Primary functioning hepatic paraganglioma mimicking hepatocellular carcinoma: A case report and literature review

Abstract

Introduction: Hepatic paraganglioma (HPGL) originates from the sympathetic nervous tissue in the liver, and is an extremely rare type of the sympathetic paragangliomas. Till now, only 11 HPGL cases have been reported.

Case presentation: A 49-year-old woman presented to our hospital with a lesion in the right lobe of the liver, which grew from 2 to 6 cm in 2 years. In addition, she had a 6-year history of diabetes. The patient was initially diagnosed as hepatocellular carcinoma and hepatectomy was performed. Surgical resection of the liver lesion was successful, but the blood pressure rose and fell sharply when the lesion was being removed. The pathological examination of the liver lesion showed that it was HPGL. After the operation, the patient recovered uneventfully. Follow-up examination showed the blood glucose level went back to the normal range in 20 days after the operation, and MRI and I-MIBG scan showed that there was no evidence of recurrence and metastasis in >2 years.

Conclusion: By means of reporting this case and reviewing 11 reported cases, we conclude that the incidence of HPGLs is extremely low and the clinical and radiological characteristics of HPGLs are nonspecific; thus, it is hard to diagnose HPGLs correctly. Surgical resection is curative therapy for HPGLs, whereas the removing of HPGLs may cause the releasing of catecholamine, and then lead to hypertension crisis and arrhythmia. Thus, antihypertensive therapy is necessary during the operation. Follow-ups after the operation are important for HPGL patients, for pathological examinations are not sufficient to differ malignant HPGLs from benign ones, and follow-ups are helpful for HPGL patients to find the recurrent foci or metastases timely.

Figure 1

Preoperative radiological examination of the reported case. (A) Ultrasonography revealed a hypoechoic mass with its diameter of 5.7 × 4.9 cm (A1), color Doppler flow imaging indicated that the mass was next to the second porta of liver (A2). From (A) to (C), yellow arrows heads direct the liver lesion; (B) abdominal MRI scanning. T1-weighted magnetic resonance (MR) image showed a low signal intensity mass which located in the segment 7 and 8 and caudate process of the liver (B1), and T2-weighted MR image showed the lesion is of high signal intensity (B2); MR with diffusion-weighted images showed that the mass was hyperintensity with restricted diffusion (B3); MR with perfusion-weighted imaging showed the mass was hyperenhanced in the arterial phase (B4) and de-enhanced in the portal phase (B5); coronal view of 2D-FIESTA sequence images showed the mass is close to the right hepatic vein (B6) and the right posterior branch of the portal vein (B7); and MR cholangiopancreatography showed no obvious abnormality in the bile duct (B8); (C) abdominal computed tomography (CT) scanning. Plain CT scan showed a hypoattenuating liver lesion (C1); arterial phase showed nodular enhancement of the lesion (C2); portal phase showed a low density nodule(C3); (D) 3D reconstruction of CT images. anterior view (D1-D4) and upper view (D5) of the reconstructed images of liver are showed. The liver lesion is labeled with light green, liver is labeled with auburn, abdominal aorta, and hepatic artery are labeled with red, portal vein and its branches are labeled with cyan, and hepatic vein and inferior vena cava are labeled with blue.

Figure 2

Postoperative pathological examination of the reported case. (A) Gross appearance showed the cut surface of the mass; (B) Hematoxylin-eosin staining of tumor tissue (black arrow). Original magnification: 200×; (C) immunohistochemical staining for indicated markers. Original magnification: 200×.