Multiple neuroendocrine tumors of the pancreas associated with pancreas divisum

• Published in: European journal of endocrinology Vol. 150; no. 6; pp. 837 - 840
• Main Authors: Raffel, A, Engers, R, Cupisti, K, Krausch, M, Kreuz, H, Schulte, KM
• Format: Journal Article
• Language: English
• Published: Colchester European Society of Endocrinology 01-06-2004; Portland Press
• Subjects: Biological and medical sciences; Biopsy, Needle; Carcinoma, Neuroendocrine - complications; Carcinoma, Neuroendocrine - diagnosis; Carcinoma, Neuroendocrine - surgery; Case Reports; Endocrinopathies; Fundamental and applied biological sciences. Psychology; Humans; Male; Medical sciences; Middle Aged; Pancreas - abnormalities; Pancreas - pathology; Pancreatectomy; Pancreatic Neoplasms - complications; Pancreatic Neoplasms - diagnosis; Pancreatic Neoplasms - surgery; Tomography, X-Ray Computed; Vertebrates: endocrinology; Multiple; Pancreas divisum; Malformation; Pancreas tumor; Digestive diseases; Neuroendocrine tumor; Congenital disease; Endocrinology; Pancreatic disease
• ISSN: 0804-4643; 1479-683X
• DOI: 10.1530/eje.0.1500837

Pancreas divisum is the most common congenital anomaly of the pancreas, characterized by missing fusion of the ventral and dorsal pancreatic duct. It may cause pancreatitis, but is rarely associated with malignancy.We report herein for the first time the rare association, in a symptomless patient, of multiple neuroendocrine tumors of the pancreas with pancreas divisum and a failure of the exocrine system. Diagnosis was made incidentally by routine abdominal ultrasound. Laboratory examinations and a fine-needle aspiration revealed the neuroendocrine nature of the tumor. Spleen-preserving left pancreas resection was performed, with evidence of multiple neuroendocrine tumors of the pancreas with the typical histological characteristics. Eighteen months later the patient is still free of tumor burden.