A long-term survivor of metachronous liver metastases of pancreatic serous cystic neoplasm associated with von Hippel–Lindau disease

• DOI: 10.1186/s40792-021-01239-y
• Publication Date: 2021-06-30T08:02:55Z
• Authors: Takashi Kokumai; Masamichi Mizuma; Katsuya Hirose; Hideaki Karasawa; Masaharu Ishida; Hideo Ohtsuka; Kei Nakagawa; Takanori Morikawa; Takashi Kamei; Atsushi Masamune; Toru Furukawa; Michiaki Unno
• Keywords: Cystadenocarcinoma

Abstract Background Pancreatic serous cystic neoplasm (SCN) is an uncommon exocrine neoplasm, which believed to be a benign entity. However, some of these neoplasms may occasionally attain metastatic ability. Von Hippel–Lindau disease (VHL) manifests dominantly inherited systemic syndrome accompanied by several or malignant tumors, including in various organs. We describe here long-term survival case who underwent surgical resection for metachronous liver metastases pancreatic SCN associated with VHL disease. Case presentation A 35-year-old woman total pancreatectomy and right nephrectomy renal cell carcinoma, respectively. At the 4th year follow-up examination after resection, contrast-enhanced computed tomography (CT) gadolinium ethoxybenzyl diethylenetriamine penta-acetic acid (Gd-EOB-DTPA)-enhanced magnetic resonance imaging (MRI) showed arterially hyper-enhanced neoplastic lesions segment VI VIII liver. Partial resections were performed 53 months initial surgery. 6th month from second surgery, one two tumors located III, VIII, respectively, detected CT Gd-EOB-DTPA-enhanced MRI. Anterior segmentectomy partial III 66 surgery 13 second, The pathologically diagnosed as synonymous cystadenocarcinoma. She remains disease-free without recurrence 6.5 years last operation. Conclusions This first report VHL. Surgical might confer favorable prognosis patients metastases.