Franziska Lettau1*, Alexander Siebenhüner2, Bart Vrugt3 and Walter Weder4
1Departement of Anaesthesiology and Intensive Care, University Hospital of Zurich, Switzerland
2Departement of Oncology, University Hospital of Zurich, Switzerland
3Departement of Pathology and Molecular Pathology, University Hospital of Zurich, Switzerland
4Departement ofThoracic Surgery, University Hospital of Zurich, Switzerland
Background: Malignant pancreatic tumors usually present exocrine or endocrine differentiation, with pancreatic ductal adenocarcinoma making up for 90% of pancreatic malignancies. In contrast, both acinar cell carcinomas and neuroendocrine tumors only make up for about 2% to 5% in pancreatic neoplasms. Mixed Acinar Neuroendocrine Carcinoma (MANEC) of the pancreas is extremely rare and only a few cases have been reported so far. The diagnosis is correlated with a poor prognosis and short overall-survival rate. We herein describe a case of such a carcinoma with multiple pulmonary metastases. Case
Presentation: In 2011, a 28-year-old male patient presented with abdominal pain and ikterus in a local hospital. Physical examination and imaging revealed a pancreatic mass and multiple suspiciously enlarged locoregional lymph nodes. Biopsy confirmed a mixed acinar-neuroendocrine carcinoma of the pancreas, previously termed as MANEC and now, recently established by the World Health Organization (WHO), as Neuroendocrine-Non Neuroendocrine Neoplasm (MiNEN). He underwent neoadjuvant chemotherapy (1st line: Oxaliplatin/Streptozocin/5-Fluoruracil; 2nd line: Cisplatin/Etoposid) before an extended total pancreatectomy and lymph node resection was performed, followed by adjuvant chemotherapy (Cisplatin/Gemcitabine). The patient was declared tumor-free in consecutive follow-ups until 2014. After subsequent discovery of multiple and bilateral pulmonary nodules, sequential metastasectomies were performed in 2014 and 2017 with all tissue containing metastases of the mixed acinar-endocrine pancreatic carcinoma. Since then the patient has been recurrence-free.
Conclusion: To our knowledge this the first report of a metastatic MiNEN tumor of the pancreas in a very young patinet with long-term survival. The tumor was treated with a multidisciplinary approach, combining surgery and neoadjuvant as well as adjuvant chemotherapy. So far no consensus exists regarding treatment of MiNENs and how outcome can be influenced most favorably. Survival for more than 7 years after initial diagnosis seems exceptional, still the pathological and immunological dynamics are not fully understood and response to treatment as well as prognosis appear to remain unpredictable.
Lettau F, Siebenhüner A, Vrugt B, Weder W. Mixed NeuroendocrineNon Neuroendocrine Neoplasm of the Pancreas with a Pulmonary Metastasizing Pattern: A Case Report. Ann Surg Case Rep. 2019; 2(1): 1011.