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143 Cards in this Set

  • Front
  • Back
Most common primary tumor of respiratory system?
Bronchogenic cancer
Most common tumors that metastasize to lungs?
- Sarcomas (osteo-)
- Colon
- Prostate
- Wilm's
- Bladder
- Breast
- Kidney
- Neuroblastoma
List the most typical benign tumors of upper respiratory tract?
- Single or multiple papillomas
- Hemangiomas = Nosebleeds
- Angiofibroma (in childhood - regress)
Most typical malignant tumors of upper respiratory tract?
- Bronchioalveolar cancer
- Bronchial carcinoid
- Hamarthomas
- Tumors of nasopharynx
- Tumors of larynx
What is bronchioalveolar cancer?
Adenocarcinoma of columnar mucosal producing cells, without any destruction of alveolar walls, but patient cough up a lot of mucus

President Havel had this condition, good prognosis:)
What is carcinoid?
Where do they arise?
How are they classified?
What syndrome may they produce?
Malignant tumor composed of neuroendocrine cells.

The carcinoids can arise in both respiratory tract and GI tract.

They are divided into:
1. Typical carcinoids = low grade
2. Atypical carcinoids = intermediate grade (higher mitotic rate)



May produce carcinoid syndrome and sometimes they are part of MENS
What is bronchial carcinoid?
How is the growth patterns?
A bronchial carcinoid of so-called Kulchitsko neuroendocrine cells

Grow either as:
1. Obstructing polypoid of bronchial lumen
2. A mucosal plaque penetrating bronchial wall out in the peribronchial tissue = Collar-button lesion
What is the spectrum of increasing agressivity of neuroendocrine tumors of lung - measured in survival over 10 years?
1. Typical carcinoids - 85%
2. Atypical carcinoids - 50%
3. SCLC - 5%!
What is the location of the most typical hamarthoma in the body?
Lung
Liver bile ducts
What is a hamarthoma?
A mass of disorganized tissue, native to that place. It is often seen as the middle point between developmental malformation and a neoplasm
Characteristics of a lung hamarthoma?
It is a nodule composed of islands of cartilage, bronchial epithelium and blood vessels
What are the most common laryngeal tumors?
1. Vocal cord nodules
2. Papillomas
3. Squamous cell neoplasms
Tell me about the laryngeal vocal cord nodules?

What are they?
What are they composed of?
What are they associated with?
How are they discovered?
Where are typical locations?
They are smooth protrusions, usually arising on the vocal cords.

Composed of fibrous tissue, covered by stratified squamous mucosa, often ulcerated due to mechanical trauma of the other vocal cord

Associated with heavy smokers or singers (thus Singer's nodes) - suggesting chronic irritation (edema)

Discovered by hoarseness

Locations are commonest on true vocal cords
Tell me about laryngeal papillomas? :)

What are they and what are their composition?
What is the difference between adult and children?
They are benign finger-like neoplasms usually growing on true vocal cords

Composed of supporting fibrovascular core, with usually stratified squamous epithelium - which may ulcerate and cause hemoptysis

Adults
- Usually solitary

Children
- Often multiple, due to RRP (recurrent respiratory papillomatosis), caused by HPV 6 and 11. But now with vaccination of mother things are better
Laryngeal carcinoma

- What type is most common?
- Where do they grow?
- What are they associated with?
- Any gender predominate?
- What age?
- How is their grading?
- How discovered?
1. Squamous cell carcinoma, often keratinizing, is the most common (95%) - less common is the adenocarcinoma

2. They most commonly grow on the true vocal cords (glottic) - where they are early discovered, but may grow supraglottic and subglottic where there are much worse prognosis since they are not discovered

3. Associated with Smoking, alcohol and asbestos exposure

4. Logically, men predominate quite lot - and usually it arises not before age of 40y.

5. Grading is usually quite nice, well differentiated squamous cell carcinomas - although all grades may be seen

6. Discovered by hoarseness.
What is the definition of lung cancer?
Carcinoma of the bronchus (bronchogenic carcinoma)
What are the symptoms of bronchogenic carcinoma?
Cough, hemoptysis, chest pain
What are the 4 types of lung cancer according to histological picture? Also add the frequency in %
1. Adenocarcinoma (40%)
2. Squamous cell carcinoma (30%)
3. Large cell carcinoma (10%)
(these are NSCLC)

And then
4. Small cell lung cancer (SCLC) / Oat carcinoma
Why is it important to distinguish between NSCLC and SCLC?
Since SCLC is treated by chemotherapy, and has worse prognosis
How does carcinomas of lung usually begin?
1. Small mucosal grey lesions
2. Grow as intraluminal masses - invade bronchial mucosa
3. May undergo central necrosis and cavitation if it grows too fast
Location and pathogenesis of squamous cell carcinoma of lungs?

