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Cystic Tumor
Dr. Bikash Ch. Sah
Surgery JR
Introduction…
Cystic lesions of the liver represent a
heterogeneous group of disorders, which
differ in etiology, prevalence, and clinical
manifestations.
 Most liver cysts are found incidentally on
imaging studies and tend to have a benign
course.
 A minority can cause symptoms and rarely
may be associated with serious morbidity and
mortality.
• Larger cysts are more likely to be symptomatic
and cause complications such as
– Spontaneous hemorrhage,
– Rupture into the peritoneal cavity or bile duct,
– Infection
– Compression of the biliary tree.
• Specific types of cysts may have unique
complications such as
– Malignant transformation in the case of a mucinous
cystic neoplasm (cystadenoma), or
– Anaphylactic shock due to a hydatid cyst
Copyrights apply
• Simple liver cyst:
Simple cysts of the liver are cystic formations
containing clear fluid
Do not communicate with the intrahepatic biliary tree.
They are generally spherical or ovoid.
Their size ranges from a few millimeters to massive
lesions occupying large volumes of the upper abdomen;
the largest reported cyst contained 17 liters of fluid.
Tend to occur more commonly in the right lobe.
In 50% of cases, the cysts are singular.
 More prevalent in women.
 The female-to-male ratio is approximately 1.5:1
among those with asymptomatic simple cysts,
while it is 9:1 in those with symptomatic or
complicated simple cysts .
 Huge cysts are found almost exclusively in
women over 50 years.
 Large cysts can compress normal liver,
inducing regional atrophy and sometimes
compensatory contralateral hypertrophy
Clinical feature:
Symptomatic patients may present with
– abdominal discomfort, pain, or nausea.
– Large cysts can produce
• Atrophy of the adjacent hepatic tissue,
• While huge cysts can cause complete atrophy of a hepatic
lobe with compensatory hypertrophy of the other lobe.
– Complications such as
• Spontaneous hemorrhage,
• Bacterial infection,
• Torsion of pedunculated cyst,
• Rupture, or
• Biliary obstruction.
Imaging studies
Ultrasonography
– probably the most helpful initial test, since it can
usually differentiate a simple cyst from other
cystic lesions.
– It should also be used for follow-up studies.
– Simple cysts appear as an anechoic unilocular
fluid-filled space with imperceptible walls and
with posterior acoustic enhancement.
Computed tomography scan,
– A simple cyst is defined as a well-demarcated water-
attenuation lesion that does not enhance following
the administration of intravenous contrast.
– Uncomplicated simple cysts are virtually never
septated.
Magnetic resonance imaging demonstrates
– A well-defined water-attenuation lesion that does not
enhance following the administration of intravenous
Gadolinium.
– On T1-weighted images the cyst shows a low signal,
whereas a very high intensity signal is shown on T2-
weighted images.
Histology and needle aspiration
Histological examination:
– Seldom needed for establishing the diagnosis.
– The following criteria can be used for a definitive
diagnosis:
• An outer layer of a thin, dense fibrous tissue
• An inner epithelial lining consisting of a single layer of
cuboidal or columnar epithelium; this layer is found in
most, but not in all, simple cysts
• Lack of mesenchymal stroma or cellular atypia
• Needle Aspiration:
– Usually not required for diagnosing cysts that
have a typical sonographic appearance.
– When it is performed,
• The aspirated fluid is always sterile and cytologically
negative.
• It may vary from a clear straw color to brown.
• High levels of carcino embryonic antigen in the cyst
fluid have been reported in mucinous cystic neoplasms
with associated invasive carcinoma
Management
Asymptomatic patients —
– The majority of asymptomatic simple cysts do not
require treatment.
– Simple cysts that are found incidentally do not
require serial imaging
– They have no malignant potential as they tend to
remain stable
Symptomatic patients
The causal relationship between abdominal pain
or discomfort and a simple cyst must be admitted
with caution and accepted only if the cyst is large
and other possible causes of the symptoms have
been excluded.
These include cholelithiasis, gastroesophageal
reflux disease, gastric dysmotility, peptic ulcer
disease, and other causes of dyspepsia.
