Editing Small bowel lymphoma


== '''Epidemiology''' ==

* Comprise about 20% of primary small bowel malignancies
* One third of GIT lymphomas occur in the small bowel
* In children <10yo, this is the most common intestinal neoplasm

== '''Risk factors''' ==

* Coeliac disease
* Immunodeficiency (e.g. AIDS)

== '''Pathophysiology''' ==

* Primary small bowel lymphoma arises from lymphoid follicles of intestinal submucosa - therefore seen most commonly in areas with lots of lymphoid tissue (ileum and sometimes jejunum)
* Mostly NHL
** Large B cell lymphoma is most common
** Follicular subtype has good prognosis
* Burkitt's lymphoma - near ileocaecal junction - very aggressive
* Malignant lymphomas can involve the small bowel as a manifestation of systemic disease too
* Gross appearance - typically large, >5cm, and may extend beneath the mucosa

== '''Presentation''' ==

* Abdo pain, anorexia, weight loss, diarrhoea, palpable mass
* Fever is uncommon and suggests systemic involvement
* Nearly always bulky lymphadenopathy

== '''Workup:''' ==

* CT Neck/CAP, PET, endoscopic evaluation of whole GIT, LDH, beta-2-microglobulin, bone marrow biopsy

== '''Staging''' ==

== '''Management:''' ==

* Limited evidence because it's so rare. Commonly resection (if low overall disease burden) with adjuvant CTX (likely CHOP or R-CHOP)
* B-cell lymphomas are more chemo-sensitive than T-cell lymphomas
* Asymptomatic - no surgery, often chemo-responsive
* Any symptoms - resection indicated - progression to haemorrhage or perforation portends a dismal prognosis
* T-cell lymphomas are more likely to need resection

== '''Complications''' ==

* More common in T-cell lymphomas
* Obstruction
* Perforation (up to 25% of all patients)

== '''Prognosis''' ==

* 5 year survival 50-60%, dictated by response to systemic therapy rather than success of surgical resection
* 5-year survival seems significantly higher in patients who got CTX than those who did not

[[Category:Small bowel]]