What is usually the risk factor?
Location
- Usually centrally in major bronchi

Pathogenesis
1. Squamous metaplasia or dysplasia of bronchial epithelium
2. Carcinoma in situ - may last for years
3. Slowly growing and invading local tissues

Risk factor is smoking
Location and pathogenesis of adenocarcinoma?

What are the different types?
What people is it more common in?
What is the most typical growth pattern?
Location
- More peripherally of the lung, often related with scarring

Pathogenesis
- Grow slowly as smaller masses, metastasize in early stage!

Types of adenocarcinomas
- Acinar (gland forming)
- Papillary
- Mucinous
- Solid types (intracellular mucin production)

More common in females and non-smokers!

Growth pattern
- Growing along the alveolar walls - called 'Lepidic' - sticking to alveoli - like butterflies sitting on a fence :)
What is thought to be the precursor of adenocarcinoma?
Atypical adenomatous hyperplasia (AAH) - which progress to adenocarcinoma in situ (AIS)

(Formerly known as bronchioloalveolar adenocarcinoma)
What is AIS of lung and what is the key features?
AIS = adenocarcinoma in situ

Key features
- 3cm or less diameter
- Growth along pre-existing structures
- Preservation of alveolar architecture
What is large cell carcinoma of lung?
Most likely a extremely poorly differentiated squamous cell or adenocarcinoma.

Otherwise, large cells, large nuclei, prominent nucleoli and quite a lot of cytoplasm
If the lung tumor obstruct the bronchial lumen - what may occur?
1. Collapse as a atelectasis
2. Consolidation with retention pneumonia
What is small cell lung carcinoma? (SCLC) What is it associated with?
What cell does it consist of? How do they look?
Where is typical location?
How is it's growth, agressiveness and metastasis?
It is a carcinoid tumor of late stage, often associated with smoking

Made of neuroendocrine cells, which may or may not have a hormonal production

They look like oats, about 2x the size of resting lymphocytes. Hyperchromatic.

Grow centrally of lungs (close to bronchi)

Grow very agressively, and metastasize quickly. This may often be first symptom: Metastasis to brain.
5 ways of local spreading of lung cancers?
1. Central tumors often invade surrounding lung parenchyma or vessels
2. Peripheral often involve the pleura
3. Hilar lymph nodes are involved, sometime mediastinal
4. Spread to pericardium
5. May compress or involve nerve plexuses in apical tumors
Invasion of large blood vessels may lead to?
Hemoptysis - severe hemorrhage
Pleural involvement may lead to?
Hemorrhagic effusion
Pericardial involvement may lead to?
Pericardial effusion - and eventually involvement of myocardium
What is a Pancoast tumor - and what is it's typical manifestations?
A apical tumor of lung - which involve structures of head and neck - leading to pancoast syndrome

Often destroy 1-2nd ribs

It usually involves
- Brachial plexus --> Sensory and motor symptoms
- Cervical sympathetic chain --> Horney's syndrome
What is Horner's syndrome?
A triad of:
1. Ptosis of eyelid
2. Miosis of eye
3. Ipsilateral enophtalmos
4. Anhydrosis
What happens if lung cancer spread to mediastinum?

Name 3 diseases (1 compression and 2 nerves)
May grow and compress structures like:
- SVC => SVC syndrome
- Recurrent laryngal => Vocal cord paralysis
- Phrenic => Diaphragmatic paralysis
What ways may the lung cancer metastasize, most commonly?
1. Hematogenous via invasion of pulmonary veins
- Liver
- Bone
- Brain
- Adrenal

2. Lymphatic
- Cervical nodes, and typically Virchow's node
It is typical that lung cancer (SCLC) is paraneoplastic. Which hormones or hormone-like substances are most common?

