17 Benign Liver Tumors

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Chapter 16 / Neuroendocrine and Noncolorectal Liver Metastases 201

poorly dierentiated NEC is typically associated with rapid progression and

death.

Numerous targeted agents have been used to treat patients with

metastatic NEC. Typically, NECs express vascular endothelial growth fac-

tor (VEGF) and its receptor (VEGFR) making them candidates for antian-

giogenic agents such as sunitinib (tyrosine kinase inhibitor with activity

against VEGFR) or bevacizumab (a VEGF antibody). Trials have demon-

strated either disease stability (in most patients) or objective partial regres-

sion (in a minority) with anti-VEGF therapy. e mTOR pathway is altered

in some NECs, particularly those pancreatic in origin. Everolimus, an orally

active mTOR inhibitor, has shown ecacy in low- to intermediate-grade

NEC as demonstrated in the multicenter RADIANT Trial. Everolimus has

been less eective in nonpancreatic NEC. Other agents (getinib, imatinib

temsirolimus, bortezomib, etc.) have been used to treat NEC patients, but

signicant and durable tumor responses are rarely obtained (< 10%) with

targeted agents alone. us, targeted agents are not typically considered

rst-line therapy and may be more eective as a chemotherapy adjunct.

Treatment Follow-up Evaluation

Current data show that despite surgical and medical therapy, disease pro-

gression occurs in more than 80% of patients with NEC hepatic metas-

tases. Given the high rate of recurrence following potentially curative

resection or progression following palliative procedures, continued sur-

veillance is warranted. Typically, follow-up is based on serial abdominal

imaging with CT or MRI and serum biomarkers (chromogranin A, neuron-

specic enolase, and other specic neuroendocrine peptides [if functional

NEC]) approximately every 3 to 6 months for low-grade NEC and every

2 to 3 months for high-grade NEC or in patients with documented symp-

tom recurrence or progression. Functional (SRS) whole body imaging is

performed only if the extent of the metastatic disease would aect liver-

directed therapy.

HEPATIC METASTASES FROM NONCOLORECTAL,

NONNEUROENDOCRINE MALIGNANCIES

As a common site for metastatic spread of most solid tumors, metastases

apparently conned to the liver will frequently arise from noncolorectal,

nonneuroendocrine malignancies. Given the benets realized after resec-

tion of colorectal and neuroendocrine metastases, hepatic resection may

FIGURE 16.7 (Continued) C. Gross specimen of resected liver tumor.

Section II / Liver202

be appropriate for some patients with noncolorectal, nonneuroendocrine

metastases. e low frequency of liver-limited disease and the wide

variation of underlying histopathology and tumor biology make outcome

prediction dicult in this patient group as a whole. In general, resection

of hepatic metastases from noncolorectal, nonneuroendocrine malignan-

cies may be considered if the primary and regional malignancy has been

resected, whole body imaging excludes extrahepatic metastases, and the

performance status of the patient permits resection. Resection of such

metastases should be undertaken only as one component of a multidisci-

plinary treatment approach with discussions among various cancer and

procedural specialists. Favorable selection factors for operation related

to the tumor include metachronous presentation of the hepatic metas-

tasis, long recurrence interval, number of metastases, extent of hepatic

resection, and documented responsiveness to chemotherapy. Clearly, the

potential survival benet of resection must oset operative risk. e entire

supportive data for hepatic resection in such patients are based on limited

series of retrospective studies in highly selected patients. In general, resec-

tion of liver metastases from primary gastrointestinal tumors (esophageal,

pancreatic, gastric, and biliary) is unlikely to lead to meaningful survival

benet. In contrast, resection of duodenal and small bowel liver metasta-

ses (more similar to colorectal cancer in biology) may have more favor-

able outcomes. Other highly selected patients with nongastrointestinal

liver metastases may potentially benet from an aggressive approach. For

example, although less than 5% of metastatic breast cancer is limited to the

liver, the overall prevalence of the disease in the general population makes

this scenario relatively frequent. Patients with hormone receptor–positive

tumors have the best outcomes after attempts at curative liver resections;

