Introduction Goblet cell appendiceal neuroendocrine tumours (GCA-NETs), comprise approximately 6% of appendiceal neuroendocrine tumours (NETs) and share histologic features of both adenocarcinomas and NETs. We are presenting our data from 37 patients, focusing on diagnostic features, prognostic markers, treatment and survival.

Methods A retrospective analysis included 18 male and 19 female patients (mean age: 48.8; range: 19–73 years). Follow-up was complete (mean follow-up: 4.1 years).

Results Although majority of patients (69%) presented with appendicitis, 15.5% had bowel obstruction and 15.5% atypical abdominal pain. 27% had metastases at presentation (one in lungs). Chromogranin-A, CEA and CA-125 were not significantly raised in these patients. Initial treatment was appendicectomy (26 patients) and 24/26 had a subsequent prophylactic right hemicolectomy. Additional hysterectomy and bilateral oophorectomy was performed in six patients and four patients just underwent either single or bilateral oophorectomy. 12% had recurrence and all had Ki67 proliferation index ≥20%. Octreoscan was negative, but FDG-PET was positive in all these patients, and in patients with distal metastases at presentation. FOLFOX chemotherapy was given prophylactically in two patients with local lymph nodes resulting in no evidence of recurrence (Median=8 months) and in two patients with distal metastases resulting in only temporary disease stabilisation. 19% have died with disease and again all had Ki67 ≥20%.

Conclusion GCA-NETs may metastasise to the lungs (first report in literature). Ki67 ≥20% seems to be related with a worse prognosis. FDG-PET is the molecular imaging of choice. No optimal biomarkers or chemotherapy regimens are available to date.

Competing interests None declared.