And what do each of them lead to?
1. ACTH
- Adrenal hyperplasia --> Increased cortisol --> Cushing

2. ADH (SIADH)
- Retention of water --> Dilutional hypernatremia

3. PTHrP
- Hypercalcemia (parathyroidhormone related peptide)

4. Serotonin (carcinoid)
What are the 5 developmental stages of lung tumors and their characteristics?
I - Small tumor with no node
II - Lymph node and pleural
III - Parietal pleura + ipsialteral node
IV - Mediastinal organs
V - SVC syndrome
Tell me about malignant mesothelioma?

Risk factors
Symptoms
It's a malignant tumor of pleura

Almost always after some asbestos exposure, it might be only a brief one, and may be latent for 40y without symptoms

The symptoms are dyspnea, chest pain etc...
Mesothelioma
- Grossly? How does it spread?
- Histological? (3 growth patterns)
Grossly
- Begin localized, and spread by continuous growth or seeding
- Lung may be covered by this yellow, gelatinous tumor that obliterate pleural space

Histology = biphasic, so:
- Give rise to pleural lining cells + underlying CT! So it can form 3 patterns:

1. Epithelial with cuboidal cells
2. Sarcomatous with spindle cells
3. Biphasic (both)
Most common benign tumor of oral cavity?
Squamous cell papilloma - although I guess most other mesenchymal and epithelial tumors may arise
What are the precancerous lesions of oral cavity?
- Leukoplakia
- Erythroplakia
- Oral submucosal fibrosis
- Actinic cheilitis
What is leukoplakia?
Epithelial thickening + hyperkeratosis (thickening of stratum corneum, the outermost layer of epidermis)
How is the leukoplakia in EBV infection?
Progressive verrucous leukoplakia (warty lesions)
How is leukoplakia in immunosuppresion?
Oral hairy leukoplakia
What is erythroplakia?
What is it associated with?
Atrophy and dysplasia of tounge

Associated with
- Smoking
- Candidiasis
What is oral submucosal fibrosis?
A complex irreversible precancerous condition with inflammation, and the inflammatory process leads to progressive fibrosis of the submucosal tissues - leading to rigid jaws and eventually unable to open mouth!

Associated with tobacco chewing and several types of nuts :P
What is actinic cheilitis?
Similar to solar keratosis of skin
Squamous cell papilloma of oral cavity - is highly associated with HPV virus.

Tell me about it:
- Grossly
- Koilocytes?
- Low or high risk variants?
- Differential diagnosis from?
Very common benign tumor of oral cavity, with HPV etiology

Grow like cauli-flower projections, with some hyperkeratosis

There are NO koilocytic changes, and might be misdiagnosed for condyloma acuminatum (!) - but this HPV virus is a low risk variant, so it stays benign
What are the typical locations of squamous cell carcinoma of oral cavity?
1. Base of cavity
2. Lower lip
3. Base / lateral of tounge
4. Soft palate
What precancerous lesion may lead to lower lip sq. cell carcinoma?
Actinic cheilitis
What is problematic with squamous cell carcinoma of tounge?
Very bad prognosis, it metastasizes in the deep head and neck lymph nodes!
What is problematic with tonsillar squamous cell carcinoma?
It consume the whole tonsil, and spread metastaticaly to cervical LN, since tonsil is full of lymphatic vessels
Risk factors of oral squamous cell carcinoma?
Alcohol
Tobacco
UV light (lower lip)
HPV 16
HPV 16 is specific for what oral squamous cell carcinoma?
Waldeyer's tonsillar ring carcinoma
Name 4 special types of oral squamous cell carcinomas?
1. Verrucous carcioma = Ackermans tumor
2. Keratoxanthoma
3. Basal cell carcinoma (both cutaneous)
4. Small cell carcinoma (neuroendocrine)
What is the primary etiological factor of nasopharyngeal carcinoma?
EBC
3 types of nasopharyngeal carcinoma?
1. Squamous cell carcinoma
2. Keratinizing carcinoma
3. Lymphoepithelial carcinoma

All may be well or poorly differentiated
Name 2 benign mesenchymal tumors and pseudotumors of nasopharynx?
1. Epulides
2. Granular cell tumor
What are epulides?
Nb! It lays in the word….

