Iwatsuki, S

Madariaga, JR

Selby, R

Starzl, TE

Tepetes, K

Webb, M

English

Konstantinos Tepetes

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Archives of Surgery

Orthotopic Liver Transplantation for Benign Hepatic Neoplasms

Name not available

Orthotopic liver transplantation for benign hepatic neoplasms

D-Scholarship@Pitt

• ~ ,. t Orthotopic Liver Transplantation for Benign Hepatic Neoplasms Konstancinos Tepeces. MD; Rick Selby. MD; Marc Webb. MD; Juan R. Madahaga. ,\1.0; Shunzaburo Iwacsulzi. MD. PhD; Thomas E. Scar::/, MD. PhD Obiective: To evaluate the frequency and outcome of liver transplantation for symptomatic, unresectable. be-nign hepatIc neoplasms. hepatic lymphangiomatosis (n= 1), hilar fibrous angio-dysplasia (n= 1). focal nodular hyperplasia (n= 1). and hem-anglOma (n= I). There were three perioperative deaths and two late deaths at 56 and 84 months. The remaining pa-tients are alive. 'With follow-up ranging from 36 to 145 months. Median survival for the nine patients who sur-vived the perioperatIve period is 88 months. The early deaths were attributable to hemorrhagic complications (n=2) and necrotizing pancreatitis (n=1). The two late deaths were due to disseminated aspergillosis and hepa-titis-associated cirrhosis. Design: Retrospective study. Setting: Presbyterian University Hospital, a tertiary care referral center for liver transplantallon affiliated with the r~iversity of Pittsburgh (Pa). Patients: All 3239 liver transplant recipients at the Uni-versity of Pittsburgh from January 1981 until January 1993. Results: Twelve (0.37%) of 3239 patients required liver transplantation for bemgn. highly s)mptomatic hepatic neoplasms that were unresectable. Origins included ad-enoma (n=6). mesenchymal hamartoma (n=2), massive Conclusion: Patients with severe symptoms from be-mgn hepatic neoplasms that are not resectable can be treated by total hepatectomy and orthotopiC liver transplanta-tion, ..... ith the expectation of good long-term results. From lire Department of Surge". Unl\'!rSIl\ oj mfffsbur~h (Pa). PHlsburg" T ransr/anl '"SillYit' (Arch Surg. 1995: 130: 153-156) 5 T:\'.;O:\RO (:"JOleA TIO;\S for or-thotopiC liver transplanta-tion Include chrOniC end-stage liver disease. fulminant hepatiC failure, inborn er-rors of metabolism, and cholestatic liver disease. t .b IncreaSingly, patients with pri-mary malignant neoplasms of the liver un-dergo transplantauon after neoadjuvant chemotherapv.; 8 Rarely, benign hepatiC le-sions require li\'er transplantation If they cause debilitating or life-threatening mani-festallons or If they are very symptomatic and too large to be resectedK~ Isolated case reports of liver transplantation for be-nIgn hepatic tumors have been pub-Iished. lll . t , \'v'e report herein our enme experience of lIver transplantation for benign liver tumors. Liver transplanta-tIOn ..... as conSIdered the treatment of choice for bemgn liver tumors In highly symptomatic patients wah potentially life-threatening complications if (I) liver resecllon was precluded due to the extent or strategic localization of the tumor or (2) hepatic reseCllon had been performed and was Inadequate to con-tral recurrent symptoms. Five patients were children. aged of to 17 ~Dears (mean. 8.6 years): one was an in-fant, aged of weeks; and the remaining six were adults, aged 20 to 47 years (mean, 36.3 years). Nine patients were female (7 5(},,). and three were male EOR~IIF In six pallents. the clinical and histological char-actenstics of the tumor were tYpIcal of he-patic adenoma. In two patients. the liver pathologiC specimens revealed mesenchy-mal hamartoma. and the remaining tu-mor origins were cavernous heman-gIOma. massive lvmphanglomatosls. hilar fibrous angiodysplasla (inOammatory pseudotumor), and focal nodular hyper-See Pariatts and MtChods on n~xl pag~ PATIENTS AND METHODS ;\5 of January 1993. 