Liver cysts can be single, multiple or diffuse, as in polycystic liver disease.

Once found, there is a

need to distinguish a simple cyst from other cystic lesions of the liver including hydatid cysts and
cystic neoplasms.
Simple hepatic cysts are common (10%) benign liver lesions and have no malignant potential. They can
be diagnosed on ultrasound, CT, or MRI. Simple hepatic cysts may be isolated or multiple and may vary
from a few millimeters to several centimeters in diameter. Simple hepatic cysts are benign developmental
lesions that do not communicate with the biliary tree 2. The current theory regarding the origin of true
hepatic cysts is that they originate from hamartomatous tissue. On histopathological analysis, true hepatic
cysts contain serous fluid and are lined by a nearly imperceptible wall consisting of cuboidal epithelium,
identical to that of bile ducts, and a thin underlying rim of fibrous stroma 2.

Certain diseases are associated with multiple hepatic cysts and include

polycystic liver disease

autosomal dominant polycystic kidney disease (ADPKD): hepatic cysts may be seen in ~40% of those
with ADPKD 2.
von Hippel Lindau disease

Polycystic liver disease (PCLD) is a hereditary condition that may arise either in patients with autosomal
dominant polycystic kidney disease (ADPKD) or in patients with a different genetic mutation that results
solely in autosomal dominant polycystic liver disease. It is characterized by progressive development of
fluid-filled biliary epithelial cysts throughout all segments of the liver. The course of polycystic liver disease
is variable but progressive. In patients with ADPKD, the number and size of cysts increase with advancing
age 1.

The diagnosis of PCLD is made when more than 20 liver cysts are scattered throughout the
liver. If the patient comes from a family with inherited PCLD, the diagnosis can be made if
more than four liver cysts are present. To establish the cysts, abdominal ultrasound and CT-
scan are helpful.6870 The MRI is an even more sensitive diagnostic tool.59 There are no
specific laboratory-test abnormalities in PCLD. Liver function and synthesis capacity are
maintained during all the stages of the disease. Gamma glutamyl transferase and alkaline
phosphatase may be normal or slightly elevated. Serum transaminases are also usually
normal or only mildly elevated.71 The CA 19.9 may be elevated (in 45%) without proof of
malignancy due to CA 19.9 production by the biliary epithelium.72
Most of the patients with PCLD are asymptomatic, however, in some patients (3%),
expansion of liver cysts causes invalidating abdominal symptoms.
The most common complication in patients with PCLD is extensive hepatomegaly. As
polycystic livers can grow up to 10 times their normal size, they compress adjacent
abdominal and thoracic organs. Symptoms typically caused by massive enlargement of the
liver include abdominal distention, early satiety, postprandial fullness, gastro-oesophageal
reflux, dyspnoea, mechanical low-back pain, which will induce severe malnutrition in some
patients.
Women who have had multiple pregnancies or who have used oral contraceptive drugs or
oestrogen replacement therapy have worse disease.1, 45, 46
Cholangiocyte proliferation/apoptosis and enhanced fluid secretion are key factors in the
pathophysiology, but the exact pathophysiology of polycystic liver disease still remains
unclear
In symptomatic patients, none of the currently available surgical options except liver
transplantation have been shown to change the natural course of the disease. The use of
somatostatin analogues has been shown to diminish liver volume.
The natural history of PCLD, regardless of aetiologic mutation, is similar and suggests a
continuous increase with age in the volume of the affected polycystic liver. This is due to the
increase in size and number of the cysts.44, 45 Data indicate that the annual growth of
polycystic livers is 0.93.2%.10, 11, 6062 Other risk factors for liver-cyst growth are renal-cyst
volume and the severity of renal dysfunction.
Other complications caused by mass effect and excessive fibrocystic disease include
hepatic venous-outflow obstruction (Budd-Chiari Syndrome), inferior vena cava syndrome,
portal-vein compression and bile-duct compression; which rather are infrequent.
Complications of the liver cysts themselves include infection, torsion, rupture or
haemorrhage.1, 26, 63, 64 However, the primary cause of morbidity in ADPKD patients is the
severity of the underlying renal cystic disease and loss of renal function.
It should be emphasised that the PCLD are not associated with an increased risk of
developing malignancy.
Polycystic liver diseases (PCLD) represent a group of genetic disorders in which cysts occur
solely in the liver, or together with renal cysts, as in autosomal dominant polycystic liver
disease (ADPLD) or are combined with cysts in the kidneys as well, as in autosomal
dominant polycystic kidney disease (ADPKD).
Although genetic testing for ADPKD and PCLD is possible, it is rarely performed because it does not
affect the therapeutic management of PLD

Mutations in four distinct genes have been linked to human PCLD. Two of these
genes, PKD1 and PKD2, are associated with ADPKD, where PCLD is the most prevalent
extra-renal manifestation. The two others genes, PRKCSH and SEC63, are found in patients
with ADPLD, which displays no renal involvement.1, 1115
The ADPLD is characterised by the presence of cysts in the liver and only few or no renal
cysts. The prevalence is estimated to be 1/100 000. To date, the disease is known to be
caused by mutations in two genes accounting for approximately one-third to one-half of
ADPLD cases: PRKCSH (locus 19p13.2), encoding the protein kinase C substrate 80K-H or
hepatocystin (527 amino acids, 59 kDa); and SEC63(locus 6q21), encoding the Sec63
protein (760 amino acids; 88 kDa).
The relationship between adult polycystic liver disease (PCLD) and biliary
microhamartomas (von Meyen- burgs complexes) was studied in a series of ten autopsy
cases in which the size of the cysts vaned from microscopic to 10 cm. Biliary
microhamartomas, found in every case, were the most abundant in livers with cyst size of I
cm or less and had almost disappeared in cases with the largest cysts. The number of
microhamartomas had no significant correlation with age or liver weight. The results
indicate that biliary microhamartoma (von Meyenburgs complex) is coupled with adult-
type polycystic liver disease, and, probably, is the histopathological lesion that transforms
into cysts. The number of micro- hamartomas is the highest in the early phase of the
disease.
Strategies:

Reduce levels of estrogen, weight management as estrogen increases liver cysts and total liver
volume
Somatostatin inhibits fluid secretion and proliferation by reducing [cAMP]cyt in cholangiocytes.
Both randomised trials showed that the beneficial effect of somatostatin analogues was also
associated with an improved general-health perception. The optimal dose and the effects on
the long run of somatostatin analogues in PCLD still remain unclear.
Somatostatin, also known as growth hormoneinhibiting hormone (GHIH) or by several
other names, is a peptide hormone that regulates the endocrine system and
affects neurotransmission and cell proliferation via interaction with G protein-
coupled somatostatin receptors and inhibition of the release of numerous secondary hormones.
Somatostatin inhibits insulin and glucagon secretion

Octreotide or lanreotide 120 mg, a somatostatin analogue, prevented the outgrowth of

kidney and liver cysts in PCK rats, a PKD rodent model.7