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

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 paraneoplas...

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Detalles Bibliográficos
Autores principales: Delvecchio, Antonella, Duda, Loren, Conticchio, Maria, Fiore, Felicia, Lafranceschina, Stefano, Riccelli, Umberto, Cristofano, Antonella, Pascazio, Bianca, Colagrande, Anna, Resta, Leonardo, Memeo, Riccardo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Baishideng Publishing Group Inc 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6715584/
https://www.ncbi.nlm.nih.gov/pubmed/31523385
http://dx.doi.org/10.4240/wjgs.v11.i8.348
Descripción
Sumario: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 follow-up of surgically treated SFTLs is strongly recommended.