12 pallents had undergone ortholO-pIC Itvcr transplantatlon for benign hepatlc neoplasms at the Lnlversity of Pittsburgh IPJ) \1edical Center. Data were collected on all pauents regarding age. sex. nature of dis-ease. dates of diagnosIS. magnitude of the clmical mantfes-tations. previous Ii\'er surger\'. and posmansplantatlOn course. The histopathologic features of the leSIOns were con-firmed after review of the surgIcal pathologIC specImens (Talt •• ). HEPATIC ADENOMAS Six female patients (five of rc:protiu(tiW age and thrce wnh a htstory of oral contraceptive use) presented with adeno-mas "'lith recurrent intraparenchymal or intra-abdominal bleeding The onl\' premenstrual-age female. a lO-l"ear-old gIrl. had a family history of hepatlc adenomatosIs. In one of the olher patients. the multiple adenomas were related 10 type I glycogen storage dIsease. and the chmcal course was complicated by liver fallure as well as bleeding. Five of Ihese patients had undergone prc\'lOUS ana-tomICal or minor liver resectlons. which proved to be in-adequate. \IESENCHYMAL HAMARTOMAS Two patients \\.;th mesench~mal hamartomas suffered from Intractable abdominal pain and progressive liver failure. Each had undergone prior laparotomy for biopsy and debulking. HEMANGIOMA .l,n mfant presented with a gIant cavernous hemangIOma Involving the entire live •. \\'1th abdominal dIstention and the bleeding tendency of the Kasabach-Merritt syndrome plasia. There were three postoperative deaths. Two pa-tients received a liver allograft in an emergency setting. and both died. One was the infant suffering from a giant cavernous hemangioma. Kasabach-Merritt syndrome. and associated systemic coagulation disorders. This patient survived the operation. but primary nonfunction of the graft developed. and he died 8 days later of an intraven-tricular hemorrhage. The other emergency transplanta-tion was in a 43-year-old woman with a large hepatiC ad-enoma causing recurrent intraparenchyrnal bleeding. After left-sided trisegmentectomy, the remaining healthy pa-renchyma was inadequate to sustain life. Emergency liver transplantation was performed. and necrotizing pancre-atitis resulted in the patient's death. The third fatality oc-curred in an elective case. that of a 4-year-old boy with a giant mesenchymal hamartoma nearly replacing the liver parenchyma who died during the operation of hemor-rhagIc shock. Of the nine patients who survived the early post-operative period. one died 56 months later of dissemi-nated aspergillosis. A second patient died 84 months later of candida I sepsis after her second orthotopic liver trans-Ilc'Clli:cd clollmg and fthrinol\'SIS wllhin the heman-gwma. assoclaled with systemIC fibrInolysIs),' \ iv~fmeAkdflMAqlplp Onr patient had massive diffuse hepatlc lvmphangloma-tl~fp and preseOled wilh lh'er failure. d\·spnea. and deblh-latlng pam. Four years pnor to referral. she had under-gone ,;plenectomv for hypersplentsm related to splenic hmphanglOmalOsis. and al that tllTTe h~pallc Iymphangl-omalOSlS was nOled. HILAR FIBROUS ANGIODYSPLASI." I nflammatorv pseudotumor IS a rare leSIOn ..:haraclenzed by proliferating fibw"ascular tissue mixed with inflammatory cells. II has been descnbed mostly In children and in a \\ide variety of anatomic locations. I! Recurrent cholangitis. por-tll hypcrtcrtSion. and secondarv biliaf\' cirrhl)sls due 10 the extensIve mvol"ement of the liver htlum resulted In the ne~d for liver replacement in an 8-ycar-old girl. FOCAL NODULAR HYPERPLASIA The patient with focal nodular hvperplasla complained of Intractable pruntus. fatigue. jaundice. and progressive lIver failure due 10 replacement of the hepatic parenchyma by tumor. In all cases. lh'er transplantation was performed ac-cording to the standardized surgical techntque elucidated by StaTZl and Demetris' and Iwatsuki et al. l In spite of the enormous size oflhe livers in most of these cases (.Ig_>. the bilateral subcostal and upper vertical midline incision without a thoraCIC extertSion pro\;ded adequate exposure when a bilateral subcostal retractor was used. Eleven pa-[{ents received posuransplantauon immunosuppressive therapy based on cyclosporine and steroids. while an-other was treated with tacrolimus (PrograO. plantation for hepatitis C-associated cirrhosis. The re-maining seven patients are alive, with a follow-up pe-riod ranging from 36 to 145 months (median, 88 months). Multiple lung lesions developed in one of the patients undergoing transplantation