Primary functioning hepatic paraganglioma mimicking hepatocellular carcinoma

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 131I-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.

Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

“…Malignancy is defined by the presence of distant metastases at sites where chromaffin cells are normally absent—bones and lymph nodes [19]. However, for lung and liver, it has to be considered that both organs normally contain ganglia, and therefore in these locations, it may be difficult to differentiate between metastases and primary PGLs, especially if past medical history is negative [20,21,22,23,24,25]. Validated prognostic pathological parameters for malignant PCCs/PGLs are lacking, although some risk stratification systems have been described, such as The Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) [26] and the more recently extended Grading of Adrenal Pheochromocytoma and Paraganglioma (GAPP) [27].…”

The Diagnosis and Clinical Significance of Paragangliomas in Unusual Locations

“…Because of this widespread distribution of paraganglia, paragangliomas can occur at virtually all locations in the body except within the brain and in bone, and although they have been reported in the extremities [ 10 ], these are exceptional curiosities. Given the normal distribution of paraganglia associated with components of the autonomous nervous system, it should be no surprise that paragangliomas can occur in the gallbladder [ 11 , 12 ], and the liver [ 8 , 13 , 14 ], where they are likely to arise from small abdominal vagus nerve branches. Paraganglia are present and can give rise to paragangliomas in various sites including the orbit, mandible, paranasal sinuses and sellar region [ 8 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 ], adjacent to or within the thyroid gland [ 8 , 24 , 25 , 26 , 27 , 28 , 29 ], in the parathyroid [ 30 ], in the mediastinum [ 8 , 31 , 32 , 33 ], within the lungs and heart [ 8 , 34 , 35 , 36 , 37 , 38 ], gut [ 8 ], and pancreas [ 8 , 39 , 40 ] and in the mesentery [ 8 , 41 , 42 ].…”

“…We recommend the use of pan-keratin antibodies, such as AE1/AE3, along with the CAM 5.2 antibody cocktail that highlights most NETs. While there are reports of unusual PGLs that may stain for keratins [ 13 , 14 , 37 , 40 ], the lack of keratin expression in a presumed NEN should raise the suspicion of PGL. In the authors’ experience, no PGLs other than the rare and unusual tumors classified as gangliocytic and cauda equine-type PGLs are positive for keratins.…”

Hepatic paraganglioma hiding as a slowly growing lesion for 24 years: a diagnostic conundrum

“…PGLs are uncommon neuroendocrine tumours usually arising from sympathetic chains or parasympathetic paraganglia 1. Hepatic PGLs are rare, with only 12 cases described in the literature 2. They are difficult to diagnose correctly as their clinical manifestations and radiological appearance are non-specific.…”