3 types!
Epulides = "in the gingiva"

It is benign tumors of the oral gingiva.

The types:
1. Fibrous epulis
2. Giant cell epulis
3. Granuloma fissuratum
What is granuloma fissuratum?
A benign proliferative lesion of oral gingiva due to a poorly fitting tooth
Name all the benign tumors of nasopharynx?
- Epulides
- Granular cell tumors
- Angiofibroma
- Pyogenic granuloma
What is a angiofibroma, in who do it occur and how may a angiofibroma lead to death?
A tumor composed of vessels of connective tissue

Only occur in men

Arise form nasopharynx
1. Destroy surrounding tissue
2. Perforate base of skull
3. Spread to meninges
4. Meningitis & death
What is a pyogenic granuloma?
A vascular lesion, protruding with a botryoid shape, typically on the gums or skin.

Associated with irritation, trauma or hormonal factors like in pregnancy
What is granular cell tumor?
A benign proliferation of cells most likely from Schwann cell or nerve sheath origin - it can arise anywhere on or in the body
What are the most common malignant mesenchymal tumors of nasopharynx?
1. Myofibroblastic sarcoma of tounge
2. Rhabdomyosarcoma
3. Kaposi sarcoma in HIV
4. Angiosarcoma
5. Liposarcoma
What is a myofibroblast?
A cell which is between fibroblast and myocyte in it's differentiation
What are the 2 pseudotumors of salivary glands called?
1. Mucocele
2. Ranula
What is an oral mucocele?
A benign cyst, caused by a swelling of connective tissue filled with mucus, due to a rupture of a salivary gland duct - usually due to local trauma

If the duct is obstructed, we call it a 'mucus retention cyst'
What is a ranula?
It is a type of mucocele - just of the oral cavity - due to a rupture of salivary gland duct

Confined to the floor of oral cavity

Although Dr. Kamaradova said it was a cyst of thyreoglossus duct remnant.
What are the cells of salivary glands - and what does that mean for tumor development?
1. Acinar cells
2. Ducts
- Intercalated cells
- Striated cells
3. Myoepithelial cells
4. Precursor cells

=> Therefore there is extremely many types of salivary tumors!!
What are the most common benign tumors of salivary glands?
1. Pleomorphic adenoma
2. Warthin tumor
3. Myoepithelioma
4. Canalicular and basal cell adenoma
Ok… But what is the most common salivary gland tumor?
Pleomorphic adenoma - and hence the name - there are many different ways it may look
What is pleomorphic adenoma?
Which cell is the precursor?
What is a satellite? Are they dangerous?
A benign tumor, also called 'myxochondroepithelioma' with proliferation of glandular cells and myoepithelial cells - and a common precursor cell!

A satellite, I think, is also a pleomorphic adenoma, but they are found at location outside the salivary gland itself.

But they are not an invasion. Only satellites.
Why is a pleomorphic tumor also called myxochondroepithelioma?
Since it has different types of cells like:
- Epithelium
- Myxoid CT
- Islands of chondroid (resembling cartilage)
What is a Warthin's tumor?

How may it grow?
A adenolymphoma, a entirely benign growth of lymhpoid tissue, without malignant potential

May grow:
- Cystic
- Papillary
What is a canalicular adenoma?
A benign tumor of salivary glands, commonly of upper lip, in older people
What is a basal cell adenoma?
A benign tumor of salivary glands, looking like pleomorphic adenoma, but more closely they resemble 'basaloid' cells - cells resembling the basal layer of epidermis
What are the 3 most common malignant salivary gland tumors?
1. Acinar cell carcinoma
2. Adenoid cyst carcinoma
3. Mucoepidermoid carcinoma
Tell me about acinar cell carcinoma of salivary glands?

