Pathology of the liver and portal venous system

4 Pathology of the liver and portal venous system



Chapter Contents











Benign focal liver lesions




Complex cysts


Some cysts may contain a thin septum, which is not a significant finding. Occasionally haemorrhage or infection may occur in a simple cyst, giving rise to low level, fine echoes within it (Fig. 4.2). Such cysts are usually treated conservatively although the larger ones may be monitored with ultrasound, particularly if symptomatic. Percutaneous aspiration of larger cysts under ultrasound guidance may afford temporary decompression but is rarely performed, as they invariably recur, and there is a risk of infection. Laparoscopic fenestration (deroofing) in which part of the cyst wall is removed allowing drainage, provides a more permanent solution to large, symptomatic cysts.1



A rare cause of a cystic lesion in the liver is the cystadenoma – a benign epithelial tumour which may appear uni- or multilocular. These have a pre-malignant potential, rarely progressing to form a cystadenocarcinoma. Close monitoring with ultrasound will demonstrate gradual increase in size, changes in the appearances of the wall of the cyst, such as thickening or papillary projections, and internal echoes in some cases, which may indicate malignant change. Cystic malignant liver lesions are uncommon, and the majority represent necrotic metastases, but it is extremely important to recognize suspicious malignant features such as solid nodules or thickened walls and septations (Fig. 4.3). A diagnostic aspiration may be performed under ultrasound guidance, and the fluid may contain elevated levels of carcinoembryonic antigen (CEA) if malignant.2 Cystadenomas are usually surgically removed due to their malignant potential (Fig. 4.4).




Rarely, cystic lesions in the liver may be due to other causes. These include pancreatic pseudocyst (within an interlobular fissure) in patients with acute pancreatitis or mucin-filled metastatic deposits in primary ovarian cancer. An AV malformation – a rare finding in the liver – may look like a septated cystic lesion. Doppler, however, will demonstrate flow throughout the structure.




Hydatid (echinococcal) cyst


Hydatid disease comes from a parasite, Echinococcus granulosus, which is endemic in the Middle East and in sheep-farming areas but rare in the UK. The worm lives in the alimentary tract of infected dogs, which excrete the eggs. These may then be ingested by cattle or sheep and subsequently complete their lifecycle in a human. The parasite spreads via the bloodstream to the liver, where it lodges, causing an inflammatory reaction. The resulting cyst can be slow growing and asymptomatic and they may be single or multiple, depending on the degree of infestation.


Ultrasound may demonstrate a spectrum of appearances, from cystic through to solid, and the diagnosis can be made by looking carefully at the wall and contents; the hydatid cyst has two layers to its capsule, which may appear thickened, separated or detached on ultrasound. Daughter cysts may arise from the inner capsule – the honeycomb or cartwheel appearance (Fig. 4.6) and the cyst may contain floating membranes and fine sand or debris.3 A calcified rind around a cyst is usually associated with an old, inactive hydatid lesion.



The diagnosis of hydatid, as opposed to a simple cyst, is an important one, as any attempted aspiration may spread the parasite further by seeding along the needle track if the operator is unaware of the diagnosis. Hydatids may be treated successfully using percutaneous ultrasound guided aspiration with sclerotherapy although surgical resection is necessary for some cases.4



Abscesses


Liver abscesses result from bacterial, fungal or parasitic infection. The most common are pyogenic abscesses secondary to abdominal infection – for example cholangitis (via the biliary tree), diverticulitis or appendicitis (via the portal vein). Diabetic patients and those with compromised immune response are particularly prone to such infections.




