Updated 3 October 2007

Lymphoma

See Gastro-intestinal lymphoma information.

Carcinoid

These tumours are dealt with in section 12: neuroendocrine tumours.

Gastrointestinal stromal tumours (GIST) and sarcomas

For c-kit positive GIST, see Section 3.2: Gastrointestinal Stromal Tumours

For c-kit negative GIST and for non-GIST sarcomas, see the Sarcoma Tumour Group guidelines.

Surgical

Radical (Curative) Intent
In four separate series of patients with small bowel adenocarcinoma the complete resection rate ranged from 30-67% with median survivals of 37 to 41 months and 5 year survivals ranging from 30-47%. One series was unable to determine if TN stage had any impact on patient outcomes, likely due to small numbers. The other series demonstrated a statistically significant difference in 5 year survival between T1/T2 tumors versus T3/T4 – 82 versus 58% respectively. Nodal involvement also predicted for a poorer survival with a 5 year survival of 29% versus 58% for those with uninvolved nodes. Similar outcomes were noted in two more recent surgical series, although the data were reported in the same fashion.

Palliative
Patients may undergo palliative resection to alleviate obstruction or active bleeding. Median survival of patients having palliative resection ranges from 10 to 21 months, with one series reporting a 5 year survival of 12%. Another series reported a median survival of 1.75 years for patients undergoing palliative surgery.

Non-surgical - Post Surgical Resection

Adjuvant Chemotherapy
There are no reported randomized clinical trials assessing the potential use of adjuvant chemotherapy for patients with completely resected small bowel carcinoma. Isolated case reports suggest that adjuvant treatment with 5FU containing regimens is provided but no data is available to determine its usefulness.

Palliative Chemotherapy
In large series from the MD Anderson Cancer Center. Of 217 patients reviewed, 48 patients received chemotherapy for locally advanced or metastatic small bowel adenocarcinoma. No response or toxicity data were provided. A phase 2 trial of FAM chemotherapy in 39 patients performed between 1983 and 1985 was recently reported with a response rate of 18% (2 complete and 5 partial). The patient population included those with carcinoma of the ampulla of Vater (4), a site usually excluded in current series of small bowel adenocarcinoma. The report also summarized additional experience from other institutions describing modest responses to various chemotherapy regimens including ECF and single agent irinotecan.

It is likely that any current active regimen used for either large bowel or gastric carcinoma would be a reasonable choice for palliative treatment.