What is morphology?
How do we know that they're acinar cells?
Does it metastasize?
1. Has morphology of acinar cells

2. Zymogenic granules are PAS positive

3. Metastasize to lymphnodes or hematogenous (bad prognosis)
What is PAS?
Periodic acid schiff stain

Method used to detect polysaccharides like glycogen, GAG's, glycoproteins, glycolipids etc...
Tell me about mucoepidermoid carcioma? :D

Which glands is most typical?
What 3 types of cells is there?
Occurs in large salivary glands

Cells are
- Mucus secreting cells
- Squamous cells
- Intermediate cells

Good prognosis for low grade, bad prognosis for high grade

May occur at more sites, like lacrimal glands
Tell me about adenoid cystic carcinoma?

What is it also called?

What is it composed of?
Rare type of cancer, at many sites like breast, lungs, lacrimal glands, but often in salivary glands

Also called cylindroma

Composed of
- Ductal cells
- Modified myoepithelium
What are the most common (but extremely uncommon) benign tumors of esophagus?
- Leiomyoma
- Hemangioma
- Squamous cell papilloma
- Granular cell tumors (scwhannoma)
What are the 2 most common malignant tumors of esophagus? With %
Squamous cell carcinoma - 90%
Adenocarcinoma - 10%
Risk factors for squamous cell carcinoma?
Male
Smoking
Alcohol
Bad diet, low fibers
Risk factors for adenocarcinoma?
GERD
Tobacco
Obesity
Complications of esophageal squamous cell carcinoma?
Achalasia
Chronic esophagitis (not BE!)
3 growth patterns of squamous cell carcinoma of esophagus?
1. Polypoid
2. Ulcerating
3. Infiltrative
How is microscopy of squamous cell carcinoma of esophagus?
Poorly differentiated, grade 1-3
Sometimes keratinized
Staging of sq.cell ca of esophagus?

T1
T2
T3
T4

Tell how deep in mucosa it goes on each
T1 - submucosa
T2 - Adventitia
T3 - Subserosa (outside esophagus)
T4 - Infiltration of other structures
Prognosis of esophageal cancers:
1. Squamous cell carcinoma
2. Adenocarcinoma
1. 35% 5y, but only 5% with metastasis

2. 20% 5y
What is the most common underlying cause of adenocarcinoma of esophagus?

What is it?
How does it develop?
What is risk of adenocarcinoma with this?
Barretts esophagus

A interchange of squamous epithelium by functional columnar epithelium with goblet cells: So intestinal metaplasia - and its a 40 times higher risk of adenocarcinoma

Develops in 5 stages:
1. Erosions
3. Erosinos + leukoplakia
5. Barretts esophagus with intestinal metaplasia
What are the most common benign tumors of stomach?
Polyps:
- Hyperplastic polyp
- Fundic gland polyp
- Adenomatous polyps
- Hamartomatous polyp
What is a hyperplastic polyp?
How do you distinguish it from intestinal polyp?
Small nice polyp, 1 cm, with an inflammatory origin

Distinguish it from colonic, since colonic are serrated lesions
What is a fundic polyp, where does it grow and what is it associated with?

What is usually the genetic mutation here?
A polyp growing from oxyntic glands (the parietal cells = oxyntic cells) - which produce HCl.

It is associated with PPI treatment, Zollinger-Ellison syndrome and H.pylori infection

So likely it origins from increased gastrin secretion - but they are luckily benign

Genetic mutation
- In gene coding for E-cadherin
Where do we find oxyntic / parietal cells in stomach?
Body and fundus
What are adenomatous polyps?
Normal adenomas - with some malignant potential if they undergo dysplasia

May be areas of intestinal metaplasia
What are the 3 most common malignant tumors of stomach?
1. Gastric adenocarcinoma (more or less all)
2. GIST
3. Lymphomas
- B-cell MALT type - associated with H.Pylori (can regress with eradication of HP!)
4. Leiomyosarcoma (rare)
What are the most important risk factors for gastric adenocarcinoma?

Malignant neoplasm of the glandular epithelial cells
1. Dietary - spicy foods - japan - wasabi
2. Genetic factors - FAP patients have APC mutation (HPCC) - so increased risk of intestinal type gastric cancer
- Blood group A
What diseases are predisposing for gastric adenocarcinoma?
1. Adenomatous polyp
2. HP gastritis
3. Chronic atrophic gastritis
4. Intestinal metaplasia
5. Duodenal reflux
6. Post gastrectomy
1. What are the most typical growth patterns for gastric adenocarcinoma and where are they located?