Ultrasound appearances


Hepatic abscesses may display a spectrum of acoustic features. Their internal appearances vary considerably; in the very early stages there is a zone of infected, oedematous liver tissue which appears on ultrasound as a hypoechoic, solid focal lesion. As the infection develops, the liver tissue becomes necrotic and liquefaction takes place. The abscess may still appear full of homogeneous echoes from pus and can be mistaken for a solid lesion, but as it progresses, the fluid content may become apparent, usually with considerable debris within it. Because they are fluid-filled, abscesses demonstrate posterior enhancement (Fig. 4.7A). The margins of an abscess are irregular, often ill-defined and frequently thickened. The inflammatory capsule of the abscess may demonstrate vascularity on colour or power Doppler, but this is not invariable, and depends on equipment sensitivity and size of the lesion. Infection with gas-forming organisms may account for the presence of gas within some liver abscesses (Fig. 4.7B).



There are three main types of abscess.







Haematoma


A haematoma is the result of trauma (usually, therefore, via the emergency department) but the trauma may also be iatrogenic, e.g. following a biopsy procedure (hence the value of using ultrasound guidance to avoid major vessels in the liver) or surgery. The liver haematoma may have similar acoustic appearances to those of an abscess but does not share the same clinical features or history (Fig. 4.8).



The acoustic appearances depend upon the timing – a fresh haematoma may appear liquid and hypoechoic, but rapidly becomes more ‘solid’ looking and hyperechoic, as the blood clots. As it resolves the haematoma liquefies and may contain fibrin strands. It will invariably demonstrate a band of posterior enhancement and has irregular, ill-defined walls in the early stages. Later on it may encapsulate, leaving a permanent cystic ‘space’ in the liver, and the capsule may calcify. Injury to the more peripheral regions may cause a subcapsular haematoma which demonstrates the same acoustic properties. The haematoma outlines the surface of the liver and the capsule can be seen surrounding it. This may be the cause of a palpable ‘enlarged’ liver (Fig. 4.8B).


Intervention is rarely necessary and monitoring with ultrasound confirms eventual resolution. More serious hepatic ruptures, however, causing haemoperitoneum, may require surgery. CEUS is useful in demonstrating the extent of injury and is particularly useful in the absence of a haemoperitoneum (see Chapter 10, Fig. 10.1) (Table 4.1).


Table 4.1 Cystic focal liver lesions – differential diagnoses









































SIMPLE CYST  
Anechoic, thin capsule, posterior enhancement (may contain thin septae) Common finding, usually insignificant
  Consider polycystic disease if multiple (rarely an AV malformation may mimic a septated cyst – exclude by using colour Doppler)
COMPLEX CYST  
Thin capsule + internal echoes Haemorrhage or infection in a cyst
Mucinous metastasis
Cystadenoma
Capsule thickened or complex, may also contain echoes Hydatid cyst
Cystadenocarcinoma
Intrahepatic pancreatic pseudocyst (rare)
SOLID/CYSTIC LESION  
Irregular margin, internal echoes + debris/solid material Abscess
Haematoma
Necrotic metastasis
Cavernous haemangioma


Haemangioma


This is the most common, solid benign tumour found in the liver. Haemangiomas are highly vascular, composed of a network of tiny blood vessels. They may be solitary or multiple. Most haemangiomas are small and found incidentally. They are rarely symptomatic but do cause a diagnostic dilemma, as they can be indistinguishable from liver metastases.


Their acoustic appearances vary; the majority are hyperechoic, rounded well-defined lesions, but they may also be hypoechoic or of mixed echogenicity. In patients with fatty livers, the haemangioma frequently looks hypoechoic relative to the background of the hyperechoic hepatic parenchyma. Larger ones can demonstrate a spectrum of reflectivity depending on their composition, and may demonstrate pools of blood and central areas of degeneration. They frequently exhibit slightly increased through-transmission, with posterior enhancement, particularly if large. This is probably due to the increased blood content compared with the surrounding liver parenchyma (Fig. 4.9).



As with many lesions, colour or power Doppler is too insensitive to pick up the slow flow in haemangiomas or to assist with lesion characterization. Microbubble contrast agents demonstrate a peripheral, globular enhancement with gradual centripetal filling to become isoechoic with the background liver in the sinusoidal phase.7 CEUS frequently provides a definitive diagnosis at the time of scanning, reassuring the patient and obviating the need for further imaging of follow-up (Fig. 4.9).


