Pheochromocytomas are rare tumours, 22% of which are extra-adrenal and are known as paragangliomas. We report a case of a young male with non-functioning paraganglioma of the liver; a very uncommon primary site. A CT scan with contrast showed a huge, highly vascular mass lesion in the right lobe of the liver and a core biopsy confirmed it to be a paraganglioma. Whole body study with iodine-131-meta-iodobenzylguanidine (MIBG) tracer highlighted the liver tumour but was negative for any other active lesions depicting that the tumour was confined to the liver. The lesion was completely resected by extended right hepatectomy and the patient had an uneventful recovery. There is no evidence of disease recurrence at 3 years of follow up

Ahmed, Arsalan

Jabbar, Abdul

Khan, Muhammad Rizwan

Raza, Rushna

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eCommons@AKUSection of General Surgery Department of SurgeryAugust 2011Primary non-functioning paraganglioma of liver: arare tumour at an unusual locationMuhammad Rizwan KhanAga Khan UniversityRushna RazaAga Khan UniversityAbdul JabbarAga Khan UniversityArsalan AhmedAga Khan UniversityFollow this and additional works at: http://ecommons.aku.edu/pakistan_fhs_mc_surg_genPart of the Surgery CommonsRecommended CitationKhan, M., Raza, R., Jabbar, A., Ahmed, A. (2011). Primary non-functioning paraganglioma of liver: a rare tumour at an unusuallocation. Journal of the Pakistan Medical Association, 61(8), 814-6.Available at: http://ecommons.aku.edu/pakistan_fhs_mc_surg_gen/14AbstractPheochromocytomas are rare tumours, 22% of whichare extra-adrenal and are known as paragangliomas. Wereport a case of a young male with non-functioningparaganglioma of the liver; a very uncommon primary site. ACT scan with contrast showed a huge, highly vascular masslesion in the right lobe of the liver and a core biopsyconfirmed it to be a paraganglioma. Whole body study withiodine-131-meta-iodobenzylguanidine (MIBG) tracerhighlighted the liver tumour but was negative for any otheractive lesions depicting that the tumour was confined to theliver. The lesion was completely resected by extended righthepatectomy and the patient had an uneventful recovery.There is no evidence of disease recurrence at 3 years offollow up.Keywords: Paraganglioma, Non-functional, Primary, Liver.IntroductionParagangliomas are extra-adrenal pheochromocytomasthat arise from paraganglion cell nests along the sympatheticchain extending from the skull-base to the pelvic floor.1 Thesetumours, like other neuroendocrine tumours, are hypervascularand usually benign.2 Patient can present with the clinicalpicture of catecholamine excess in case of functional tumour ormay be completely asymptomatic. About 10% ofpheochromocytomas are malignant and about 22% arise fromextra-adrenal sites.1 Few unusual sites have been reportedincluding the gallbladder, biliary ductal system, larynx, lungVol. 61, No. 8, August 2011 814Case ReportPrimary non-functioning paraganglioma of liver: a raretumour at an unusual locationMuhammad Rizwan Khan,1 Rushna Raza,2 Abdul Jabbar,3 Arsalan Ahmed4Department of Surgery,1,2 Department of Medicine,3 Department of Pathology,4 Aga Khan University & Hospital, Karachi.and the urinary bladder.3,4 Primary hepatic site is extremelyrare and very few cases have been reported to date.1,2,5-8 Sincehepatic metastasis of pheochromocytomas is more frequent, itis important to exclude the presence of a primary adrenaltumour in these patients.Case ReportA 24-year old male presented with a 6 weeks historyof generalized bodyaches and headaches, low grade feverwith chills and sweating. He also complained of intermittentvague abdominal pain associated with episodes of diarrhoea.There was no history of hypertension or weight loss. Pasthistory was not significant except for an episode of entericfever two years back. On examination he had a soft non-tender palpable liver 3cm below the costal margin. His liverfunction tests were within normal limits except for elevationof serum gamma-glutamyl transpeptidase (GGT) level to 93IU/L (normal range: 