Hereditary Non-Polyposis Colorectal Cancer (HNPCC) Criteria

Questions and Answers

What is the percentage of risk of cancer in ulcerative colitis at 30 years from diagnosis?

10%

15%

25%

20% (correct)

What is the minimum age of patients in which Amsterdam II criteria apply?

No age restriction (correct)

50 years

30 years

40 years

What is the presentation of left-sided colonic tumors?

With iron deficiency anaemia

With vague upper abdominal symptoms

With a change in bowel habit or rectal bleeding (correct)

With intestinal obstruction or peritonitis

What is the percentage of cases that present as an emergency with intestinal obstruction or peritonitis?

20% Signup and view all the answers

What is the age range in which this type of cancer is most common?

Eighth decade of life Signup and view all the answers

What is necessary for Amsterdam II criteria to be met?

Three or more family members with an HNPCC-related cancer Signup and view all the answers

What is the primary therapy for non-metastatic colon cancer?

Surgery Signup and view all the answers

What is the aim of surgery in colon cancer?

To excise the primary lesion with a 5 cm margin Signup and view all the answers

What percentage of patients with colonic cancer will present as an emergency?

20% Signup and view all the answers

What is the Tis stage in the TNM classification?

Carcinoma in situ: intraepithelial or invasion of lamina propria Signup and view all the answers

What is the Stage C2 in the TNM classification?

Penetrating through muscularis propria. Nodes involved Signup and view all the answers

What is the most important determinant of prognosis for colorectal cancer?

Tumour stage and lymph node status Signup and view all the answers

What is the approximate five-year survival rate for colorectal cancer?

50% Signup and view all the answers

What is the surgical procedure for Familial Adenomatous Polyposis (FAP)?

Total proctocolectomy and IPAA Signup and view all the answers

What is the main objective of surgery in colon cancer?

To excise the primary lesion with adequate margin and reconstitute bowel continuity Signup and view all the answers

What is the purpose of follow-up for patients with colorectal cancer?

To identify synchronous bowel tumours Signup and view all the answers

What surgical procedure may be advisable for a right-sided lesion with perforation and substantial contamination?

Ileo/colostomy Signup and view all the answers

What is the likelihood of cure in colon cancer?

Greater when disease is detected at early stage Signup and view all the answers

What chemotherapy combination is often used in the treatment of colorectal cancer?

FOLFOX Signup and view all the answers

What is the five-year survival rate for a T1N0 colorectal cancer?

97% Signup and view all the answers

What is the approximate length of the rectum?

15 cm Signup and view all the answers

What is the main route of lymphatic drainage of the rectum?

Upwards along the superior rectal vessels to the para-aortic nodes Signup and view all the answers

What is the most common histological type of rectal polyps?

Adenomas Signup and view all the answers

What is the purpose of colonoscopy in patients with rectal polyps?

To determine whether further polyps are present Signup and view all the answers

What is the purpose of CT of the chest and abdomen in rectal cancer?

To exclude distant metastases Signup and view all the answers

What is the accuracy of transrectal ultrasound (EUS) in tumor staging?

80-95% Signup and view all the answers

What is the limitation of transrectal ultrasound (EUS) in assessing rectal cancer?

It cannot assess the upper rectum Signup and view all the answers

What is the clinical importance of determining extension of disease into the anal canal in rectal cancer?

It affects the planning of sphincter-preserving surgery Signup and view all the answers

What is the primary goal of radical excision of the rectum?

To remove the mesorectum and associated lymph nodes Signup and view all the answers

What is usually possible for tumours whose lower margin is 2 cm above the anal canal?

Sphincter-saving operation (anterior resection) Signup and view all the answers

What is the administration of a course of neoadjuvant chemoradiotherapy intended to do?

Increase the chance of curative surgery Signup and view all the answers

What is necessary before treatment can be planned for rectal cancer?

Fitness of the patient and extent of spread Signup and view all the answers

What type of resection is usually performed for tumours whose lower margin is close to the anal canal?

Abdominoperineal Resection (APR) Signup and view all the answers

What is considered for patients who are unfit for radical surgery?

