Though outcomes for patients with recurrent/refractory malignant germ cell tumors (mGCTs) are poor, therapies targeting mTOR and EGFR inhibition have shown promise in vitro. We hypothesized that the combination of sirolimus and erlotinib will show activity in patients with recurrent/refractory mGCTs. Patients were enrolled in a prospective phase II clinical trial; central review of existing pathology specimens was performed. Of the five patients evaluated, two had their diagnoses revised to pancreatic acinar cell carcinoma and alpha-fetoprotein (AFP)-secreting gastric adenocarcinoma, respectively. Although mGCTs are common AFP-secreting neoplasms, recurrence or refractoriness to standard regimens should prompt histologic reevaluation for other diagnoses.

Given the rare incidence of pancreatic acinar cell carcinoma (PACC), its post-resection clinical outcomes remain unclear. Treatment strategies for PACC have relied on those of pancreatic ductal adenocarcinoma (PDAC). The present study retrospectively investigated clinicopathologic characteristics of resected PACC registered in the Korea Tumor Registry System Biliary Pancreas database. Among 59 patients with a mean age of 59.2 years and a male predominance (83.1%), 43, 5, 7, and 4 had pure PACC, ductal differentiations, mixed neuroendocrine carcinomas, and intraductal and papillary variants, respectively. The mean tumor size was 4.6 cm, consisting of eight at T1, 26 at T2, and 25 at T3 stages. Metastasis to regional lymph node was identified in 15 (25.4%) patients. Thirty-one (52.5%) patients received adjuvant therapy. Five-year survival rate was 57.4%. The median survival was 78.8 months. In survival comparison according to the stage with AJCC system, N stage (lymph node metastasis), but not T stage, showed significant differences (p = 0.027). Resected PACC appeared to have clinical outcomes distinct from those of PDAC in this nationwide study. Therefore, large-scale multinational studies are needed to overcome the rarity of PACC and to establish an appropriate treatment strategies and staging system.