Abstract:Currarino syndrome (CS) is an autosomal dominant syndrome caused by mutations in MNX1 and characterized by anorectal abnormalities, partial sacral agenesis, and presacral masses. The presacral masses are typically benign; however, malignant degeneration can occur, and presacral neuroendocrine tumors (NETs) have been reported in six cases. We report three individuals from two families affected by CS in which multiple individuals developed presacral NETs. The first family, 491, had six members with features of C… Show more
Introduction: Presacral neuroendocrine tumors (PSNET) are a rare disease that currently lacks a standardized treatment approach. In this report, we present a unique case of PSNET with liver metastasis that progressed into a pseudosacral cyst following complete surgical resection and Sulfatinib treatment with radiofrequency ablation.
case summary: A 49-year-old woman presented to our department with hematochezia. Magnetic resonance imaging revealed a presacral mass with liver metastasis. We performed a complete resection of the mass through a transperineal approach and a terminal ileostomy. Postoperative pathology confirmed a G2 neuroendocrine tumor. After multidisciplinary consultation, the patient received targeted treatment with Sulfatinib and underwent radiofrequency ablation of the liver tumors using fluorescence laparoscopy. Six months later, the patient developed an anterior sacral pseudocyst, which required surgical intervention.
Conclusion: Before undergoing surgical treatment for presacral neuroendocrinology, it is important to consider the potential risks of poor healing of the presacral incision and formation of pseudocysts. These complications may be caused by various factors, including the procedure itself and the use of targeted drugs.
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