A long-term survivor of metachronous liver metastases of pancreatic serous cystic neoplasm associated with von Hippel–Lindau disease
Abstract Background Pancreatic serous cystic neoplasm (SCN) is an uncommon exocrine neoplasm, which is believed to be a benign entity. However, some of these neoplasms may occasionally attain metastatic ability. Von Hippel–Lindau disease (VHL) manifests a dominantly inherited systemic syndrome accom...
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| Format: | Article |
| Language: | English |
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SpringerOpen
2021-06-01
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| Series: | Surgical Case Reports |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s40792-021-01239-y |
| _version_ | 1818724105382264832 |
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| author | Takashi Kokumai Masamichi Mizuma Katsuya Hirose Hideaki Karasawa Masaharu Ishida Hideo Ohtsuka Kei Nakagawa Takanori Morikawa Takashi Kamei Atsushi Masamune Toru Furukawa Michiaki Unno |
| author_facet | Takashi Kokumai Masamichi Mizuma Katsuya Hirose Hideaki Karasawa Masaharu Ishida Hideo Ohtsuka Kei Nakagawa Takanori Morikawa Takashi Kamei Atsushi Masamune Toru Furukawa Michiaki Unno |
| author_sort | Takashi Kokumai |
| collection | DOAJ |
| description | Abstract Background Pancreatic serous cystic neoplasm (SCN) is an uncommon exocrine neoplasm, which is believed to be a benign entity. However, some of these neoplasms may occasionally attain metastatic ability. Von Hippel–Lindau disease (VHL) manifests a dominantly inherited systemic syndrome accompanied by several benign or malignant tumors, including cystic tumors, in various organs. We describe here a long-term survival case who underwent surgical resection for metachronous liver metastases of pancreatic SCN associated with VHL disease. Case presentation A 35-year-old woman with VHL underwent total pancreatectomy and right nephrectomy for pancreatic SCN and renal cell carcinoma, respectively. At the 4th year follow-up examination after the resection, contrast-enhanced computed tomography (CT) and gadolinium ethoxybenzyl diethylenetriamine penta-acetic acid (Gd-EOB-DTPA)-enhanced magnetic resonance imaging (MRI) showed arterially hyper-enhanced neoplastic lesions in the segment VI and VIII of the liver. Partial resections of the liver were performed 53 months after the initial surgery. At the 6th month follow-up examination from the second surgery, one and two tumors located in the liver segment III, and VIII, respectively, were detected by contrast-enhanced CT and Gd-EOB-DTPA-enhanced MRI. Anterior segmentectomy and partial resection of the segment III were performed 66 months after the initial surgery and 13 months after the second, respectively. The tumors were pathologically diagnosed as liver metastases of pancreatic SCN synonymous with serous cystadenocarcinoma. She remains disease-free without recurrence 6.5 years after the last operation. Conclusions This is the first report of a case of metastatic SCN associated with VHL. Surgical resection might confer a favorable prognosis in patients of pancreatic SCN with liver metastases. |
| first_indexed | 2024-12-17T21:21:07Z |
| format | Article |
| id | doaj.art-f0d3a8d8dcef4f36a2427b927931b93e |
| institution | Directory Open Access Journal |
| issn | 2198-7793 |
| language | English |
| last_indexed | 2024-12-17T21:21:07Z |
| publishDate | 2021-06-01 |
| publisher | SpringerOpen |
| record_format | Article |
| series | Surgical Case Reports |
| spelling | doaj.art-f0d3a8d8dcef4f36a2427b927931b93e2022-12-21T21:32:12ZengSpringerOpenSurgical Case Reports2198-77932021-06-01711810.1186/s40792-021-01239-yA long-term survivor of metachronous liver metastases of pancreatic serous cystic neoplasm associated with von Hippel–Lindau diseaseTakashi Kokumai0Masamichi Mizuma1Katsuya Hirose2Hideaki Karasawa3Masaharu Ishida4Hideo Ohtsuka5Kei Nakagawa6Takanori Morikawa7Takashi Kamei8Atsushi Masamune9Toru Furukawa10Michiaki Unno11Department of Surgery, Tohoku University Graduate School of MedicineDepartment of Surgery, Tohoku University Graduate School of MedicineDepartment of Investigative Pathology, Tohoku