Abstract
BACKGROUND: Primary pancreatic paraganglioma is exceedingly rare. Most patients with pancreatic paraganglioma lack a typical clinical presentation, and the tumor is difficult to accurately differentiate from other pancreatic neuroendocrine tumors, making the misdiagnosis rate extremely high. Surgical excision is the primary treatment modality but is considered high risk. Because of its rich vascularity, the tumor easily bleeds during surgery, especially malignant paragangliomas invading large blood vessels. Thus, a thorough preoperative evaluation of the tumor is necessary. Here, we report a primary malignant pancreatic paraganglioma, the second such case in a young patient that was successfully resected surgically. CASE SUMMARY: A 26-year-old female patient was admitted to the hospital with unexplained abdominal pain. Dual-layer spectral-detector computed tomography (DLCT) revealed a mixed density mass in the pancreatic body and tail. The patient was transferred to our hospital after previous failed surgical resection at other hospitals. The patient and her family strongly desired surgery. After a thorough preoperative evaluation and adequate preparation, a large mass with the greatest dimension of 8.0 cm was successfully resected. The final pathological diagnosis was malignant paraganglioma. The patient was discharged in good condition 2 wk postoperatively. CONCLUSION: The rare malignant pancreatic paraganglioma reported here was difficult to diagnose preoperatively. Early filling of the draining vein may be a crucial diagnostic imaging feature. DLCT can provide more precise information for surgical resection through dual-energy imaging.