Pancreatic Involvement by Mixed Serous Neuroendocrine Neoplasm Detected by Endosonography With Fine-Needle Tissue Acquisition in Von Hippel-Lindau Disease

The Von Hippel-Lindau disease (VHL) is an autosomal dominant condition characterized by multiple cystic tumors in several organs, including the pancreas. The symptoms are variable, and suspicion must be raised with typical lesions, such as a hemangioblastoma of the central nervous system (CNS) or retina, associated with a renal cell carcinoma, a pheochromocytoma or multiple pancreatic cysts, besides neuroendocrine tumors (NET). The diagnosis in a patient without a family history should be suspected in case of a hemangioblastoma of the CNS and/or retina, which could also be associated with other lesions, such as pancreatic cysts and NETs. The mixed serous-neuroendocrine neoplasia (MSNN) is a combination of serous cyst neoplasia (SCN) and pancreatic-neuroendocrine tumors (p-NET). There are only a few reports in the literature of this type of neoplasia and the majority are associated with VHL. Based on the distribution pattern of SCN and of p-NETs, it is classified into four subtypes: diffuse, mixed, solitary and coalition. This is an unprecedented report of two cases of VHL, evaluated by endoscopic ultrasound-guided fine needle biopsy (EUS-FNB), which allowed the identification and diagnosis and determined the follow-up of these patients using microhistology (McH) and allowed the differentiation of MSNN, mixed tumors and solitary tumors.