for adenoma 9 years later. Needle biopsy of the lesiortS revealed hepatocellular car-cinoma, and although there was no evidence of malig-nan! neoplasms in the pathologic specimens of the na-tive liver, it is probable that the malignant tumor arose from an undiscovered focus in the original adenoma. She is currently alive with disease. Benign hepatiC neoplasms have been considered a rela-tively rare entity, but thdr increased recognition can be attributed to advances in diagnostic imaging technol-ogy. a Incidental liver lesions can be safely observed as long as benign histological characteristics and clinical be-havior are apparent, and the lesion is under radiolOgiC observation_9 However, with the exception of hemangiO-mas and possibly focal nodular hyperplasia, lesioTlS larger ARCH SL'RG"iOL 130. fEB IW5 154 , I .' r . .-Patleals With Benlg. Hepltlc Tumof1 Trnted by Ortllotoplc lIYIr Transplantation ........... PllstlnllllllllntlCfoR PrnIoua Uwr III",,, a.nIvIl Status ~:DfD ." :' ., . :'.. ,.... WJaI Hepatic AdtAOmU ::. ':;. :'1 ~D-K . 3 Y Exploratory laparotomy 17ylF 20ylf 31 ylF 35 ylF 43 ylF Adenomas Intraparenclr(maJ andlor inlIdperitoneal hemorrtlaoe 2y 5y 2y 15 Y Smo (x2) Portacaval shunt Left lateral 5e9mentectomy Right triseomentectomy Exploratory laparotomy (x2) Left hisegmentectomy PlIUents WltII Miscellaneous Tumors Pain. re5pir1tO!y distress. liver failurl 2 y Biopsy and attempted • yJ\t Mesenchymai hamartoma 4 wtVM Cavernous 8yIF 1,2 ylF hemangioma Hilar fibrous . anoiodysplasia (inflammatory pseudo!umoc) Diffuse focal nodular hyperplasia Pain. liver failure. portal hypertension Kasabadl-Merritt syndrome Recurrent choIanoitis. portal hypertension Pain. pruritus. jaundice 2y 4wk o4y 3mo Sy than 3 em should be excised, especial Iv when the histo-lOgIcal characteristics of the tumor are ambiguous. or there is a considerable risk for complications. [n extreme cases. a curative excision of the tumor cannot be accom-plished due to the extension of the lesion in the liver pa· renchyma and its bilobar location [n such cases. a vari-ety of palliative therapeutic strategies have been proposed. including radIOtherapy and hepatic artery ligation or em-bolization. but good long-term results are rareK~11 Liver transplantation has been used successfully in sporadi-cally reported cases of benign hepatic neoplasms com-plicated by life· threatening manifestations. as well as for treatment of the diffuse benign cystic leSIOns of polycys-tic liver disease in panenrs with poor quality of life due to liver size UR.M., Giorgio Zetti. MD. [gnazio Marino. MD. Saloru Todo. MD. AndreasG. Tzakis. MD, Guiseppe Camen. MD. S.I.. T.E.S .. John J Fung. MD, PhD, un-published data. 199PFKn·f!fSKI~ resection Oebulkino (x 2) Splenectomy EJ(j)loratory laparotomy Exploratory laparotomy 65mo 145 ma 101 ma 130 ma 36mo 10 d 58 ma 8d 88mo 56mo 55 me Alive Alive Alive Alive Died 01 necrotizing pancreatitis Alive Intraoperative death Died 01 coaoulopathic cerebral hemormaoe ... : Alive Died of disseminated aspergillosis Alive [n the cases we report herein. total hepatectomy was Indicated because of failure of alternative thera-pies to control the disease and relieve ItS symptoms. Eleven of 12 patients had already undergone at least one laparotomy to treat the disease. with eventual fail-ure. Five of the six patients in the adenoma group underwent urgent operation because of intraparenchy-mal and/or Intraperitoneal hemorrhage. Three of the six underwent anatomical liver resection to eradicate Masslw IItp.Jtrc IymphanglomatcJSls and stlf}mara 01 portal hypertension. the disease. with eventual recurrences. The oln-ious rtsk for a life-threatening hemorrhage necessitated total hepatectomy and liver replacement in these patients. The median interval between the primary diagnosis of adenoma and transplantation was 36 months. During this time period. sustained growth and recurrent hemorrhage was the rule. and death was Imminent for all of these patients Y.llhout transplanta-tion. For this reason. unresectable hepatic adenomas that arc associated wllh hemorrhage and./or continued growth shl)uld be an indication for liver replacement. Often. the course