Percutaneous aspiration has been advocated as
a diagnostic test for relief of symptoms
Therapeutic approaches
• Several therapeutic approaches have been
described for symptomatic, large simple cysts,
including
– Needle aspiration with or without injection of
sclerosing agents( alcohol, minocycline)
• With a high failure rate and rapid recurrence .
• Percutaneous needle aspiration with injection of sclerosing
agents is safe, effective, and relatively noninvasive, however
complication tends to recur.
– Internal drainage with cystojejunostomy,
– Wide unroofing or cystic resection.
– Varying degrees of liver resection
Wide unroofing
• Advantage
– low incidence of cyst recurrence or complications.
– laparoscopic approach was associated with
• Shorter operative time,
• Shorter hospital stay,
• Less operative blood loss
• No need for a debilitating incision
• No difference in cyst recurrence rate
• Laparoscopic unroofing may not be possible in
patients with a superior or posterior location of
the cyst
Potential complications of laparoscopic
unroofing include
– Wound infection,
– Chest infection,
– Bile leak,
– Subphrenic hematoma, and
– Prolonged postoperative drainage through
abdominal drain (more than three days).
Mucinous Cystic Tumor
• Cystadenoma
• Cystadenocarcinoma
Cystadenoma
Cystadenoma of the liver is
– A rare neoplasm.
– Generally is manifested as a large cystic mass,
usually 10 to 20 cm.
– Mainly affects woman older than 40 years.
– Tend to grow slowly but can eventually progress
to their malignant counterpart,
cystadenocarcinomas.
• Macroscopic
– The cyst has a globular external surface with
multiple protruding cysts and locules of various
sizes.
– The fluid contained in these cysts is usually
mucinous.
• On microscopic examination
– Atypical cuboidal or columnar cells resting on a
basement membrane, with ovarian-like stroma, line
the cysts.
– The epithelium often forms polypoid or papillary
projections.
Clinical feature
• Mostly asymptomatic,
• symptoms can include
– Abdominal pain,
– Anorexia, nausea
– Abdominal distention
Ultrasound usually demonstrates
A cystic structure with varying wall thickness,
nodularity, septations, and fluid-filled locules.
Contrast-enhanced CT demonstrates
enhancement of the cyst wall and septa.
Complications
Complications of hepatic cystadenomas include the
following:
Bleeding
Rupture
Obstructive jaundice
Malignant transformation
Infection
Gastric outlet obstruction
Ascites
Inferior vena cava obstruction
• The preferred treatment for noninvasive
MCNs is resection,
– which should be performed whenever possible,
since malignant transformation of the cyst lining
has been described in as many as 15 percent of
patients.
• A hepatic resection should be considered
whenever a cystic lesion is suspected of
containing invasive carcinoma.
• Removal of the cyst can be accomplished by
enucleating it from the surrounding liver.
• Partial excision is invariably associated
– With recurrence and
– With worse prognosis compared with complete
resection.
• Aspiration is also associated with rapid
recurrence of fluid and symptoms
Cystadenocarcinoma
• AKA: Mucinous cystic neoplasms (MCNs) with
associated invasive carcinoma:
– Are usually found in older adults, although they have
been reported in patients in their thirties .
– While the tumors can invade adjacent tissues and
metastasize,
– Their prognosis has generally been better than that
associated with cholangiocarcinoma.
• MCNs with associated invasive carcinoma
– Have a thick wall that may show large tissue
masses protruding from the internal cyst lining.
• Occasionally been identified preoperatively by
aspiration and examination of the contents of
the cyst,
– This procedure carries a risk of bleeding and
– Peritoneal seeding of the tumor.
• Elevated levels of carcinoembryonic antigen
have been reported.
• Histologic examination:
– Complex glandular structures embedded in the
stroma of the wall
– Glands demonstrates with a cribriform growth
pattern and nuclei displaying mild atypia.
Imaging:
Typically seen as a single multilocular cystic
tumor with septal or mural nodules .
• Imaging cannot reliably differentiate
cystadenoma from cystadenocarcinoma, but
the presence of septal nodularity may favor
the diagnosis of biliary cystadenocarcinoma
versus a biliary cystadenoma .
• Contrast-enhanced ultrasound demonstrates
minimal enhancement since the tumors are
largely avascular. Abnormally increased
vascularity may be present peripherally or in
the septa.
