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Doege-Potter syndrome by malignant solitary fibrous tumor of the liver: A case report and review of literature

World Journal of Gastrointestinal Surgery 2019年第8期11卷 348-357页

作者： Delvecchio Antonella Duda Loren Conticchio Maria Fiore Felicia lafranceschina stefano Riccelli Umberto Cristofano Antonella Pascazio Bianca Colagrande Anna Resta Leonardo Memeo Riccardo Department of Emergency and Organ Transplantation General Surgery Unit “M. Rubino” University of Bari Bari 70124 Italy Department of Pathology University of Bari Bari 70124 Italy Department of Oncology Ente Ecclesiastico Ospedale F. Miulli Acquaviva delle Fonti 70021 Italy Department of General Surgery Ospedale San Paolo Bari 70123 Italy

BACKGROUND Solitary fibrous tumor of the liver (SFTL) is a rare occurrence with a low number of cases reported in literature. SFTL is usually benign but, 10%-20% cases are reported to be malignant with a tendency to metastasize. The majority of malignant SFTL cases are associated with a paraneoplastic hypoglycaemia defined as Doege-Potter syndrome. Surgery is the best therapeutic treatment, however, long- life follow-up is recommended. CASE SUMMARY A 74-year-old man, was admitted to the emergency department after a syncopal episode with detection of hypoglycaemia resistant to medical treatment. The computed tomography revealed a solid mass measuring 15 cm of the left liver. An open left hepatectomy was performed with complete resection of tumor. Histopathological analyses confirmed a malignant SFTL. CONCLUSION Large series with long-term follow-up have not been published neither have clinical trials been undertaken. Consequently, the methodical long-term followup of surgically treated SFTLs is strongly recommended.

关键词： Solitary fibrous tumor Malignant solitary fibrous tumor of the liver Mesenchymal tumor Hepatic tumor Doege-Potter syndrome

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