
Pancreatic
Pituitary
What glands are implicated in MEN II
Pheo/ andreal medullary hyperplasia
Parathyroid hyperplasia
Thyroid - Medullar cancer and C - cell hyperplasia
What are the metabolic problems associated with MEN (5)
Zollinger-Ellison syndrome, hyperinsulinism, pheochromocytoma, Cushing’s syndrome,
hyperparathyroidism
What glands are implicated in MEN IIb or III
Pheo
Medullay thyroid hyperplasia
Medullar Ca and C cell hyperplasia of the thyroid
Mucosal neruomas
Marafanoid features
What life threatening cancers are associated with MEN
Medullary thyroid
Pancreatic
2 life threatening sequelae of MEN
- Hypertension
- Relentless peptic ulcer syndrome
Testing for MEN (what to use and what not to use
To use
- Urinary catecholamine estimation
- Plasma calcitonin
Not
- Plasma calcium estimation
- Not serum ionized calcium/phosphate ratio
What cells is medullary carcinoma of the thyroid derived from, what do they secrete
Parafollicular cells or C cells
Secrete calcitonin
- may also secrete serotonin and prostaglandin
- sometimes carcinoid, rarely ACTH
Are MEN genetic
arise sporadically 80% of cases, the other 20% from genetic associations (MEN IIa & b)
What gene is implicated in medullary thyroid carcinoma (when due to MEN)
germ line mutations in the RET proto-oncogenes
What is the stroma of medullar carcinoma of the thyroid rich in
Amyloid
Medullary thyroid carcinoma slow or fast growing
slow
Biochemically what will people with medullary thyroid carcinoma have or not have
Will have high circulating calcitonin
WONT have
- Hypocalcemia
- Hypercalcemia
- Hypothyroidism
What is the most common thyroid cancer in adults and children
Papillary carcinoma of the thyroid
What is papillary thyroid carcinoma strongly assocaited with
Radiation - especially in childhood
How do people with papillary thyroid cancer often present
With nodal metases (50% at time of diagnosis)
What is the prognosis of papillary thyroid cancer
90% in 20 yrs > the best survival with thyroid neoplasia
What dystrophic calcification is papillary thyroid cancer a/w
pasmmoma bodies
What is the growth pattern of papillary thyroid cancer
papillary (often plus follicular) growth pattern > but co-existing with follicular DOES NOT worsen prognosis
What hormone is papillary thyroid cancer dependent on and what implications does this have
TSH dependent > implications for therapy, relapse during pregnancy, etc…
Where do carcinoid tumours arise from (structurally and what type of cell)
GIT (including pancreas),
Also bronchi
Thymus
Occasionally ovary
Neuroendocrine cells
When are carcinoid tumours often found
Incidentally at surgery or autopsy
What type of carcinoid tumours rarely metastaise
appendiceal, rectal carcinoids infrequently metastasize
What carcinoid tumours are frequently aggressive
ileal, gastric and colonic carcinoids are frequently aggressive
What is the main hormone released in carcinoid syndrome and what cancers is it associated with
Serotonin
- Hepatic mets
What are the 4 vasoactive substances that can be released in carcinoid syndrome + other rare hormones
- serotonin
- bradykinin & kallikreins
- prostaglandin
- histamine
ACTH or gastrin
Symptoms of carcinoid syndrome
- cramping abdominal pains
- facial flushes
- tachycardia
- pulmonary stenosis
- tricuspid regurgitation
When is gastric cancer defined as early
When it is confined to the mucosa and submucosa
Post resection survival of gastric cancer
90-95% if confined to mucosa/sub mucosa
Risk factors for gastric cancer
diet
smoking
H. pylori infection
autoimmune gastritis
partial gastrectomy
Final common pathway for gastric cancer
dysplasia of the gastric mucosa
Locations of gastric cancer and their frequencies
50-60% at pylorus & antrum 25% cardia
15-25% body and fundus
Name the cancer that is gastric in origin and mets to the ovaries and the cell associated
Krukenberg (signet ring cell)
What type of cancer causes rigid thickened stomach wall
Linitis plastica
What type of polyps are benign
hyperplastic
juvenile
inflammatory
Peutz-Jeghers polyps
What types of polyps are neoplastic
