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

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.

An urological cause of hypoglycaemia: A case report of the Doege-Potter syndrome

“…Definitive diagnosis of an SFT depend upon histological identification of a spindle cell tumor with alternating areas of cellularity and hypocellular collagen rich areas, as well as consistent CD34 expression and negative expression patterns for cytokeratin AE1/AE3, desmin and S-100. Although the majority of SFTs are benign, 20% may present local recurrence and metastases (9,10). Malignant criteria include: hypercellularity (> 4 mitotic figures/10 high power fields), atypia, infiltrative growth pattern and necrosis (11).…”