2. What are the 2 types of histologies?

3. Where does gastric adenocarcenoma spread to?
1.
- Exophytic - fundus
- Ulcerative - antrum (rolled edged ulcers, biopsy of rolled edge!)
- Diffuse - entire gastric wall is thickened => Linitis plastica

2.
- Intestinal, where malignant cells are arranged in acini through muscle of stomach wall!
- Diffuse - typical in linitis plastica - where they have signet rings (globules of mucin push nucleus to side)

3. Virchow's supraclavicular lymph node
What is Krukenberg tumor?
Gastric adenocarcinoma metastasis to ovaries
What is the most common mesenchymal tumor of stomach?

Tell me about it :D
GIST - gastrointestinal stromal tumor

Origins form the pacemaker cells of the stomach, called Interstitial cells of Cajal

Grows like solitary well circumscribed mass, submucosally - with spindle shaped cells

Cause hemorrhage
What are the most typical neoplasms of small intestine?
1. Mesenchymal sarcomas
2. Lymphomas and MALTomas
3. Neuroendocrine carcinoids
4. Carcinomas = extremely rare
What are the most common benign mesenchymal tumors of small intestine?
- Lipoma
- Leiomyoma
- Schwannoma
- Lymphangioma
- GIST
- MALTomas
Most common malignant mesenchymal tumors of small intestine?
- Leiomyosarcoma
- Lymphomas
- Kaposi sarcoma
What are the most common lymphomas of GIT? Nb! Non-hodgkin lymphomas
1. MALT lymphoma
2. Diffuse large B-cell lymphoma
What is the neuroendocrine carcinoid tumors of GIT?

Where is most commonly located?
What do they produce?
How do they look in LM?
They are tumors of enteroendocrine cells, called Kulchitski tumor

Located mostly in appendix

Producing serotonin, leading to carcinoid syndrome

LM: Pale regular cells...
What is carcinoid syndrome?
Hyperseretoninemia
1. Bronchiconstriction
2. Flush of face
3. GIT hypermobility --> Diarrhea
4. Endocardial fibrosis of right heart - valvular lesions

Nb! Occurs only in high grade carcinoid!

Low grade carcinoid - serotonin is sufficiently broken down by liver
What are the most common benign tumors of appendix?
1. Hyperplastic polyp
2. Sessile serrated lesions / adenomas
Most common intermediate tumors of appendix?
1. Neuroendocrine carcinoids
2. Mucocele of appendix
What is mucocele of appendix?
A appendix filled of mucin, most likely due to
1. Obstructed appendix full of mucin
2. Mucinous cystadenoma or mucinous cystadenocarcinoma
What is pseudomyxoma peritonei - in association with mucocele of appendix?
If it ruptures, like in mucinous cystadenocarcinoma (or adenoma) - it lead to intraperitoneal seeding whole abdomen is filled with semi-solid mucin
What is linitis plastica?
It is the diffuse gastric adenocarcinoma, which undergo so-called desmoplastic changes

So it leads to a hardening of the gastric wall, feeling like thick pargement
What are the most common malignant tumors of appendix?
Adenocarcinomas

Mucinous cystadenocarcinomas
What is the main classification of colonic polyps?
1. Non-neoplastic
2. Neoplastic
What are the types of non-neoplastic polyps and their syndromes?
1. Juvenile polyps = Juvenile polyposis syndrome

2. Peutz-Jegher polyp = Peutz-Jeghers syndrome

3. Hyperplastic polyps

4. Inflammatory polyps
What is a juvenile polyp? How does it look?

Risk of malignancy?

What is a inflammatory polyp?
Juvenile polyp:
A hamartoma
A globular protursion of rectal mucosa, may be ulcerated and inflamed

With a small risk of malignancy

Inflammatory polyp:
Polyps arising in inflammatory bowel diseases, like ulcerative colitis and Chron's disease
1. What is a Peutz-Jegher polyp? How does it look?