• Discrete soft tissue masses, thick and coarse
calcifications, and heterogeneous CT
attenuation or MR intensity within the loculi
may also be seen, although these features are
nonspecific.
Management
• If invasive carcinoma is suspected, treatment
should consist of a formal liver resection.
• Enucleation is not recommended, since it may
be associated with an increased risk of
recurrence.
• The lesion is potentially curable by complete
excision.
• The effect of nonsurgical therapy (eg, radiation
or chemotherapy) is unknown.
Polycystic Liver Disease Liver cysts
 Are commonly seen in patients with the autosomal
dominant inherited adult polycystic kidney disease.
 Cysts, which appear to be derived from biliary epithelium,
 The cysts are histologically similar to simple cysts (see
earlier).
 The main difference between the two entities is the number
of cysts.
 When liver cysts are present in patients with adult
polycystic kidney disease, they are always multiple in
number.
 Also, there are usually numerous microscopic hepatic cysts
as well as the grossly visible macrocysts.
• Despite the large number of liver cysts, hepatic parenchyma
and function are usually preserved
• Liver cysts are always preceded by kidney cysts.
• Their prevalence in adult polycystic kidney disease
increases with age.
• In those younger than 20 years, the prevalence of liver
cysts is 0%, whereas in those older than 60 years, it is 80%
• Two mutations have been found to cause this disorder:
– A mutation in the PRKCSH gene that encodes a protein called
hepatocystin and
– A mutation in the SEC63 gene that encodes for a component of
the protein translocation machinery in the endoplasmic
reticulum
Clinical feature
Liver cysts in patients with adult polycystic
kidney disease are
– Generally asymptomatic,
– Numerous large cysts may cause abdominal pain
and distention.
LFT results are almost always normal.
Rare complications can occur; these include
infection and intracystic bleeding
• Ultrasound and CT reveal multiple simple
cysts throughout the liver and kidneys.
Managment
• Treatment of polycystic liver disease is reserved
for
– Severe symptoms related to large cysts and
complications.
• Treatment includes:
– Percutaneous aspiration with or without sclerotherapy,
– Cyst fenestration (by laparotomy or laparoscopy),
– Hepatic resection,
– Orthotopic liver transplantation
• Liver transplantation is used
– only with progressive disease
• After fenestration
• or Resection with liver
• or Renal dysfunction.
• In the context of renal failure, a combined
kidney and liver transplantation may be
appropriate
Bile Duct Cysts
• Bile duct cysts or choledochal cysts
• Are congenital dilations of the biliary tree that are usually
diagnosed in childhood but can present in adulthood.
• Because of the risk of malignancy and recurrent cholangitis,
treatment is excision with reestablishment of
biliaryenteric continuity.
• Most bile duct cysts involve the extrahepatic biliary tree,
• but in type IV cysts, there is involvement of the
extrahepatic bile duct and intrahepatic ducts.
• In contrast, Caroli disease (type V) is characterized by
multiple intrahepatic cysts.
• Thus, bile duct cysts must be considered in the differential
diagnosis of a patient with multiple hepatic cystic lesions.
The intrahepatic lesions of type IV bile duct cysts and Caroli
disease are multifocal dilations of the segmental bile ducts
separated by portions of normal-caliber bile ducts.
• Approximately 50% of cases of Caroli disease are associated
with congenital hepatic fibrosis; the cysts are diffusely
located throughout the liver. In the other 50% of cases, the
dilations may be confined to a portion of the liver, usually
the left hemiliver. Recurrent bacterial cholangitis usually
dominates the clinical course of these diseases, and death
generally ensues within 5 to 10 years without adequate
treatment. When intrahepatic bile duct cysts are localized,
hepatic resection, with or without biliary reconstruction, is
the treatment of choice. Treatment of diffuse hepatic
involvement is poor; in complicated cases, the only
probably effective treatment is transplantation
• Extrahepatic cysts presenting as hepatic cystic
lesions —
– In rare cases, extrahepatic cysts, which are
adjacent to the liver (eg, adrenal, renal or
mesenteric cystic lesions) may closely mimic
hepatic cysts
– May be difficult to differentiate from the latter
without a surgical intervention
Tomorrow, is the first blank page of a 365 page
book. Write a good one."