of these aggressive lesions can be anticipated at the outset of S\l"nplOms. and the patients undergo elective transplantation before extreme cir-cumstances exist. The remaining six patients presented uniformh" y.;th abdomll1ai pain. malaise. faugur. and other manift:sla-tions related lO liver size and the extensive replacement of hepatiC parench\l"na by the neoplasm. All six patients had a ver; poor quality of life and had suffered poten-tially lethal complications. U\'er transplantation can be conSidered the treatment of choice for symptomatic be-l11gn hepatic neoplasms that are extenSively replacing the liver parenchyma. ------1."# .. 11[·*_1------Excellent long-term outcome after elective liver trans-plantation [or large. life-threatening. benign hepatiC le-sions should prompt consideration of this therapeutic op-tion in difficult cases. Reprint requests to 4th Floor Falll Clinic. 3601 Fifth Ave. Pittsburgh. PA 15213 (Dr Sciby). 1 Slarzl TE. Oeme\rls I>J. Liver Transplantation. St LoUIS. Mo: Mosby-Year Book: 1990119-130 Plchlmayr R. Ringe B Laucnart W. Wonlgell K. liver transplantation Trans-plant Proc. 1987:19103-112. 3 Iwatsukl S StaTZI TE. Todo S. et al. Expenence In 1000 liver Iranselants under cycloSPOTine-stero.d therapy' a surMal report. Transp/anr ?roc. 1988.20 (SUDPI 11498-504 Samuel O. 9~nnamcn JP. Bismuth H. Guggennelm J. Clardullo M. Saliba F. en-lena of selection lor liver Iransplantallon Transplant Proc. 1987192383-2386. 5. B.smulh H Samuel 0 Guggennelm J. Castalng D. Bernuau J. RueH B. Emer-gency liver lransplantatlon for fulminant lJ,epahtls. Ann Intern Mea. 1987.107 337-341. ESQUivel CO Iwalsuki S. Gordon RD_ Marsh JW, Koneru B. Makowa L. Indi-callons for pelllatnc liver transplantation. J Pedlatr. 1987:3:1039-1045. YOkayama I. Todo S. Iwatsuki S. Staal TE. Liver transplantation in the treat-ment of pnmary liver cancer. Hepatogastroenterology. 1990:37:188-193. 8 Iwatsuki S. Starn TE, Sheanan DG, et al. Hepatic resection versus transplan-tation for hepatocellular carcinoma. Ann Sun) t 991 :214:221-229 Iwatsukl S Todo S Starn TE Excislonal therapy for benign hepallc lesions Surg GyneCQi OOsret. 1990: t 71.240-246. 10 Miller C, Mazzaferro V. Makowlta L. et al. OrthotOPIC liver transplantation for massive nepatlc Iymphanglomatosis. Surgery 19UU:1MP:49M-~9RK 11 Klompmaker I. Siooff M. van der Meer J, de Jong GM. de Sruqn KM. 6ama JL OrthOtopIC Irver transplanlatlOn In a patient With a giant cavernous hemangioma 01 the liver and Kasaoach-Mernn syndrome. TransplantatIOn 1989.48 1 ~9-1sM 12. Hemeghan MA, Kaplan CG. Pnebe CJ, Pnebe CJ Jr. Partin JS Inflammalory pseudotumor at tne liver a rare cause at obstructive faundlce ana portal hv-pertenslon In a child Pedlarr Radiol. 1984:14:433-435. 13 Kasabach H. Mernn K. Capillary hemangioma wltn extenSive purpura. reeorl 01 a case. AJDC 1940:59:1063-1070. 14 Nicnolas F III. van Heerden J. Wellancll. Benign liver lumors. Surq Clin North Am. 198969297-314 15 Belli L. DeCarlis l. Beatl C. RondlOara G. San salone V. BramOilla G Sur_,cal treatment of symptomatic Qlant hemangiomas of the liver. Surg Gynecol Oll-stet 1992:174:474-478. 16 Manno I, Scantlebury V, Bronslher O. fwats~kl S. Slarzl TE. Tolal Mepalec:cmy and liver transplantations tor hepatocellular adenomalosls and focal nodular hyper1)IaSIa. Transpllnt. 1992;5(suppf 1 ):201-205. 17. Sefby R. Starn TE, YUOlS E, Todo S. Tzakls AG, Brown BI. liver transplanta-han tor type I and type IV glycooen storage disease. Eur J Pediatr. 1993: 152 (suppl1):71-76. 18. Starzl TE. Reyes J, Tzakls A, Mieles L. Todo S, Gordon R.liver transplan\;ltlon for POlycystiC disease. Arch Surq 1990:125:575-577 SUlgiml ""alolllY 5 tructures entering the adductor canal are the (1) femoral anery and vem. (2) profunda artery and vein. (3) nerve to the vas IUS medialIs. and (4) saphenous nerve. artery. and vein. 1. Hollonshtad WHo Anatomv fur 5urgt"MI Phlladelph ••. P. Hlltber-Harper: 195-1: 393 .... RCH St.:RGNOL DO. FEB 19q~ 156 ........................................ 

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Orthotopic Liver Transplantation for Benign Hepatic Neoplasms

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