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Liver Transplantation for a Metastatic Pancreatic Solid-Pseudopapillary Tumor (Frantz Tumor): A Case Report

BACKGROUND: Solid-pseudopapillary neoplasms (SPN) of the pancreas, first described by Frantz in 1959, are a very rare entity and account for 0.13–2.7% of all pancreatic neoplasms. They are seen predominantly in young women in their second and third decade of life. CASE REPORT: We report a case of a...

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Detalles Bibliográficos
Autores principales: Wójciak, Michał, Gozdowska, Jolanta, Pacholczyk, Marek, Lisik, Wojciech, Kosieradzki, Maciej, Cichocki, Andrzej, Tronina, Olga, Durlik, Magdalena
Formato: Online Artículo Texto
Lenguaje:English
Publicado: International Scientific Literature, Inc. 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6248032/
https://www.ncbi.nlm.nih.gov/pubmed/30061554
http://dx.doi.org/10.12659/AOT.908764
Descripción
Sumario:BACKGROUND: Solid-pseudopapillary neoplasms (SPN) of the pancreas, first described by Frantz in 1959, are a very rare entity and account for 0.13–2.7% of all pancreatic neoplasms. They are seen predominantly in young women in their second and third decade of life. CASE REPORT: We report a case of a 51-year-old female first diagnosed with a pancreatic tumor in 2010 following a computed tomography (CT) scan of the abdomen. The lesion was originally thought to be a neuroendocrine tumor subsequently treated with chemotherapy, which delayed the appropriate treatment. The tumor was rediagnosed as a SPN only after pancreatectomy was performed. Due to the fact that the neoplasm metastasized to the liver, the patient underwent an orthotopic cadaveric liver transplantation (OLTx) in 2013. During the postoperative period lymph node metastases were identified in the abdomen. The patient received surgical treatment and palliative radiotherapy. Presently no signs of recurrence are found either in the bed of pancreatic resection or in the transplanted liver. The function of the transplant organ has demonstrated no abnormalities over the 4-year follow-up. CONCLUSIONS: SPN of the pancreas is a rare disease associated with heterogeneous clinical course ranging from benign to metastatic. Choosing appropriate treatment requires individual clinical assessment of the disease’s spread. Partial living donor liver transplantation or cadaveric liver transplantation might prove an effective therapeutic option for patients with multiple SPN metastases in the liver. It ought to be remembered, however, that the experience in this area is quite limited.