- Brad Paisley
#Live your Fear # Live your #life
Thank you…

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Cystic neoplasm of liver

  • 1. Cystic Tumor Dr. Bikash Ch. Sah Surgery JR
  • 2. Introduction… Cystic lesions of the liver represent a heterogeneous group of disorders, which differ in etiology, prevalence, and clinical manifestations.  Most liver cysts are found incidentally on imaging studies and tend to have a benign course.  A minority can cause symptoms and rarely may be associated with serious morbidity and mortality.
  • 3. • Larger cysts are more likely to be symptomatic and cause complications such as – Spontaneous hemorrhage, – Rupture into the peritoneal cavity or bile duct, – Infection – Compression of the biliary tree. • Specific types of cysts may have unique complications such as – Malignant transformation in the case of a mucinous cystic neoplasm (cystadenoma), or – Anaphylactic shock due to a hydatid cyst
  • 6. Simple cysts of the liver are cystic formations containing clear fluid Do not communicate with the intrahepatic biliary tree. They are generally spherical or ovoid. Their size ranges from a few millimeters to massive lesions occupying large volumes of the upper abdomen; the largest reported cyst contained 17 liters of fluid. Tend to occur more commonly in the right lobe. In 50% of cases, the cysts are singular.
  • 7.  More prevalent in women.  The female-to-male ratio is approximately 1.5:1 among those with asymptomatic simple cysts, while it is 9:1 in those with symptomatic or complicated simple cysts .  Huge cysts are found almost exclusively in women over 50 years.  Large cysts can compress normal liver, inducing regional atrophy and sometimes compensatory contralateral hypertrophy
  • 8. Clinical feature: Symptomatic patients may present with – abdominal discomfort, pain, or nausea. – Large cysts can produce • Atrophy of the adjacent hepatic tissue, • While huge cysts can cause complete atrophy of a hepatic lobe with compensatory hypertrophy of the other lobe. – Complications such as • Spontaneous hemorrhage, • Bacterial infection, • Torsion of pedunculated cyst, • Rupture, or • Biliary obstruction.
  • 9. Imaging studies Ultrasonography – probably the most helpful initial test, since it can usually differentiate a simple cyst from other cystic lesions. – It should also be used for follow-up studies. – Simple cysts appear as an anechoic unilocular fluid-filled space with imperceptible walls and with posterior acoustic enhancement.
  • 10. Computed tomography scan, – A simple cyst is defined as a well-demarcated water- attenuation lesion that does not enhance following the administration of intravenous contrast. – Uncomplicated simple cysts are virtually never septated. Magnetic resonance imaging demonstrates – A well-defined water-attenuation lesion that does not enhance following the administration of intravenous Gadolinium. – On T1-weighted images the cyst shows a low signal, whereas a very high intensity signal is shown on T2- weighted images.
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  • 13. Histology and needle aspiration Histological examination: – Seldom needed for establishing the diagnosis. – The following criteria can be used for a definitive diagnosis: • An outer layer of a thin, dense fibrous tissue • An inner epithelial lining consisting of a single layer of cuboidal or columnar epithelium; this layer is found in most, but not in all, simple cysts • Lack of mesenchymal stroma or cellular atypia
  • 14. • Needle Aspiration: – Usually not required for diagnosing cysts that have a typical sonographic appearance. – When it is performed, • The aspirated fluid is always sterile and cytologically negative. • It may vary from a clear straw color to brown. • High levels of carcino embryonic antigen in the cyst fluid have been reported in mucinous cystic neoplasms with associated invasive carcinoma
  • 15. Management Asymptomatic patients — – The majority of asymptomatic simple cysts do not require treatment. – Simple cysts that are found incidentally do not require serial imaging – They have no malignant potential as they tend to remain stable
  • 16. Symptomatic patients The causal relationship between abdominal pain or discomfort and a simple cyst must be admitted with caution and accepted only if the cyst is large and other possible causes of the symptoms have been excluded. These include cholelithiasis, gastroesophageal reflux disease, gastric dysmotility, peptic ulcer disease, and other causes of dyspepsia. Percutaneous aspiration has been advocated as a diagnostic test for relief of symptoms
  • 17. Therapeutic approaches • Several therapeutic approaches have been described for symptomatic, large simple cysts, including – Needle aspiration with or without injection of sclerosing agents( alcohol, minocycline) • With a high failure rate and rapid recurrence . • Percutaneous needle aspiration with injection of sclerosing agents is safe, effective, and relatively noninvasive, however complication tends to recur. – Internal drainage with cystojejunostomy, – Wide unroofing or cystic resection. – Varying degrees of liver resection
  • 18. Wide unroofing • Advantage – low incidence of cyst recurrence or complications. – laparoscopic approach was associated with • Shorter operative time, • Shorter hospital stay, • Less operative blood loss • No need for a debilitating incision • No difference in cyst recurrence rate • Laparoscopic unroofing may not be possible in patients with a superior or posterior location of the cyst
  • 19. Potential complications of laparoscopic unroofing include – Wound infection, – Chest infection, – Bile leak, – Subphrenic hematoma, and – Prolonged postoperative drainage through abdominal drain (more than three days).
