Mayo Clinic experience with very rare exocrine pancreatic neoplasms

Limited data are available to guide the management of very rare exocrine neoplasms of the pancreas (VREP). Available evidence suggests that VREP have different risk factors and prognoses from those of adenocarcinoma of the pancreas. The primary objectives for this study were to determine the survival, comorbidities, and response to treatment of patients seen at Mayo Clinic with VREP.

We reviewed patients from 1975 to 2005 who had VREP and compared them to patients with adenocarcinomas that were matched for TNM, grade, and decade of treatment.

Sixty-six patients with VREP were identified. The most commonly identified neoplasms were acinar cell carcinoma (n = 15), small cell carcinoma (n = 12), and squamous cell carcinoma (n = 8). Abdominal discomfort and jaundice were the most common presenting symptoms. The median overall survival for patients with VREP, 10.4 months (range, 3.7-23 months), was better than that for matched controls, 8.2 months (range, 4-15.4 months) (P = 0.01). There was no difference in the survival of patients with stage 4 disease between cases, 8 months (range, 2.3-21.8 months), and controls, 6.7 months (range, 2.3-10.8 months) (P = 0.17).

We present one of the largest series of VREP to date. The overall survival of all patients with VREP was better than matched controls, but no statistical difference was seen between the groups with stage 4 disease.