Local procedure such as transanal excision Signup and view all the answers

Study Notes

Amsterdam II Criteria for HNPCC

Three or more family members with an HNPCC-related cancer (colorectal, endometrial, small bowel, ureter, renal pelvis), one of whom is a first-degree relative of the other two
Two successive affected generations
At least one colorectal cancer diagnosed before the age of 50 years
FAP excluded
Tumours verified by pathological examination

IBD and Cancer Risk

The risk of cancer in ulcerative colitis increases with duration of disease
At ten years from diagnosis, the risk is around 1%
The risk increases to 10-15% at 20 years and may be as high as 20% at 30 years

Clinical Features of Colonic Cancer

Occurs in patients over 50 years of age and is most common in the eighth decade of life
20% of cases present as an emergency with intestinal obstruction or peritonitis
Left-sided colonic tumors usually present with a change in bowel habit or rectal bleeding
More proximal lesions typically present later with iron deficiency anaemia or a mass
Patients may present for the first time with metastatic disease

Staging of Colonic Cancer

Stage B2: Penetrating through muscularis propria; nodes not involved
Stage C1: Extending into muscularis propria but not penetrating through it. Nodes involved
Stage C2: Penetrating through muscularis propria. Nodes involved
Stage D: Distant metastatic spread

TNM Classification

TX: Primary tumor cannot be assessed
T0: No evidence of primary tumor
Tis: Carcinoma in situ: intraepithelial or invasion of lamina propria
T1: Tumor invades submucosa
T2: Tumor invades muscularis propria
T3: Tumor invades through the muscularis propria into pericolorectal tissues
T4a: Tumor penetrates to the surface of the visceral peritoneum
T4b: Tumor directly invades or is adherent to other organs or structures

Surgery for Colonic Cancer

Surgery is the gold standard and principal therapy of primary and non-metastatic ca colon
Curative, palliative, and accurate disease staging
Goal is to excise the primary lesion with adequate margin (~5 cm of normal bowel proximal and distal to the tumor)
Reconstitute bowel continuity

Emergency Surgery

20% of patients with colonic cancer will present as an emergency, the majority with obstruction, but occasionally with haemorrhage or perforation
Right hemicolectomy and anastomosis can be performed for right-sided lesions
Left-sided lesions may require a Hartmann’s procedure or resection and anastomosis

Stage and Prognosis

The most important determinant of prognosis is tumor stage and, in particular, lymph node status
Overall five-year survival for colorectal cancer is approximately 50%

Chemotherapy

5Fu, Leucovorin, Oxaliplatin, Irinotecan, Bevacizumab, and Cetuximab are used in various combinations
FOLFOX, FOLFIRI, and Capecitabine are common regimens

Rectum and Rectal Cancer

The rectum measures approximately 15 cm in length
Divided into lower, middle, and upper thirds
Blood supply consists of superior, middle, and inferior rectal vessels
Lymphatic drainage follows the blood supply, with the principal route being upwards along the superior rectal vessels to the para-aortic nodes

Symptoms of Rectal Disease

Bleeding per rectum
Altered bowel habit
Mucus discharge
Tenesmus
Prolapse

Rectal Polyps

Either single or multiple
Adenomas are the most frequent histological type
Villous adenomas may be extensive and undergo malignant changes
All adenomas must be removed to avoid carcinomatous change
All patients must undergo colonoscopy to determine whether further polyps are present

Workup

CT of the chest and abdomen to exclude distant metastases
PET scanning can be helpful in identifying metastases if imaging is otherwise equivocal
Endoluminal ultrasound, performed using a probe placed in the rectal lumen, can be used to assess the local spread of the tumor
MRI and Transrectal ultrasound (EUS) are used for clinical staging and to demonstrate layers of rectal wall

Management of Rectal Cancer

Management has become increasingly complex due to various surgical, neoadjuvant, and adjuvant options
Fitness of the patient and extent of spread must be assessed
Radical excision of the rectum, together with the mesorectum and associated lymph nodes, should be the aim in most cases
Neoadjuvant chemoradiotherapy can reduce tumor size and make curative surgery possible
Local procedures such as transanal excision, laser destruction, or interstitial radiation should be considered for unfit patients or those with early tumors

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Description

This quiz covers the Amsterdam II criteria for Hereditary Non-Polyposis Colorectal Cancer (HNPCC) and the risk of cancer in ulcerative colitis.