  • 20. Mucinous Cystic Tumor • Cystadenoma • Cystadenocarcinoma
  • 21. Cystadenoma Cystadenoma of the liver is – A rare neoplasm. – Generally is manifested as a large cystic mass, usually 10 to 20 cm. – Mainly affects woman older than 40 years. – Tend to grow slowly but can eventually progress to their malignant counterpart, cystadenocarcinomas.
  • 22. • Macroscopic – The cyst has a globular external surface with multiple protruding cysts and locules of various sizes. – The fluid contained in these cysts is usually mucinous.
  • 23. • On microscopic examination – Atypical cuboidal or columnar cells resting on a basement membrane, with ovarian-like stroma, line the cysts. – The epithelium often forms polypoid or papillary projections.
  • 24. Clinical feature • Mostly asymptomatic, • symptoms can include – Abdominal pain, – Anorexia, nausea – Abdominal distention
  • 25. Ultrasound usually demonstrates A cystic structure with varying wall thickness, nodularity, septations, and fluid-filled locules. Contrast-enhanced CT demonstrates enhancement of the cyst wall and septa.
  • 26. Complications Complications of hepatic cystadenomas include the following: Bleeding Rupture Obstructive jaundice Malignant transformation Infection Gastric outlet obstruction Ascites Inferior vena cava obstruction
  • 27. • The preferred treatment for noninvasive MCNs is resection, – which should be performed whenever possible, since malignant transformation of the cyst lining has been described in as many as 15 percent of patients. • A hepatic resection should be considered whenever a cystic lesion is suspected of containing invasive carcinoma.
  • 28. • Removal of the cyst can be accomplished by enucleating it from the surrounding liver. • Partial excision is invariably associated – With recurrence and – With worse prognosis compared with complete resection. • Aspiration is also associated with rapid recurrence of fluid and symptoms
  • 29. Cystadenocarcinoma • AKA: Mucinous cystic neoplasms (MCNs) with associated invasive carcinoma: – Are usually found in older adults, although they have been reported in patients in their thirties . – While the tumors can invade adjacent tissues and metastasize, – Their prognosis has generally been better than that associated with cholangiocarcinoma.
  • 30. • MCNs with associated invasive carcinoma – Have a thick wall that may show large tissue masses protruding from the internal cyst lining.
  • 31. • Occasionally been identified preoperatively by aspiration and examination of the contents of the cyst, – This procedure carries a risk of bleeding and – Peritoneal seeding of the tumor. • Elevated levels of carcinoembryonic antigen have been reported.
  • 32. • Histologic examination: – Complex glandular structures embedded in the stroma of the wall – Glands demonstrates with a cribriform growth pattern and nuclei displaying mild atypia.
  • 33. Imaging: Typically seen as a single multilocular cystic tumor with septal or mural nodules . • Imaging cannot reliably differentiate cystadenoma from cystadenocarcinoma, but the presence of septal nodularity may favor the diagnosis of biliary cystadenocarcinoma versus a biliary cystadenoma .