When found in children, haemangiomas tend to be large and do produce symptoms. These masses produce shunting of blood from the aorta via the main hepatic artery and, in extreme cases, present with resulting cardiac failure. They are often heterogeneous in appearance and larger vessels within them may be identified with Doppler. Although many regress over a period of time, others may have to be embolized with coils under radiological guidance to control the symptoms.



Focal nodular hyperplasia


Focal nodular hyperplasia (FNH) is the second most common solid, benign liver tumour. It is made up of a hyperplastic proliferation of liver cells with hepatocytes, Kupffer cells, biliary and fibrous elements. It is most commonly found in young women, and is usually discovered by chance, being asymptomatic. Its ultrasound characteristics vary, from hypo-, iso- to hyperechoic compared with background liver (Fig. 4.10) and it may be multifocal. As with the haemangioma, it presents a diagnostic dilemma when found on CT or ultrasound, as its characteristics vary. As with haemangioma, CEUS is extremely helpful in characterizing incidental FNH, as it usually displays rapid arterial stellate filling, followed by centripetal enhancement with contrast uptake isoechoic with background liver in the sinusoidal phase.8 It may, however, be difficult to differentiate from the rarer adenoma which also exhibits rapid arterialization on CEUS.9



Management of this benign mass is usually conservative, but surgical resection may be necessary in larger lesions.



Adenoma


The hepatic adenoma is a benign focal lesion consisting of a cluster of atypical liver cells (Fig. 4.11). Within this, there may be pools of bile or focal areas of haemorrhage or necrosis. This gives rise to a heterogeneous, patchy echotexture. The smaller ones tend to be homogeneous with a smooth texture. Their lipid content causes a tendency to be hyperechoic, although usually less reflective than a haemangioma, and many have similar reflectivity to the surrounding liver parenchyma.





Focal fatty change


Not a ‘lesion’ as such, but included here due to its focal appearance. It may pose a diagnostic dilemma on initial ultrasound.



Focal fatty infiltration


Fatty infiltration of the liver is a common occurrence which may affect the whole, or part of the liver. It is associated with obesity and alcoholism, and can also occur in pregnancy, diabetes and with certain drugs.


The deposition of fat confined to certain focal areas of the liver is related to the blood supply to that area. Fatty infiltration increases the reflectivity of the parenchyma, making it hyperechoic. This can simulate a focal mass, such as a metastasis. Unlike a focal lesion however, it does not display any mass effect and the course of related vessels remains constant. It tends to have a characteristic straight-edged shape, rectangular or ovoid, corresponding to the region of local blood supply (Fig. 4.12). Foci of fatty change may be multiple, or may affect isolated liver segments. The most common sites are in segment IV around the porta, in the caudate lobe (segment I) and in the posterior area of the left lobe (segment III).



CEUS is useful and accurate in differentiating focal fatty change from a true lesion, as the contrast uptake is identical to background liver, and the contrast convincingly demonstrates the lack of mass effect (Fig. 4.12D) This technique usually obviates the need for further imaging in focal fatty change.







Malignant focal liver lesions



Metastases


The liver is one of the most common sites to which malignant tumours metastasize. Secondary deposits are usually bloodborne, spreading to the liver via the portal venous system (e.g. in the case of gastrointestinal malignancies) or hepatic artery (e.g. lung or breast primaries,) or spread via the lymphatic system. Some spread along the peritoneal surfaces – for example ovarian carcinoma. This demonstrates an initial invasion of the subserosal surfaces of the liver (see Fig. 4.16A below), as opposed to the more central distribution seen with a haematogenous spread (see Fig. 4.16B below). The former, peripheral pattern is more easily missed on ultrasound because small deposits are often obscured by near-field artefact or rib shadows. It is therefore advisable for the operator to be aware of the possible pattern of spread when searching for liver metastases.