however, most breast cancer liver metastases do not express receptors

(triple negative). us, the overall median survival after hepatectomy for

breast cancer metastases is typically 12 months, and development of extra-

hepatic disease is common after resection. Resection of hepatic renal cell

carcinoma metastases results in a good prognosis similar to outcomes for

colorectal metastases resection. e best outcomes in renal cell metastasis

patients occur in those with a prolonged disease-free interval. Resection of

sarcoma liver metastases remains the only potentially curative option given

the limited eective systemic therapies. Outcomes appear to be improving

for metastatic gastrointestinal stromal (GIST) tumors due to development

of eective targeted chemotherapy agent. Melanoma, specically ocular in

origin, harbors one of the worst prognoses after liver resection due to either

occult or metachronous development of disseminated disease soon after

liver resection.

e standard approach to hepatic resection for malignancy should

be employed when considering atypical indications. Important planning

includes evaluating the margins of resection and adequate functional liver

remnant. e operative approach may be laparoscopic or open depending

on individual surgeons’ experience preference. Patients with noncolorectal,

nonneuroendocrine liver metastases may also be treated with percutane-

ous ablation. Some noncolorectal, nonneuroendocrine metastases such as

renal cell cancers or melanomas are quite vascular, and this feature may

predict responsive to transarterial therapy. Based on the experience of iso-

lated limb perfusion for melanoma and sarcoma, isolated hepatic per fusion

is a potential option at specialized centers for highly selected patients with

liver-only unresectable disease. is regional treatment allows higher doses

of chemotherapy to be delivered directly to the tumor, limiting systemic

toxic ity.

Chapter 16 / Neuroendocrine and Noncolorectal Liver Metastases 203

Benign Liver Tumors

Jason B. Liu and Marshall S. Baker

Chapter 17 / Benign Liver Tumors 205

Treatment

Most simple cysts do not require treatment or follow-up. Giant simple cysts

(≥ 6 cm) that become symptomatic warrant operative management. Cysts

that are noted to be increasing in size raise concern for a cystadenoma or

cystadenocarcinoma and also warrant operative management. For patients

in whom imaging is not denitive or patient history raises suspicions for pos-

sible hydatid disease, Echinococcus should be ruled out with serology prior to

surgical intervention. Methods for treatment of simple cysts include percuta-

neous aspiration with or without a sclerosant (e.g., alcohol, minocycline, or

tetracycline), laparoscopic or open marsupialization (“unroong”), or lapa-

roscopic or open resection. Simple aspiration typically leads to guaranteed

recurrence. e recurrence rate is moderately decreased with the addition of

a sclerosant following aspiration, but minor complications such as nausea,

vomiting, or increased pain can occur. Repeated treatments may also be nec-

essary for large cysts. Laparoscopic marsupialization with fulguration of the

cyst epithelium remains the treatment of choice for giant, symptomatic cysts

with a reported recurrence rate as low as 2% in published series. Recurrence is

associated with incomplete unroong and location within the posterior liver

segments. An omental pedicle ap placed within the cyst bed may decrease

recurrence by adhesion formation. Formal cystectomy or segmentectomy has

also been performed but is associ ated with increased morbidity and mortality.

Polycystic Liver Disease

Presentation

Polycystic liver disease (PLD) occurs in association with autosomal dominant

polycystic kidney disease (ADPKD) or as an isolated form of autosomal domi-

nant polycystic liver disease (ADPLD). e development of polycystic liver

occurs over time in patients with ADPKD, while renal cysts are rarely asso-

ciated with isolated ADPLD. e increased prevalence of polycystic livers in

ADPKD is attributed to the improved life expectancy of these patients. Hepatic

involvement is also associated with female gender and severity of renal disease.