3-50 IU/L). Blood counts were alsonormal, although haemoglobin was low at 10.8 mg/dL(normal range: 12.4-16.8 mg/dL) and ESR was 105mm/hr(normal range: 3-13 mm/hr). An ultrasound of the abdomen done at anotherhospital revealed a 15x18 cm, heterogeneous, cystic cumsolid mass in the liver which was speculated to be a liverabscess. Upon aspiration, haemorrhagic fluid was found andno malignant cells were seen. IHA for amoebiasis,echinococcus serology and fluid cultures were negative. A CTscan of the abdomen with IV contrast was done whichshowed a huge, highly vascular, 20x18x14 cm size mass inthe right lobe of liver which was compressing the inferiorvena cava, right and middle hepatic veins (Figure-1). It wasshowing intense enhancement in arterial phase in theperiphery and none in the center. Blood supply was mainlyfrom right hepatic artery. Differentials of giant cavernoushaemangioma or angiosarcoma were suggested. An ultrasound guided core biopsy of the lesion wasperformed. Histopathology revealed a neoplastic lesion withnests of cuboidal cells separated by fibrovascular septae,surrounded by flattened layer of sustentacular cells. Moderateamphophilic granular cytoplasm containing deeplyhyperchromatic rounded nuclei were seen withinconspicuous nucleoli. No definitive pleomorphism,increased mitotic activity or necrosis was noted (Figure-2).The neoplastic cells showed negativity for cytoplasmicglycogen on PAS special stain and were positive forChromogranin and Synaptophysin. On the basis ofmorphology and immunohistochemical results, a diagnosis ofparaganglioma was made.An endocrinology consult was sought for anysyndromic associations, functional component or possibilityof metastasis from a primary adrenal or spinal tumour.Sympathetic blockade of alpha or beta receptors was notadvised as the patient had no symptoms of functional tumourand urinary vanillylmandelic acid (VMA) levels were withinnormal limits (2.4 mg/24hr, normal range: 2-7mg/24hr).Serum Calcitonin was < 2.0pg/ml (normal range: 0.8-9.9pg/ml) and serum Calcium level was 9.1mg/dL (normalrange: 8.5-10.2mg/dL); therefore multiple endocrineneoplasia (MEN) IIa syndrome was excluded. Whole bodystudy with Iodine-131 MIBG highlighted the liver tumour butwas negative for any other active lesions depicting that thetumour was confined to the liver.In view of the highly vascular nature of the lesion,preoperative angiography and embolization was planned. Thetumour showed extensive vascularity, with feeding vesselsbranching directly from the aorta, right hepatic artery, rightadrenal artery and small contribution from superior mesentericartery. Embolization of direct branches from hepatic arteryand adrenal arterial branches was done using polyvinylalcohol particles. Significant reduction in vascularity was815 J Pak Med AssocFigure-1: Axial CT scan showing highly vascular giant paraganglioma of liveroccupying whole of the right lobe of liver.Figure-2: 20X magnified view of histological section of paraganglioma.achieved by embolization with some residual tumour blushfrom renal artery and superior mesenteric artery.Three days post embolization, extended righthepatectomy was carried out. As expected, the tumour wasstill highly vascular involving the right hepatic lobe withmultiple collateral blood vessels. Left lobe was hypertrophiedwith normal texture. Adrenal glands were also normal.Extended right hepatectomy was done and the finalhistopathology confirmed paraganglioma of liver with clearmargins of excision. The lymph nodes recovered at the neckof the gallbladder showed reactive changes without anyevidence of malignancy.Post-operatively, the patient was kept in intensive careunit for 24 hours. Diet was progressed gradually, and he wasencouraged to mobilize. Liver function tests and blood countreturned to normal limits and he was discharged from hospitalin 5 days. He was followed with ultrasound and LFTs everythree months and CT scan every year. He remainedasymptomatic without any radiological evidence ofrecurrence at 3 years of follow up.DiscussionParaganglioma occurring primarily