2. What is a hyperplastic polyp? How does it look?
1. Peutz-Jegher polyp:
A hamartoma, but more branches like a tree with also branching smooth muscle from muscularis mucoisae

2. Hyperplastic polyp:
A small sessile nodule, which do not become malignant. They may look very similar to serrated adenomas - which have malignant potential like all other adenomas
How do we morphologically divide neoplastic polyps?
1. Conventional adenomas (low & high grade)

2. Serrated lesions
Which are the serrated lesions?
Can be just a hyperplastic polyp (benign) or sessile serrated adenoma
What are the 4 types of polyp shapes?
1. Tubular
2. Tubulovillous
3. Villous (like small intestine)
4. Sessile / serrated
What are the most important polyposis syndromes?
1. FAP (familial adenomatous polyposis)
2. Gardner syndroem
3. Peutz-Jegher syndrome
4. Serrated polyposis (?)
Which gene is mutated in FAP and gardner syndrome?
What is the risk of adenocarcinoma with this mutation?
APC gene - increase the chance of developing polyps - it's a tumor suppressor gene
Gardner syndrome is also called?
Familial colorectal polyposis (FCP)
Gardner syndrome is characterized by multiple polyps in colo-rectum. But it also increases risk of what extracolonic neoplasms?
1. Osteomas of skull
2. Thyroid carcinoma
3. Desmoid tumors (like in NF)
What is a desmoid tumor?
Benign, slow-growing musculoaponeurotic tumors (Desmoid = tendon-like in greek) - without malignant potential.

However, in agressive neurofibromatosis, they can be locally agressive.

Most cases sporadic, but some are associated with FAP, gardner syndrome and agressive neurofibromatosis
What extracolonic events is likely to occur in Peutz-Jegher syndrome?
1. Perioral hyperpigmentation
2. Extraintestinal malignant tumors of e.g. pancreas
What are the most common malignant tumor of colon?
Colonic carcinoma -
How is the difference in colon carcinomas in right and left colon? And how is it connected to size of bowel and time of detection?
Right (ascending colon and caecum)
- Polypoid carcinoma
- Very small which are very difficult to discover (And hard to get to with colonoscope)
- The lumen is wide + liquid feces, so there is no obstruction and symptoms do not present before it's often too late

Left (descending and sigmoid)
- Stenosing carcinoma
- So it quickly obstructs + feces is more solid
- More quicker symptoms
What are the typical symptoms of right colon carcinoma?
1. Blood loss & anemia
2. Abdominal mass
3. Liver metastases

Due to reasons mentioned above
What are the typical signs of left colon carcinoma?
They are obstructing, so changed bowel movements, obstructions and rectal bleedings
What chromosome does the APC gene lie on?
Chromosome 5
So what are the 5 types of colorectal carcinoma - how they may look?
1. Exophytic (polypoid)
2. Ulcerating
3. Obstructing
4. Infiltrative
5. Stenosing

Or combinations!
How do the colorectal carcinoma usually look in LM?
More or less always a adenocarcinoma
In staging - what is T4 characteristics?
Infiltration of bowel wall. In pedunculated polyps - the stalk is always infiltrated first!
What is lynch syndrome? What is it also called?

What other cancers are you in increased risk of?

What is the underlying cause of this increased risk?
Also called hereditary non-polypose colorectal cancer (HNPCC) - a autosomal dominant condition where you have increased risk of colon cancer!

A cancer without polyps!

Other cancers:
- Endometrial (these 2 are most common)
- Stomach, ovary, bladder etc.. (Less common)

Cause
- Mutation in MMR (mismatch repair genes)
What is the 'Amsterdam criteria'?
Diagnostic criteria to identify families with higher chance of Lynch syndrome

1. 3 or more 1st degree relative with colorectal cancer (with FAP excluded)
2. Over 2 successive generations
3. At least one of them before 50 years
What are the most typical mesenchymal tumors of large bowel?
- Leiomyoma
- Schwannoma
- GIST
- Kaposi sarcoma
What are the 2 most common GIT lymphomas occurring?
B-cell/T-cell non-HD lymphomas:
1. MALT lymphoma
2. DLBCL
What is Troisier's sign?
Spread of gastric adenocarcinoma to Virchow's supraclavicular lymph node
Most typical sites of spread of gastric adenocarcinoma?
1. Lymphatics to Virchow's
2. Hematogenous to liver
3. Transcoelomic to ovaries (Krukenberg)
3. Late to lungs
Which blood group is familial more common with gastric carcinoma?
Blood group A