PLD is discovered commonly during the fourth or fth decade of life.

Patients are typically asymptomatic, but symptoms associated with hepatic

enlargement can occur. Abdominal pain in the setting of PLD is usually asso-

ciated with cyst hemorrhage or infection. Infection of hepatic cysts carries a

2% mortality rate and should be treated aggressively with intravenous antibi-

otics and percutaneous drainage. Liver function is preserved, and laboratory

values typically only demonstrate a mild elevation in γ-glutamyl transpepti-

dase (GGT) and alkaline phosphatase when symptoms are present.

Diagnosis

e same modalities used to diagnose simple hepatic cysts are used to

diagnose PLD (Fig. 17.1). Ultrasound remains the preferred diagnostic tool

due to its accuracy and low cost. ree types of PLD have been suggested

based upon CT characterization of the number and size of cysts as well as

the amount of hepatic parenchyma between cysts. Type I patients have a

limited number of large cysts with large areas of normal hepatic paren-

chyma. Type II patients have multiple medium-sized cysts isolated to cer-

tain segments. Type III patients are characterized by near replacement of

the hepatic parenchyma with diuse cysts. Surgical intervention for PLD is

grossly reective of these three categories.

Treatment

erapy for PLD is aimed at minimizing the proportion of hepatic cysts while

maximizing hepatic parenchyma, thereby relieving symptoms associated

Section II / Liver206

with mass eect while avoiding the risk for liver failure. Strategies are iden-

tical to the management of simple hepatic cysts except for the addition of

orthotopic liver transplantation in diuse type III disease. Patients with type

I disease are approached with marsupialization or percutaneous drainage

with sclerotherapy. No signicant dierences in morbidity, mortality, or

recurrence rates have been seen using laparoscopic versus open approaches.

Hepatic resection becomes applicable in patients with type II or type III dis-

ease whereby removal of multiple segments that are grossly aected can be

benecial in volume reduction. Transplantation is reserved for patients who

have severely impaired quality of life, are in intractable pain and for whom

there are no other surgical options for management. Candidates usually have

cachexia, signicant weight loss, malnutrition, recurrent cyst infections,

portal hypertension, and ascites. e decision to pursue liver transplanta-

tion requires weighing the high mortality rates (12% to 30%) associated with

transplant against the potential improvement in quality of life.

Cystadenoma

Presentation

Cystadenoma is the most common primary cystic neoplasm of the liver with

an associated malignant potential. e inci dence is limited to case reports and

small case series. ese cysts occur most commonly in women between 40 and

60 years of age, are discovered incidentally on diagnostic imaging, and are gen-

erally asymptomatic. Patients may develop symptoms of right upper quadrant

pain and anorexia due to mass eect from cyst growth.

Diagnosis

Diagnosis of cystadenoma is based primarily on ultrasonographic nd-

ings. ese include irregular borders, hypoechoic cyst with hyperechoic

FIGURE 17.1 Axial CT imaging for a 54-year-old male presenting with epigastric pain. He

was found to have isolated PLD and underwent laparoscopic left hepatic lobectomy and

marsupialization of the dominant right hepatic cyst. He is currently asymptomatic 3 years

postresection.

Chapter 17 / Benign Liver Tumors 207

septations, solid components, papillary projections, wall enhancement,

and dorsal shadows demonstrating calcied areas. Diculty arises when

dierentiating between cystadenoma, cystadenocarcinoma, complex cysts

(i.e., simple cysts with resolved internal hemorrhage), and hydatid cysts.

Multiphase CT or diusion weighted MRI imaging may reveal vascularity

of the septa perhaps suggesting cystadenocarcinoma. Hydatid cysts dem-

onstrate similar patterns on imaging, and Echinococcus should be ruled out

with serology. CEUS can be helpful to rule in cystadenoma and cystadeno-

carcinoma as this modality may detect vascular ow, which is absent in

complex cysts. Analysis of cyst uid nearly always shows elevated CA 19–9

concentration.

