Hepatic adenomatosis – a rare double complication of ... - medIND

Case Snippets
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Correspondence to: Dr Kandaswamy. E-mail:
kgvignesh@yahoo.com, kgvignesh@gmail.com
Received October 7, 2005. Accepted December 25, 2005
Hepatic adenomatosis – a rare double
complication of multiple adenoma rupture
and malignant transformation
Nitin Arvind, Duraimurugan D, J S Rajkumar
Department of Surgical Gastroenterology,
Lifeline Rigid Hospitals, Kilpauk, Chennai
Hepatic adenomatosis (HA) is a rare disorder that is
susceptible to hemorrhagic complications and, rarely,
to malignant transformation. We report a 24-year-old
woman who was found to have HA with >10 tumors;
she presented with simultaneous rupture of two of
the adenomas, along with malignant change in one.
Hematoma evacuation and caudate lobe resection were
done, and she has been doing well 6 months later.
[Indian J Gastroenterol 2006;25:209-210]
Hepatic adenomatosis is a condition with presence
of multiple adenomas in the liver, variously defined
as more than 4 adenomas 1 or more than 10 adenomas. 2
In contrast to solitary adenomas, adenomatosis shows
no association with the use of oral contraceptives,
anabolic steroids and type I glycogen storage disease.
Increase in size and propensity to rupture during
pregnancy is also not known with hepatic adenomatosis.
The potential for spontaneous bleeding and malignant
transformation are, however, common to both.
A 24-year-old woman was referred with history of
spontaneous, sudden-onset abdominal pain of 12 days'
duration. Her abdomen was soft, with palpable liver and
epigastric and right hypochondrial tenderness. Ultrasonogram
showed fluid in the pouch of Douglas. Culdocentesis
showed hemorrhagic fluid. Coupled with a history of
delayed menstrual cycle, a provisional diagnosis of ruptured
ectopic gestation was made. At laparotomy, approximately
900 mL of altered blood was found in the peritoneal
cavity and the fallopian tubes were found to be normal.
The surface of the liver was irregular and nodular
and a large hematoma was seen over the posterior superior
surface of the right lobe. The clots in the peritoneal
cavity were evacuated, a wash given, and the abdomen
closed. She was then referred to us for management.
The patient gave no history of recent trauma or usage
of oral contraceptives. She had been operated on for
atrial septal defect 10 years previously and had had a
caesarean section 2 years back. On examination, she had
an enlarged, tender liver. Her vital signs were stable; red
and white blood cell counts were normal and liver function
tests were normal, except for raised alkaline phosphatase
(250 IU/L; normal: 30-180) and gamma glutamyl
transpeptidase (276 IU/L; normal: 5-80).
MRI showed a 14 cm × 12 cm lesion involving the
posterior superior aspect of the right lobe of the liver,
minimally hyperintense in T2-weighted images and
hypointense in delayed images with inhomogenous early
enhancement. There was evidence of fat within the lesion
along with multiple areas of hemorrhage. There was another
rounded lesion with similar signals in the caudate
lobe with surrounding hemorrhage. About 8 smaller lesions
with similar signals were seen in the right lobe, the
largest measuring 4 cm. Another 5-cm lesion was seen in
segment II of the left lobe.
Soon after admission, the patient again developed
signs of peritonism. At laparotomy, the liver was found to
be studded with rounded, nodular swellings. A large subcapsular
hematoma was seen over the region of segment
V with a rent in the capsule over it, and another subcapsular
hematoma over the caudate lobe. The hematoma was
evacuated and non-anatomical resection of the caudate
lobe adenoma along with surrounding liver parenchyma
was done along with segmentectomy V (Fig). The patient
responded well to surgery. On follow up she has not shown
complications or increase in size of the remaining adenomas.
Histology suggested changes consistent with adenoma
in the caudate lobe specimen. The larger specimen
showed moderately differentiated hepatocellular carcinoma
(grade II/III of Edmondson and Steiner) whereas the smaller
adenoma that had ruptured showed no such change.
The conditions that predispose to hepatic adenomatosis
are poorly understood. One speculation
has to do with congenital or acquired abnormalities
Fig: Operative photograph after segmentectomy 5. Nodularity
of rest of liver is also seen
Indian Journal of Gastroenterology 2006 Vol 25 July - August 209

of the hepatic vasculature. 3 Adenomas are
hypervascular tumors containing multiple sinusoids
of capillaries with thin walls that are perfused exclusively
by the high-pressure hepatic arterial flow.
Due to poor connective tissue support, there is a
predisposition to bleeding. Malignant change, although
rare, is also known to occur.
They are usually asymptomatic and are diagnosed
incidentally, but occasionally present with
abdominal pain, hepatomegaly or altered liver function
tests. They can present with catastrophic intraperitoneal
bleed. Ultrasonography, CECT or MRI can
be utilized in the diagnosis, with MRI proving to be
the best bet. On unenhanced CT, they are hypodense,
but lesions with hemorrhage appear hyperdense or
heterogeneous. These lesions show significant enhancement
on arterial phase images as they are supplied
by the hepatic artery. The presence of a capsule
and demonstration of intralesional fat on outof-phase
T1-weighted images are helpful in diagnosis
on MRI. Fine needle biopsy has the dual drawback
of precipitating bleeding, and seeding of the
needle track if the mass turns out to be malignant.
Hepatic resection is indicated in the presence of
a major complication such as intraperitoneal bleed,
but its usefulness in the uncomplicated patient has
been debated. The consensus is that larger and more
vulnerable tumors (>5 cm, subcapsular, exophytic
and hemorraghic) need to be resected, 4 while smaller
asymptomatic tumors can be monitored. Orthotopic
liver transplantation may be used in patients who
have progressive symptoms after partial resection,
or in whom carcinoma is suspected. 3
References
1. Wiener Y, Dushnitzky T, Slutzki S, Halevy A. Synchronous
bleeding of liver adenomatosis and possible relation to
acoustic trauma. HPB Surg 2001;3:267-9.
2. Chiche L, Dao T, Salame E, Galais MP, Bouvard N, Schmutz
G, et al. Liver adenomatosis: reappraisal, diagnosis, and
surgical management: eight new cases and review of the
literature. Ann Surg 2000;231:74.
3. Grazioli L, Federle MP, Ichikawa T, Balzano E, Nalesnik
M, Madariaga J. Liver adenomatosis: clinical, histopathologic,
and imaging findings in 15 patients. Radiology
2000;216:395-402.
4. Montano-Loza A, Rios-Vaca A, Remes-Troche JM, Meza-
Junco J, Trinidad-Hernandez S. Hepatic adenomatosis in a
Hispanic patient – a case report and review of the literature.
Ann Hepatol 2002;1:136-9.
Correspondence to: Dr. Arvind, E47/2, 3rd Street, Annanagar
East, Chennai 600 102. E-mail: drnitinarvind@gmail.com
Received October 17, 2005. Accepted December 25, 2005
210 Indian Journal of Gastroenterology 2006 Vol 25 July -