BACKGROUND: Solid pseudopapillary tumor (SPT) of the pancreas, also known as Franz tumor, Hamoudie tumor, solid-cystic-papillary epithelial neoplasm, or solid and cystic tumor, is a neoplasm of transitory (potential) malignancy, seen predominantly in young women. CASE REPORT: This report presents a female patient treated for a solid pseudopapillary tumor of the pancreas with hepatic metastases. The tumor was first diagnosed in 2006. Non-specific abdominal pain was the first presenting symptom. The patient underwent distal pancreatic resection and splenectomy in July 2006. Multifocal metastatic disease seen at surgery precluded radical resection. Following definitive pathology confirmation and the exclusion of extrahepatic metastases, the patient was referred to our transplant centre 18 months after pancreatic surgery, to be considered for orthotopic liver transplantation (OLTx). The extent of the disease was once again evaluated by imaging studies, followed by exploratory laparotomy. The patient underwent cadaveric liver transplantation in March 2008, with triple immunosuppression (tacrolimus, MMF, and steroids) following surgery. Presently, more than 5 years post-transplant, the patient has no signs of recurrent neoplasmatic disease. CONCLUSIONS: This is the first liver transplantation for a metastatic pancreatic pseudopapillary tumor in Poland, with the longest follow-up period described in the literature. Follow-up suggests a cautiously optimistic prognosis despite primary unresectability of hepatic metastases and the necessity for immunosuppressive therapy.
BACKGROUND: Solid pseudopapillary tumor (SPT) of the pancreas, also known as Franz tumor, Hamoudie tumor, solid-cystic-papillary epithelial neoplasm, or solid and cystic tumor, is a neoplasm of transitory (potential) malignancy, seen predominantly in young women. CASE REPORT: This report presents a female patient treated for a solid pseudopapillary tumor of the pancreas with hepatic metastases. The tumor was first diagnosed in 2006. Non-specific abdominal pain was the first presenting symptom. The patient underwent distal pancreatic resection and splenectomy in July 2006. Multifocal metastatic disease seen at surgery precluded radical resection. Following definitive pathology confirmation and the exclusion of extrahepatic metastases, the patient was referred to our transplant centre 18 months after pancreatic surgery, to be considered for orthotopic liver transplantation (OLTx). The extent of the disease was once again evaluated by imaging studies, followed by exploratory laparotomy. The patient underwent cadaveric liver transplantation in March 2008, with triple immunosuppression (tacrolimus, MMF, and steroids) following surgery. Presently, more than 5 years post-transplant, the patient has no signs of recurrent neoplasmatic disease. CONCLUSIONS: This is the first liver transplantation for a metastatic pancreatic pseudopapillary tumor in Poland, with the longest follow-up period described in the literature. Follow-up suggests a cautiously optimistic prognosis despite primary unresectability of hepatic metastases and the necessity for immunosuppressive therapy.
Authors: Michał Wójciak; Jolanta Gozdowska; Marek Pacholczyk; Wojciech Lisik; Maciej Kosieradzki; Andrzej Cichocki; Olga Tronina; Magdalena Durlik Journal: Ann Transplant Date: 2018-07-31 Impact factor: 1.530
Authors: Emmanuel Ii Uy Hao; Ho Kyung Hwang; Dong-Sub Yoon; Woo Jung Lee; Chang Moo Kang Journal: Medicine (Baltimore) Date: 2018-12 Impact factor: 1.817