Treatment

Surgical resection with clear margins is the denitive treatment for cyst-

adenoma due to the risk of malignant transformation, which is generally

reported to be 10% to 15%. Some authorities consider enucleation eective

therapy for cystadenoma.

Hydatid (Echinococcus) Cyst: Cystic Echinococcosis and Alveolar

Echinococcosis

Hydatid cysts arise from infection with the tapeworm Echinoccocus gran-

ulosus that live in the small intestines of canines and sheep. Humans are

accidental intermediate hosts due to ingestion of eggs via the fecal–oral

route. Other virulent species include E. multilocularis, E. vogeli, and E. oli-

garthus. Echinococcal disease is endemic in many Mediterranean coun-

tries, Eastern Europe, the Middle East, East Africa, East Asia, Australia,

and South America. In the United States, most cases occur in immigrants

from endemic regions. Infection with E. granulosus causes cystic echi-

nococcosis (CE), while infection with E. multilocularis causes alveolar

echinococcosis (AE).

Cystic Echinococcosis (CE)

Presentation. E. granulosus has an annual incidence of 1 to 200 per 100,000

and is endemic to temperate climates such as the Mediterranean, Central

Asia, Australia, and South America where pastoral communities are promi-

nent. Cyst growth is slow and can remain asymptomatic for years. Cysts

can be found throughout the body with the liver (approximately 80%) most

commonly aected followed by the lung (approximately 20%). Symptoms

occur due to either mass eect (e.g., cholestasis, portal hypertension, Budd-

Chiari syndrome) or rupture. Severe presentations include bacterial super-

infection/hepatic abscess formation, secondary cholangitis due to rupture

into the biliary tree, or peritonitis and anaphylaxis due to intra-abdominal

rupture.

Diagnosis. CE is diagnosed based upon patient history, clinical ndings

(e.g., abdominal pain, fever, chest pain, and dyspnea), ultrasonography,

and positive serology. Eosinophilia is rarely present unless there is already

leakage of antigen into the circulation. Ultrasonography has a sensitivity

of 90% to 95% and is the imaging modality of choice (Fig. 17.2). e World

Health Organization (WHO) classication bases treatment decisions on

ultrasound ndings, which dierentiate cysts into active, transitional, and

inactive states (Table 17.1). MRI or magnetic resonance cholangiopancrea-

tography (MRCP) can be used to evaluate any lesions that may involve the

biliary tree or for preoperative planning. e diagnosis is further corrob-

orated with detection of serum antibodies, which has a sensitivity range

between 85% and 98%.

Section II / Liver208

Treatment. ere have been no randomized trials regarding the treatment

of CE. Treatment decisions are made based upon the standardized WHO

ultrasound-based classication as well as the available medical and surgical

expertise in the endemic region. Surgery, percutaneous treatments, and

antiparasitic drug therapy are the mainstays.

FIGURE 17.2 Computed tomography (A) and ultrasound (B) imaging of an echinococcal

cyst in a 74-year-old male of Greek origin. This cyst demonstrates ultrasound features typi-

cal of a CE4 type cyst. It is heterogeneous with degenerative contents and no daughter

cysts. On CT the wall is thickened and partially calcied. These ndings are characteristic

of inactive cysts. The patient was managed with observation and has been asymptomatic

over 3 years of follow-up.