  • 34. • Contrast-enhanced ultrasound demonstrates minimal enhancement since the tumors are largely avascular. Abnormally increased vascularity may be present peripherally or in the septa. • Discrete soft tissue masses, thick and coarse calcifications, and heterogeneous CT attenuation or MR intensity within the loculi may also be seen, although these features are nonspecific.
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  • 36. Management • If invasive carcinoma is suspected, treatment should consist of a formal liver resection. • Enucleation is not recommended, since it may be associated with an increased risk of recurrence. • The lesion is potentially curable by complete excision. • The effect of nonsurgical therapy (eg, radiation or chemotherapy) is unknown.
  • 37. Polycystic Liver Disease Liver cysts  Are commonly seen in patients with the autosomal dominant inherited adult polycystic kidney disease.  Cysts, which appear to be derived from biliary epithelium,  The cysts are histologically similar to simple cysts (see earlier).  The main difference between the two entities is the number of cysts.  When liver cysts are present in patients with adult polycystic kidney disease, they are always multiple in number.  Also, there are usually numerous microscopic hepatic cysts as well as the grossly visible macrocysts.
  • 38. • Despite the large number of liver cysts, hepatic parenchyma and function are usually preserved • Liver cysts are always preceded by kidney cysts. • Their prevalence in adult polycystic kidney disease increases with age. • In those younger than 20 years, the prevalence of liver cysts is 0%, whereas in those older than 60 years, it is 80% • Two mutations have been found to cause this disorder: – A mutation in the PRKCSH gene that encodes a protein called hepatocystin and – A mutation in the SEC63 gene that encodes for a component of the protein translocation machinery in the endoplasmic reticulum
  • 39. Clinical feature Liver cysts in patients with adult polycystic kidney disease are – Generally asymptomatic, – Numerous large cysts may cause abdominal pain and distention. LFT results are almost always normal. Rare complications can occur; these include infection and intracystic bleeding
  • 40. • Ultrasound and CT reveal multiple simple cysts throughout the liver and kidneys.
  • 41. Managment • Treatment of polycystic liver disease is reserved for – Severe symptoms related to large cysts and complications. • Treatment includes: – Percutaneous aspiration with or without sclerotherapy, – Cyst fenestration (by laparotomy or laparoscopy), – Hepatic resection, – Orthotopic liver transplantation
  • 42. • Liver transplantation is used – only with progressive disease • After fenestration • or Resection with liver • or Renal dysfunction. • In the context of renal failure, a combined kidney and liver transplantation may be appropriate
  • 43. Bile Duct Cysts • Bile duct cysts or choledochal cysts • Are congenital dilations of the biliary tree that are usually diagnosed in childhood but can present in adulthood. • Because of the risk of malignancy and recurrent cholangitis, treatment is excision with reestablishment of biliaryenteric continuity. • Most bile duct cysts involve the extrahepatic biliary tree, • but in type IV cysts, there is involvement of the extrahepatic bile duct and intrahepatic ducts. • In contrast, Caroli disease (type V) is characterized by multiple intrahepatic cysts.
  • 44. • Thus, bile duct cysts must be considered in the differential diagnosis of a patient with multiple hepatic cystic lesions. The intrahepatic lesions of type IV bile duct cysts and Caroli disease are multifocal dilations of the segmental bile ducts separated by portions of normal-caliber bile ducts. • Approximately 50% of cases of Caroli disease are associated with congenital hepatic fibrosis; the cysts are diffusely located throughout the liver. In the other 50% of cases, the dilations may be confined to a portion of the liver, usually the left hemiliver. Recurrent bacterial cholangitis usually dominates the clinical course of these diseases, and death generally ensues within 5 to 10 years without adequate treatment. When intrahepatic bile duct cysts are localized, hepatic resection, with or without biliary reconstruction, is the treatment of choice. Treatment of diffuse hepatic involvement is poor; in complicated cases, the only probably effective treatment is transplantation
  • 45. • Extrahepatic cysts presenting as hepatic cystic lesions — – In rare cases, extrahepatic cysts, which are adjacent to the liver (eg, adrenal, renal or mesenteric cystic lesions) may closely mimic hepatic cysts – May be difficult to differentiate from the latter without a surgical intervention
  • 46. Tomorrow, is the first blank page of a 365 page book. Write a good one." - Brad Paisley #Live your Fear # Live your #life