Published online Dec 14, 2013. doi: 10.3748/wjg.v19.i46.8687
Revised: August 25, 2013
Accepted: September 3, 2013
Published online: December 14, 2013
Processing time: 183 Days and 16.9 Hours
AIM: To describe disease characteristics and treatment modalities in a group of rare patients with metastatic gastric carcinoid type 1 (GCA1).
METHODS: Information on clinical, biochemical, radiological, histopathological findings, the extent of the disease, as well as the use of different therapeutic modalities and the long-term outcome were recorded. Patients’ data were assessed at presentation, and thereafter at 6 to 12 monthly intervals both clinically and biochemically, but also endoscopically and histopathologically. Patients were evaluated for the presence of specific symptoms; the presence of autoimmune disorders and the presence of other gastrointestinal malignancies in other family members were also recorded. The evaluation of response to treatment was defined using established WHO criteria.
RESULTS: We studied twenty consecutive patients with a mean age of 55.1 years. The mean follow-up period was 83 mo. Twelve patients had regional lymph node metastases and 8 patients had liver metastases. The primary tumor mean diameter was 20.13 ± 10.83 mm (mean ± SD). The mean Ki-67 index was 6.8% ± 11.2%. All but one patient underwent endoscopic or surgical excision of the tumor. The disease was stable in all but 3 patients who had progressive liver disease. All patients remained alive during the follow-up period.
CONCLUSION: Metastatic GCA1 carries a good overall prognosis, being related to a tumor size of ≥ 1 cm, an elevated Ki-67 index and high serum gastrin levels.
Core tip: Metastatic gastric carcinoid type 1 (GCA1) are extremely rare and there is no data regarding their natural history, treatment and prognosis. Based on our study, metastatic GCA1 carries a good overall prognosis. Metastatic spread appears to be related to a tumor size of ≥ 1 cm, an elevated Ki-67 index, and to high serum gastrin levels. Endoscopic surveillance is extremely important for follow-up. Surgical resection should be performed only in patients in whom total tumor excision is expected. Although still controversial, somatostatin analogues could be considered as first line treatment to lower the elevated gastrin levels and suppress enterochromaffin like cell hyperplasia.