adenomas
(tubular, villous and mixed tubulovillous)
Chance of an adenoma <1cm progressing to invasive cancer
1%
Chance of an adenoma 1-2cm progressing to invasive cancer
10%
Chance of an adenoma >2cm progressing to invasive cancer
45%
What 3 factors are implicated in the association between polyp and cancer
- Polyp size
- Relative proportion of villous component
- Degree of cell atypia (dysplasia) in the neoplastic cells
Peak incidence of colorecal carcinoma
60-70
Proportion of colorectal cancers located in rectosigmoid
<25%
Do most colorectal carcinomas occur in people with FAP or UC
No most occur in their absence
Is carcinoma insitu considered a malignant lesion and why/why not
- No
- Has not yet acquired attributes that will cause mets
- because lymphatic channels are largely absent from colonic mucosa, intra-mucosal carcinoma is regarded as having little or no metastatic potential
- invasion into submucosa indicates adenocarinoma which has now acquired metastatic potential > real in all case
What histological evidence suggests benign colonic epithelial neoplasia
- adenoma with carcinoma in situ
- adenoma showing severe epithelial dysplasia with focal intramucosal carcinoma
Why is Gardner’s syndrome is a clinically more sinister variant of FPC
Aggressive neoplasms, other than colonic carcinoma, may determine mortality in Gardner’s syndrome
What is Gardners syndrome (what other things does it exhibit)
- An autosomal dominant familial polyposis variant
- It is an inherited disease that is characterised by gastrointestinal polyps, multiple osteomas (benign bone tumours), and various skin and soft tissue tumours.
- epidermal cysts, fibromatosis, abnormal dentition, higher frequency of duodenal and thyroid cancer
What is Gardners syndrome characterised by
lots of adenomatous polyps (minimu 100) in colon & elsewhere in the gut ! risk of becoming adenocarinoma 100%
How do you mitigate colon cancer risk in Gardners syndrome
Prophylactic colectomy
Average age of onset of Gardners syndrome and when does progression to cancer occur without surgery
- Teens to 20’s
- in 10-15 years
Tumour marker for Gardeners syndrome
CEA
What side of the colon does Gardners cause cancer
Right
What is Turcots associated with
FPC and CNS tumours
What cancers/ gastric disorders are implicated by dietary factors
FPC
UC
What Genes are associated with Gardeners and which isnt
HNPCC
- NOT APC, DNA, K-ras, not DCC, or p53
Most common form of renal cancer
RCC
Most important risk factor for RCC
Smoking
When does RCC present
50-60
Is RCC more common in men or women
Men
Is RCC genetic
Most sporadic
4% can be rare autosomal dominant forms
Does RCC present early or late
Normally presents late with mets, sometimes they are solitary and stay so
Clinical manifestations/complications of RCC
- amyloidosis
- polycythemia
- hypercalcemia
- fever & cachexia
Paraneoplastic syndrome associated with RCC (6)
- polycythemia (EPO pure RBC blood volume increase)
- hypercalcemia
- hypertension
- masculinisation or feminization
- Cushing’ s syndrome
- Amyloidosis - AA type
Common site of spread for RCC
renal vein extension very common > haematogenous metastases are common
Describe the gross appearance of RCC, and what type of cell does it have lots of
- basic yellow-grey color, but variegated with haemorrhage, necrosis, cystic change, etc.
- High lipid content
Describe the gross appearance of Wilms tumour
Wilm’s tuour is usually of very uniform cut surface in contrast to the
variegated colours and consistency of RCC
Peak age of cervical cancer
40-45
Risk factors for cervical cancer
early of first intercourse
multiple sex partners
high risk male sex partners
married women
Main HPV types associated with cervical cancer
16, 18, 31, 33
Peak incidence of CIN III
~ 30 years
% of cervical cancer associated with HPV
85%
2 Main groups of testicular cancer
- germ cell tumours (95% of cases)
- nongerminal tumours (stromal or sex cord tumours)
Give 2 examples of germ cell testicular tumours
seminoma & teratoma are both germ cell tumours