in the liver is ararity. This unusual location can be associated with ectopicchromaffin tissue in the liver.2 Hepatic metastasis ofpheochromocytomas from a primary adrenal tumour is amore frequent occurrence.8We ruled this out in our case by anegative MIBG and CT scan with IV contrast. Biochemical evaluation of paraganglioma includes24-hour urine norepinephrine, epinephrine, metanephrines,normetanephrines, dopamine, and VMA. Plasmametanephrines and normetanephrines can also elicit thediagnosis of familial syndromes, such as multiple endocrineneoplasia Type 2, Von Hippel Lindau or familialparagangliomas. In our case, normal 24-hour urine VMAdepicted a non-functional nature of the tumour. It could alsobe possible that any catecholamines produced by the tumourwere being metabolized by the liver. This is more likely if thevenous drainage of the tumour is into the portal system.9The differential diagnoses of cystic-solid lesions inthe liver are many and should always include aneuroendocrine tumour. Percutaneous preoperative biopsy(fine needle aspiration or core biopsy) should only be donewith extreme caution and preparation if a paraganglioma issuspected, as it can potentially result in life-threatening crisis.In our patient, the tumour was essentially asymptomatic andthe suspicion of paraganaglioma was very low since the liveris an extremely uncommon site for primary paraganglioma,therefore, a core biopsy with all the precautions was done.In our patient, the tumour was in the right lobe ofliver. It was huge, highly vascular and located in closeproximity to the inferior vena cava and middle hepatic vein.Preoperative angioembolization decreased the vascularity ofthe tumour to some extent, but surgical procedure was still achallenge in this situation. The procedure was carried outsuccessfully in this patient and he made an uneventfulrecovery. Long-term follow up of pheochromocytomas isusually recommended due to the potential malignant natureof this tumour and likelihood of occult primary lesions.[8] Inour case so far the patient has remained asymptomaticwithout any evidence of recurrence.ConclusionIn conclusion, the reported patient is a very rare caseof primary benign hepatic paraganglioma. The diagnostic andtherapeutic approach to this problem has been detailed. Thediagnosis was speculated on the CT scan and confirmed bybiopsy while VMA and MIBG scan excluded any otherprimary or metastatic lesion. Chemical embolizationfollowed by extended right hepatectomy eliminated theprimary tumour with uneventful recovery of the patient.References1. Chang H, Xu L, Mu Q. Primary functioning hepatic paraganglioma: a casereport. Adv Ther 2006; 23: 817-20.2. Rimmelin A, Hartheiser M, Gangi A, Welsch M, Jeung MY, Jaeck D, et al.Primary hepatic pheochromocytoma. Eur Radiol 1996; 6: 82-5.3. Freschi M, Sassi I. Paraganglioma of the gallbladder. Pathologica1990;82:459-63.4. Juraschek F, Engloff H, Buemi A, Laedlein-Greilsammer D. Paraganglioma ofthe urinary bladder. Urology 1983; 22: 659-63. 5. Corti B, D'Errico A, Pierangeli F, Fiorentino M, Altimari A, Grigioni WF.Primary paraganglioma strictly confined to the liver and mimickinghepatocellular carcinoma. Am J Surg Pathol 2002; 26: 945-9. 6. Reif MC, Hanto DW, Moulton JS, Alspaugh JP, Bejarano P. Primary hepaticpheochromocytoma? Am J Hypertension 1996; 9: 1040-3.7. Kang YS, Kuhlman JE, Fishman EK, Zerhouni EA. Nonfunctionalparaganglioma of the liver. Clin Imaging 1991; 15: 216-9.8. Roman SA, Sosa JA. Functional paragangliomas presenting as primary livertumours. South Med J 2007; 100: 195-6.9. Tanaka S, Ito T, Tomoda J, Higashi T, Yamada G, Tsuji T. Malignantpheochromocytomas with hepatic metastasis diagnosed 20 years after resectionof the primary adrenal lesion. Intern Med 1993; 32: 789-94.Vol. 61, No. 8, August 2011 816

Primary non-functioning paraganglioma of liver: A rare tumour at an unusual location

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Primary non-functioning paraganglioma of liver: A rare tumour at an unusual location

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