CTandSonography of Multiple Bile-Duct Hamartomas Simulating Malignant by air20214

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CT and Sonography    of Multiple Bile-Duct Hamartomas
Simulating Malignant Liver Disease (Case Report)
Danny         Eisenberg,1       Lawrence             Hurwitz,2      and        Albert    C.   Vu3



    Multiple  bile-duct       hamartomas       are a relatively  common                             logic reconstruction       [1 4J of this lesion reveals that each
                                                                                                                                                  ,


incidental   autopsy       finding [1 ], yet they are almost unknown                                hamartoma       contains    a single ramified    lumen. Some authors
in the radiologic       literature    [2]. We present      a case of this                           [1] believe      that there is no communication            to the biliary
benign entity that resembled             malignant  disease on both CT                              system,     although     a recent    report   [4] states     that such a
and sonography.                                                                                     communication        does exist and that it obliterates     over a period
                                                                                                    of time. In any case, when communication                 does exist it is
                                                                                                    microscopic       and not widely patent as in Caroli’s disease.
Case     Report
                                                                                                               Bile-duct       hamartoma              is well     known         in the     pathologic         litera-
   A 70-year-old       black man presented             with a 2-year history of right-              ture [1 5] and was an incidental
                                                                                                                      ,                      finding in 0.6%                                             of 2000
upper-quadrant     pain without           weight loss. Past history             included es-        biopsies  [1] and in 2.8% of 707 autopsies         [1]. The                                            size of
sential hypertension,   chronic           obstructive  lung disease,              alcoholism,       these cystic structures     usually ranges from 0.01 to                                               0.5 cm
and vagotomy         and pyloroplasty       for peptic ulcer.                                       [1 4, 5], although
                                                                                                           ,             occasionally   they may be as large                                             as 1 cm
   Physical      examination        and laboratory      findings    were       unremarkable         [5].         Macroscopically,              they      appear        as grayish         or black      nodules.
except for mild elevation of liver enzymes. Chest radiograph, barium
                                                                                                       There is a well-known          association       between      multiple    bile-
enema, upper gastrointestinal    series, excretory urogram, and upper
                                                                                                    duct hamartoma         and polycystic       liver disease.      In a series of
panendoscopy    were all negative for malignancy. Sonography (Fig. 1)
and CT (Fig. 2) of the liver demonstrated               multiple   irregular     hypoechoic
                                                                                                    70 cases of polycystic       liver disease found at autopsy,             Melnick
and hypodense         structures,     respectively,     measuring       up to 1 cm in size          [6] found 29 cases of coexisting              multiple   bile-duct     hamarto-
and situated in both lobes of the liver. In addition,            larger round and                   mas. One theory is that the bile-duct             hamartoma        arises when
smooth typical liver cysts were also visualized.            Using sonography,        a              embryonic     bile ducts fail to involute, and that the larger cysts
biopsy of the posterior     right lobe of the liver was obtained with a 14-                         of polycystic    liver disease result from gradual dilatation              of the
gauge Tru-Cut     needle (Travenol          Lab, Inc, Deerfield,     IL). It demon-                 hamartomas.        Ohto and Vshio [4] hypothesize             that as a result
strated multiple cystic structures           located   by the portal zones that                     of this           cystic     dilatation,          obliteration           of the      communication              to
were lined with bile-duct          epithelium      and embedded         in a fibrous                the biliary          occurs.
                                                                                                                           system
stroma. Most of these dilated ducts measured               1 00-200     m (0.1-0.2
                                                                                                       Most authors    [1 5] believe that neoplastic
                                                                                                                                         ,             transformation
mm) in diameter (Fig. 3A). Some were larger (Fig. 3B), measuring                  the
                                                                                                    of bile-duct hamartoma       does not occur. However,    one report
maximum     size obtainable      from a Tru-Cut biopsy specimen-2                 mm
                                                                                                    [7] describes                two    cases          of benign            adenomatous           transforma-
(2000   sm). A diagnosis             of multiple      bile-duct    hamartomas           or von
Meyenburg     complexes    was made. Repeat       CT, sonography,       and                         tion and one case of adenocarcinoma                                        originating       from the site
biopsy of the liver 6 months later demonstrated     identical findings. At                          of a bile-duct                hamartoma.              This       last    case     is debatable,           since
follow-up  1 year after the initial workup,   the patient was well and                              there was concurrent              adenocarcinoma       of the pancreas          that
without evidence of malignancy.                                                                     was of different       histologic     appearance.
                                                                                                        We could find only one reference               to bile-duct     hamartoma
                                                                                                    in the radiologic         literature     [2]. The authors        described        its
Discussion
                                                                                                    angiographic        appearance        as including    “multiple   areas of ab-
   A bile-duct     hamartoma,        also known        as              von Meyenburg                normal vascularity         approximately       1 cm in diameter        persisting
complex,     is a proliferation       of bile ducts                  lined by normal-               into the venous phase. In several areas they took the form of
appearing    epithelium      set in a fibrous stroma                  that is frequently            grape-like     clusters of small rings 2-3 mm in diameter.                 Tumor
hyalinized    [1]. Bile-duct     hamartoma       should               not be confused               vessels,     laking and arteriovenous              shunting     were not ob-
with mesenchymal          hamartoma        of the liver,               which is a com-              served.”
pletely different     and larger growth found in                    infants [3]. Histo-                    The CT and sonographic                           findings         in the current          report      and


   Received February 3, 1986; accepted after revision April 6, 1986.
   ‘ Department  of Radiological Sciences, University of Califomia, Irvine Medical Center, 101 The City Dr., Orange, CA 92668. Address reprint requests                                                         to 0.
Eisenberg, Attn. Karl.
   2Depaitment   of Gastroenterology,  University of California, Irvine Medical Center, Orange, CA 92668.
   3Department of Radiology, Long Beach Veterans Administration Medical Center, Long Beach, CA 90822.
AJR 147:279-280,       August   1986 0361 -803X/86/1         472-0279      C American Roentgen Ray Society
280                                                                         EISENBERG      ET AL.                                                         AJR:147, August 1986



                                                                                                                                       Fig.      1-Transverse            sonogram
                                                                                                                                    through right lobe of liver. Scattered hy-
                                                                                                                                    poechoic lesions measuring up to 0.5cm
                                                                                                                                    in diameter (arrows).




                                                                                                                                        Fig. 2.-Contrast-enhanced        CT of
                                                                                                                                    right lobe of liver.Multiple irregularhypo-
                                                                                                                                    dense lesions of variable sizes up to 1 cm
                                                                                                                                    in diameter.  Note larger, rounded   typical
                                                                                                                                    liver cysts.



                                                                                                                                        Fig. 3.-Low-power       view of entire
                                                                                                                                    width of wedge liver biopsy. A, Typical
                                                                                                                                    (0.1-0.2 mm) hamartomas.       B, Larger (2-
                                                                                                                                    mm) hamartoma (arrows). Partial collapse
                                                                                                                                    of lesion due to preparation.




the angiographic        findings     in the previous             report    are rare,       REFERENCES
extensive,     macroscopic       manifestations           of a common,             pre-
                                                                                           1 . Thommesen        N. Biliary hamartomas   (von Meyenburg complexes)
dominantly      microscopic      entity, accounting             for its more fre-
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follow-up. All these were found in the case reported.                      Definitive          burg’s complex       on the liver surface. Endoscopy              1984;16:71-74
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elsewhere     in the body. Therefore,         it should be considered               and       Pathol 1955;59: 162-172
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