Metachronous triple cancer associated with Peutz–Jeghers syndrome treated with curative surgery: a case report
Abstract Background Peutz–Jeghers syndrome (PJS) is an autosomal dominant disorder characterized by mucocutaneous pigmentation and hamartomatous gastrointestinal polyposis. It is well known that individuals with PJS are at an increased risk of cancer in a variety of organs. Case presentation Here, w...
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Japan Surgical Society
2018-08-01
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| Series: | Surgical Case Reports |
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| Online Access: | http://link.springer.com/article/10.1186/s40792-018-0492-6 |
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| author | Toru Yoshikawa Tomoyuki Abe Hironobu Amano Keiji Hanada Tomoyuki Minami Tsuyoshi Kobayashi Shuji Yonehara Masahiro Nakahara Hideki Ohdan Toshio Noriyuki |
| author_facet | Toru Yoshikawa Tomoyuki Abe Hironobu Amano Keiji Hanada Tomoyuki Minami Tsuyoshi Kobayashi Shuji Yonehara Masahiro Nakahara Hideki Ohdan Toshio Noriyuki |
| author_sort | Toru Yoshikawa |
| collection | DOAJ |
| description | Abstract Background Peutz–Jeghers syndrome (PJS) is an autosomal dominant disorder characterized by mucocutaneous pigmentation and hamartomatous gastrointestinal polyposis. It is well known that individuals with PJS are at an increased risk of cancer in a variety of organs. Case presentation Here, we present a patient with PJS who achieved long-term survival by undergoing repeat curative surgery for metachronous triple cancer. Her medical history included hilar cholangiocarcinoma and cervical carcinoma; curative surgery was performed for both conditions. On annual follow-up, the level of carcinoembryonic antigen was elevated at 6.9 ng/ml. Enhanced computed tomography revealed a cystic tumor consisting of mural nodules at the pancreatic head; the maximal diameter was 15 mm. Magnetic resonance imaging clearly demonstrated the tumor with low intensity on T1-weighted images and high intensity on T2-weighted images. Endoscopic ultrasound sonography showed a high echoic tumor at the pancreatic head, which was confirmed as adenocarcinoma by fine-needle aspiration biopsy. The preoperative diagnosis was intraductal papillary mucinous carcinoma (IPMC; T1N0M0, stage IA). Subtotal stomach-preserving pancreaticoduodenectomy was performed and the final diagnosis was IPMC, stage 0 (TisN0M0). Conclusions Aggressive surgery for metachronous triple cancer resulted in good long-term prognosis. Continuous and systematic follow-up would allow the detection of malignancy at an early stage and make treatment with curative surgery possible. |
| format | Article |
| id | doaj-art-313bfa03ef384572be1487e89f4f1c12 |
| institution | OA Journals |
| issn | 2198-7793 |
| language | English |
| publishDate | 2018-08-01 |
| publisher | Japan Surgical Society |
| record_format | Article |
| series | Surgical Case Reports |
| spelling | doaj-art-313bfa03ef384572be1487e89f4f1c122025-08-20T02:37:29ZengJapan Surgical SocietySurgical Case Reports2198-77932018-08-01411510.1186/s40792-018-0492-6Metachronous triple cancer associated with Peutz–Jeghers syndrome treated with curative surgery: a case reportToru Yoshikawa0Tomoyuki Abe1Hironobu Amano2Keiji Hanada3Tomoyuki Minami4Tsuyoshi Kobayashi5Shuji Yonehara6Masahiro Nakahara7Hideki Ohdan8Toshio Noriyuki9Department of Surgery, Onomichi General HospitalDepartment of Surgery, Onomichi General HospitalDepartment of Surgery, Onomichi General HospitalDepartment of Gastroenterology, Onomichi General HospitalDepartment of Gastroenterology, Onomichi General HospitalDepartment of Gastroenterological and Transplant Surgery, Graduate School of Biomedical and Health Sciences, Hiroshima UniversityDepartment of Pathology, Onomichi General HospitalDepartment of Surgery, Onomichi General HospitalDepartment of Gastroenterological and Transplant Surgery, Graduate School of Biomedical and Health Sciences, Hiroshima UniversityDepartment of Surgery, Onomichi General HospitalAbstract Background Peutz–Jeghers syndrome (PJS) is an autosomal dominant disorder characterized by mucocutaneous pigmentation and hamartomatous gastrointestinal polyposis. It is well known that individuals with PJS are at an increased risk of cancer in a variety of organs. Case presentation Here, we present a patient with PJS who achieved long-term survival by undergoing repeat curative surgery for metachronous triple cancer. Her medical history included hilar cholangiocarcinoma and cervical carcinoma; curative surgery was performed for both conditions. On annual follow-up, the level of carcinoembryonic antigen was elevated at 6.9 ng/ml. Enhanced computed tomography revealed a cystic tumor consisting of mural nodules at the pancreatic head; the maximal diameter was 15 mm. Magnetic resonance imaging clearly demonstrated the tumor with low intensity on T1-weighted images and high intensity on T2-weighted images. Endoscopic ultrasound sonography showed a high echoic tumor at the pancreatic head, which was confirmed as adenocarcinoma by fine-needle aspiration biopsy. The preoperative diagnosis was intraductal papillary mucinous carcinoma (IPMC; T1N0M0, stage IA). Subtotal stomach-preserving pancreaticoduodenectomy was performed and the final diagnosis was IPMC, stage 0 (TisN0M0). Conclusions Aggressive surgery for metachronous triple cancer resulted in good long-term prognosis. Continuous and systematic follow-up would allow the detection of malignancy at an early stage and make treatment with curative surgery possible.http://link.springer.com/article/10.1186/s40792-018-0492-6Curative surgeryMetachronous cancerPeutz–Jeghers syndrome |
| spellingShingle | Toru Yoshikawa Tomoyuki Abe Hironobu Amano Keiji Hanada Tomoyuki Minami Tsuyoshi Kobayashi Shuji Yonehara Masahiro Nakahara Hideki Ohdan Toshio Noriyuki Metachronous triple cancer associated with Peutz–Jeghers syndrome treated with curative surgery: a case report Surgical Case Reports Curative surgery Metachronous cancer Peutz–Jeghers syndrome |
| title | Metachronous triple cancer associated with Peutz–Jeghers syndrome treated with curative surgery: a case report |
| title_full | Metachronous triple cancer associated with Peutz–Jeghers syndrome treated with curative surgery: a case report |
| title_fullStr | Metachronous triple cancer associated with Peutz–Jeghers syndrome treated with curative surgery: a case report |
| title_full_unstemmed | Metachronous triple cancer associated with Peutz–Jeghers syndrome treated with curative surgery: a case report |
| title_short | Metachronous triple cancer associated with Peutz–Jeghers syndrome treated with curative surgery: a case report |
| title_sort | metachronous triple cancer associated with peutz jeghers syndrome treated with curative surgery a case report |
| topic | Curative surgery Metachronous cancer Peutz–Jeghers syndrome |
| url | http://link.springer.com/article/10.1186/s40792-018-0492-6 |
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