Delvecchio A, Duda L, Conticchio M, Fiore F, Lafranceschina S, Riccelli U, Cristofano A, Pascazio B, Colagrande A, Resta L, Memeo R. Doege-Potter syndrome by malignant solitary fibrous tumor of the liver: A case report and review of literature. World J Gastrointest Surg 2019; 11(8): 348-357 [PMID: 31523385 DOI: 10.4240/wjgs.v11.i8.348]
Corresponding Author of This Article
Antonella Delvecchio, MD, Surgeon, Department of Emergency and Organ Transplantation, General Surgery Unit “M. Rubino”, University of Bari, 17 Street Mario Pagano, Ceglie Messapica, Bari 70124, Italy. antodel88@libero.it
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Case Report
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Delvecchio A, Duda L, Conticchio M, Fiore F, Lafranceschina S, Riccelli U, Cristofano A, Pascazio B, Colagrande A, Resta L, Memeo R. Doege-Potter syndrome by malignant solitary fibrous tumor of the liver: A case report and review of literature. World J Gastrointest Surg 2019; 11(8): 348-357 [PMID: 31523385 DOI: 10.4240/wjgs.v11.i8.348]
Antonella Delvecchio, Maria Conticchio, Felicia Fiore, Stefano Lafranceschina, Umberto Riccelli, Riccardo Memeo, Department of Emergency and Organ Transplantation, General Surgery Unit “M. Rubino”, University of Bari, Bari 70124, Italy
Loren Duda, Anna Colagrande, Leonardo Resta, Department of Pathology, University of Bari, Bari 70124, Italy
Antonella Cristofano, Department of Oncology, Ente Ecclesiastico Ospedale F. Miulli, Acquaviva delle Fonti 70021, Italy
Bianca Pascazio, Department of General Surgery, Ospedale San Paolo, Bari 70123, Italy
Author contributions: Delvecchio A and Memeo R designed the report and the review; Conticchio M, Fiore F, Pascazio B and Lafranceschina S collected the patient’s clinical data; Riccelli U, native English speaker, responsable of revision English language; Duda L, Colagrande A and Resta L provided relevant figures and described anatomopathological characteristics; Cristofano A designed oncological features; all authors issued final approval for the version to be submitted.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that no conflict of interest exists.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Corresponding author: Antonella Delvecchio, MD, Surgeon, Department of Emergency and Organ Transplantation, General Surgery Unit “M. Rubino”, University of Bari, 17 Street Mario Pagano, Ceglie Messapica, Bari 70124, Italy. antodel88@libero.it
Telephone: +39-32-08013447
Received: June 13, 2019 Peer-review started: June 19, 2019 First decision: August 2, 2019 Revised: August 9, 2019 Accepted: August 13, 2019 Article in press: August 13, 2019 Published online: August 27, 2019 Processing time: 75 Days and 3.6 Hours
Abstract
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.
Core tip: Solitary fibrous tumors (SFTs) are rare mesenchymal tumors first described in 1931 by Klemperer and Rabin. Usually, SFTs are benign, but 10%-20% of them are reported to be malignant with a tendency to metastasize. At present, 22 cases of liver malignant SFTs, including our patient, have been reported in literature. Some cases are associated with a paraneoplastic hypoglycaemia definied as Doege-Potter syndrome. Surgery is the best therapeutic modality and prolongs life, but also improves the clinical characteristics associated with Doege-Potter syndrome if present. Longlife follow-up is recommended.