Ultrasound appearances


The acoustic appearances of liver secondaries are extremely variable (Figs 4.15, 4.16). When compared with normal surrounding liver parenchyma, metastases may be hyperechoic, hypoechoic, isoechoic or of mixed pattern. It is not possible to characterize the primary source by the acoustic properties of the metastases.




Metastases tend to be solid with ill-defined margins. Some metastases, particularly the larger ones, contain fluid as a result of central necrosis (Fig. 4.16E), or because they contain mucin, for example from some ovarian primaries. Occasionally, calcification is seen within a deposit, causing distal acoustic shadowing, and this may also develop following treatment with chemotherapy. In some diseases, for example lymphoma, the metastases may be multiple but tiny – not immediately obvious to the operator as discrete focal lesions but as a coarse-textured liver (Fig. 4.16F). This type of appearance is non-specific and could be associated with a number of conditions, both benign and malignant.


Diagnosis of focal liver lesions, such as metastases, is made more difficult when the liver texture is diffusely abnormal or when there are dilated intrahepatic ducts, because the altered transmission of sound through the liver masks small lesions. Other possible ultrasound features associated with metastases include a lobulated outline to the liver, hepatomegaly and ascites.


If the finding of liver metastases is unexpected, or the primary has not been identified, it is useful to complete a full examination to search for a possible primary carcinoma and to identify other sites of carcinomatous spread. Lymphadenopathy (particularly in the para-aortic, para-caval and portal regions) may be demonstrated on ultrasound, as well as invasion of adjacent blood vessels and disease in other extrahepatic sites including spleen, kidneys, omentum and peritoneum. CT is the usual next step, to identify a possible primary site and to stage the disease, in particular demonstrating extra-hepatic disease which is often not seen on ultrasound.


Doppler is unhelpful in characterizing liver metastases, most of which appear poorly vascular or avascular. Fundamental non-contrast ultrasound lacks sensitivity in the diagnosis of liver metastases, as many lesions are either isoechoic, or small (subcentimetre) rendering them almost invisible. The use of microbubble contrast agents radically improves both the characterization and detection of metastatic deposits on ultrasound.12 The injection of a bolus of contrast agent when viewed using pulse-inversion demonstrates variable vascular phase enhancement in the arterial and portal phases, but the sinusoidal phase invariably lacks contrast uptake (Fig. 4.16G, H). This is a particularly useful technique as it increases the contrast resolution between metastasis and background liver, meaning that even subcentimetre lesions are reliably demonstrated.


CEUS also increases the operator’s confidence in the absence of metastases, particularly in cases with altered LFTs and prior history of malignancy (Fig. 4.16J). This is useful in obviating the need for further imaging in normal livers.



Clinical features and management


Many patients present with symptoms from their liver deposits, rather than the primary carcinoma. The demonstration of liver metastases on ultrasound may often prompt further radiological investigations for the primary. The symptoms of liver deposits may include non-obstructive jaundice, obstructive jaundice (which may occur if a large mass is present at the porta), hepatomegaly, right-sided pain, increasing abdominal girth from ascites and altered LFTs.


Ultrasound-guided biopsy may be useful in diagnosing the primary and complements further imaging such as X-rays and contrast bowel studies. Accurate staging of the disease is performed with CT, MRI and/or PET-CT,13 which have improved sensitivity for identifying extrahepatic and systemic disease, such as peritoneal deposits and lymphadenopathy, and which can more accurately demonstrate adjacent spread of primary disease.


The prognosis for most patients with liver metastases has traditionally been poor, particularly if multiple, and depends to a large extent on the origin of the primary carcinoma. A regime of surgical debulking (removal of the primary carcinoma, adjacent invaded viscera, lymphadenopathy etc.) together with chemotherapy can slow down the progress of the disease. Increasingly, however, there are treatment options which result in increased, good quality survival and, in some cases, cure. This makes it important that secondary disease is diagnosed early and accurately in order to offer patients a significant chance of survival.

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Dec 26, 2015 | Posted by in GASTROINTESTINAL IMAGING | Comments Off on Pathology of the liver and portal venous system

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