University Graduate School of MedicineDepartment of Surgery, Tohoku University Graduate School of MedicineDepartment of Surgery, Tohoku University Graduate School of MedicineDepartment of Surgery, Tohoku University Graduate School of MedicineDepartment of Surgery, Tohoku University Graduate School of MedicineDepartment of Surgery, Tohoku University Graduate School of MedicineDepartment of Surgery, Tohoku University Graduate School of MedicineDivision of Gastroenterology, Tohoku University Graduate School of MedicineDepartment of Investigative Pathology, Tohoku University Graduate School of MedicineDepartment of Surgery, Tohoku University Graduate School of MedicineAbstract Background Pancreatic serous cystic neoplasm (SCN) is an uncommon exocrine neoplasm, which is believed to be a benign entity. However, some of these neoplasms may occasionally attain metastatic ability. Von Hippel–Lindau disease (VHL) manifests a dominantly inherited systemic syndrome accompanied by several benign or malignant tumors, including cystic tumors, in various organs. We describe here a long-term survival case who underwent surgical resection for metachronous liver metastases of pancreatic SCN associated with VHL disease. Case presentation A 35-year-old woman with VHL underwent total pancreatectomy and right nephrectomy for pancreatic SCN and renal cell carcinoma, respectively. At the 4th year follow-up examination after the resection, contrast-enhanced computed tomography (CT) and gadolinium ethoxybenzyl diethylenetriamine penta-acetic acid (Gd-EOB-DTPA)-enhanced magnetic resonance imaging (MRI) showed arterially hyper-enhanced neoplastic lesions in the segment VI and VIII of the liver. Partial resections of the liver were performed 53 months after the initial surgery. At the 6th month follow-up examination from the second surgery, one and two tumors located in the liver segment III, and VIII, respectively, were detected by contrast-enhanced CT and Gd-EOB-DTPA-enhanced MRI. Anterior segmentectomy and partial resection of the segment III were performed 66 months after the initial surgery and 13 months after the second, respectively. The tumors were pathologically diagnosed as liver metastases of pancreatic SCN synonymous with serous cystadenocarcinoma. She remains disease-free without recurrence 6.5 years after the last operation. Conclusions This is the first report of a case of metastatic SCN associated with VHL. Surgical resection might confer a favorable prognosis in patients of pancreatic SCN with liver metastases.https://doi.org/10.1186/s40792-021-01239-ySerous cystadenocarcinomaSerous cystic neoplasmVon Hippel–Lindau diseaseMetastasis |
| spellingShingle | Takashi Kokumai Masamichi Mizuma Katsuya Hirose Hideaki Karasawa Masaharu Ishida Hideo Ohtsuka Kei Nakagawa Takanori Morikawa Takashi Kamei Atsushi Masamune Toru Furukawa Michiaki Unno A long-term survivor of metachronous liver metastases of pancreatic serous cystic neoplasm associated with von Hippel–Lindau disease Surgical Case Reports Serous cystadenocarcinoma Serous cystic neoplasm Von Hippel–Lindau disease Metastasis |
| title | A long-term survivor of metachronous liver metastases of pancreatic serous cystic neoplasm associated with von Hippel–Lindau disease |
| title_full | A long-term survivor of metachronous liver metastases of pancreatic serous cystic neoplasm associated with von Hippel–Lindau disease |
| title_fullStr | A long-term survivor of metachronous liver metastases of pancreatic serous cystic neoplasm associated with von Hippel–Lindau disease |
| title_full_unstemmed | A long-term survivor of metachronous liver metastases of pancreatic serous cystic neoplasm associated with von Hippel–Lindau disease |
| title_short | A long-term survivor of metachronous liver metastases of pancreatic serous cystic neoplasm associated with von Hippel–Lindau disease |
| title_sort | long term survivor of metachronous liver metastases of pancreatic serous cystic neoplasm associated with von hippel lindau disease |
| topic | Serous cystadenocarcinoma Serous cystic neoplasm Von Hippel–Lindau disease Metastasis |
| url | https://doi.org/10.1186/s40792-021-01239-y |
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