Chapter 17 / Benign Liver Tumors 209

Classication of World Health Organization

17.1

TABLE

Phase Characteristics Surgery

Percutaneous

Therapy

Drug

Therapy

Suggested Initial

Therapy

CE1 Active Unilocular, simple cyst with visible cyst

wall and hydatid sand, “snowake sign”

No Yes Yes PAIR + albendazole

CE2 Active Multiseptated daughter cysts within

mother cyst; “wheel-like,” “rosette-like,”

“honeycomb-like” structures

Yes Ye s Yes Other percutaneous therapy +

albendazole

CE3a Transitional Detached laminated membrane from cyst

wall, “water-lily” sign

No Yes Yes PAIR + albendazole

CE3b Transitional Solid with daughter vesicles, “complex

mass”

Yes Ye s Yes Other percutaneous therapy +

albendazole

CE4 Inactive Heterogeneous, degenerative contents,

“ball of wool” sign

No No No None

CE5 Inactive Calcied cyst wall, cone-shaped shadows No No No None

Section II / Liver210

Surgery is indicated for the treatment of WHO class CE2-CE3b (active

cysts with multiple daughter vesicles), single liver cysts situated supercially

in the liver, infected cysts when percutaneous therapy is unavailable, cysts

communicating with the biliary tree, and cysts compressing adjacent vital

organs. e primary goal is to remove the entirety of parasitic material while

avoiding spillage and secondary echinococcosis. Total cystectomy can be

performed using open or laparoscopic techniques although the frequency

of spillage has not been compared. Total cystectomy is ideally performed

in a “closed” manner whereby the cyst is removed by means of a partial

hepatectomy without opening the cyst. Cystectomy done “open” whereby

the cyst is injected with protoscolicidal agents (i.e., 20% hypertonic saline),

unroofed, and the pericystic tissue is excised has also been described but is

believed to carry increased risk of dissemination. Involvement of the bili-

ary tree is more commonly found in large cysts greater than 7.5cm. Biliary

communication can be detected intraoperatively by using dye or uoros-

copy, and the communication can be simply suture ligated. Perioperative

benzimidazoles (e.g., albendazole, mebendazole) are recommended to

minimize the risk of perioperative dissemination.

Percutaneous treatment including PAIR (puncture, aspiration, injec-

tion, reaspiration) and modied catheterization techniques is indicated for

inoperable patients, recurrent disease, or cysts that fail to respond to benz-

imidazoles only. Communication with the biliary tree must be ruled out

prior to performing PAIR to avoid the risk of chemically induced sclerosing

cholangitis. Success is associated with many of the types of CE except for

CE2 and CE3b, which tend to relapse after PAIR. ese types are treated

with surgical resection, as discussed above, or with modied catheteriza-

tion techniques that involve large-bore catheters, sclerosing agents, and

curettage.

Cyst types CE4 and CE5 (inactive or heavily calcied cysts with no

daughter cysts) can be simply surveyed without treatment. ese are gener-

ally of limited clinical signicance and unlikely to cause biliary complica-

tions or symptoms.

Alveolar Echinococcosis (AE)

Presentation. Infection with E. multilocularis has an annual incidence of

0.03–1.2 per 100,000 and is isolated to regions of central and eastern Europe,

Russia, China, and northern Japan. It has been noted that the frequency of

cases in Western Europe has increased with the popularity of fox hunting.

Interestingly, AE aects the liver primarily and does not form cysts like CE.

e larvae invade locally or spread hematogenously akin to malignancy.

e disease is chronic with an average incubation period of 5 to 15 years.

Symptoms include cholestatic jaundice, abdominal pain, fatigue, weight

loss, and hepatomegaly.

Diagnosis. Diagnosis of AE is based on patient history, clinical presen-

tation, ultrasonography, and positive serology. Cross-sectional imaging

including CT and MRI/MRCP are used to further evaluate the local extent

of the disease particularly during preoperative planning. Classication of

AE is based upon the WHO PNM staging system analogous to the TNM

staging of malignancy.

Treatment. After diagnosis is conrmed and the extent of disease delin-

eated, the PNM staging system is used to direct therapy. Treatment involves

surgical resection, orthotopic liver transplantation, antiparasitic drug

therapy, or endoscopic or percutaneous interventions. e primary goal of

treatment is complete surgical excision whenever possible. Endoscopic or

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