Page 184 - Medicine and Surgery

P1: KOA
BLUK007-04 BLUK007-Kendall May 25, 2005 7:57 Char Count= 0

180 Chapter 4: Gastrointestinal system

Tcell lymphomas form ulcerated plaques or strictures Table 4.5 Features of carcinoid syndrome

in the proximal small bowel. Coeliac disease predis-
System Symptom Frequency (%)
poses toaTcelllymphoma, treatment with gluten free
Skin Flushing 85
diets may reduce the risk.
Telangectasia 25
ABurkitt lymphoma like tumour occurs in children Cyanosis 18

from North Africa and the Middle East. Gastrointestinal Diarrhoea and pain 80
Immunoproliferative small intestine disease (IPSID)
Cardiac Valvular lesions 50
is a condition characterised by plasma cell prolifera- Respiratory Bronchospasm 19
tion in the lamina propria of the small bowel. IPSID
occurs mainly in the Mediterranean and presents with
Macroscopy/microscopy
malabsorption. Transformation into lymphoma may
Carcinoidtumoursappearasayellow–whitemuralnod-
occur.
ule composed of cords and nests of cells with secretory
Perforation, bleeding and obstruction may occur with

granules.
any form of lymphoma.
Investigations
Management Plasma chromogranin A is raised in carcinoid tumours.
Resection is performed where possible, radiotherapy Urinary 5-hydroxyindoleacetic acid (a 5 HT metabolite)
and chemotherapy are often used postoperatively, al- is useful for carcinoid tumours of the midgut. Staging
though their efficacy is uncertain. Inoperable disease is investigations include octreotide scanning, liver ultra-
treated by combination chemotherapy; radiotherapy is sound, spiral CT scanning, MRI scanning and endo-
usedforpalliation.IPSIDnotcomplicatedbylymphoma scopic ultrasound.
is treated with antibiotics.
Management
Resection of the primary tumour (e.g. by appendicec-
Carcinoid tumours of the intestine tomy)isperformed where possible. Octreotide (somato-
statin analogue) relieves diarrhoea and flushing and
Definition may reduce tumour growth. Other treatments include
Tumours originating from neuroendocrine or APUD chemotherapy, embolisation and thermal ablation.
(amine precursor uptake and decarboxylation) cells of
the intestine.
Large bowel neoplastic polyps
Definition
Aetiology/pathophysiology
Apolyp is defined as a tumour attached by a stalk to the
Carcinoid tumours most commonly occur in the ap-
surface from which they arise. Neoplastic polyps of the
pendix,terminalileumandrectum.Smallbowel,colonic
colon are also known as adenomas.
and stomach lesions grow slowly and metastasise to
the liver. Rectal and appendix carcinoids almost never
Incidence
metastasise.
Affects 5–10% of the population in the Western world.
Clinical features Age
Most lesions are asymptomatic although appendix car- Sporadic cases increase with age.
cinoids may present with acute appendicitis. Carcinoid
syndrome occurs in 5% with liver metastases, the fea- Aetiology/pathophysiology
tures of which (see Table 4.5) depend on the hormones Polyps may be solitary (sporadic) or multiple, e.g. fa-
produced. milial adenomatous polyposis. Neoplastic polyps may