Websurg, e-Surgery 關於腹腔鏡手術

Clinical Case

HEPATIC LESION

F Jamali, MD, D Mutter, MD, PhD, Hôpitaux Universitaires de Strasbourg, Strasbourg, France

1. Summary

The increasingly widespread use of imaging modalities has led to a rise in the discovery of unsuspected hepatic lesions. This multiple choice question and answer case illustrates the differential diagnosis, workup and management of such a lesion.

2. Patient

19-year-old woman

3. Past medical and surgical history

No significant past medical or surgical history except for a normal delivery 7 months ago.

4. History of present illness

The patient presents with vague epigastric and right upper quadrant abdominal pain associated with early satiety.

5. Physical examination

A large, firm palpable mass is felt in the epigastric region. It is non-tender and mobile. No suspicious adenopathy. The remainder of the physical exam is normal.

6. Laboratory values

CRP: 100 (N<20)
Hct: 29% (N= 35-55)
Rest of routine laboratory workup is negative.

7. Ultrasonography

Hepatic solid tumor located in the left lobe measuring 15cm in diameter with some free peritoneal fluid.

8. Quizzes

8.1. Quiz 1

What are the 3 most common solid hepatic tumors?
1) Hepatic metastases from a distant primary neoplasm
2) Primary liver carcinoma: HCC or fibrolamellar variant
3) Liver cell adenoma
4) Focal nodular hyperplasia
5) Hepatic hemangioma
6) Hepatic sarcoma
7) Angiomyolipoma
Correct answers:
1) Hepatic metastases from a distant primary neoplasm
Metastatic neoplasms represent the most common malignant tumors of the liver in the Western world. The relative proportion of primary to secondary liver neoplasms is estimated to be 1:20.
3) Liver cell adenoma
The frequency of hepatic adenomas has risen significantly since the introduction of the birth control pill in 1960. These lesions occur almost exclusively in women.
5) Hepatic hemangioma
It is the most common nodule found in the liver. It is five times more frequent in females than in males.
Wrong answers:
2) Primary liver carcinoma: HCC or fibrolamellar variant
Primary liver carcinoma is relatively uncommon in the Western world; however its incidence is high in parts of Asia and Africa. Chronic hepatitis B and C virus infections are the principal etiologic factor worldwide.
4) Focal nodular hyperplasia
A benign lesion with no malignant potential. It is relatively less common compared to liver cell adenomas. It is found in women twice as often as in men.
6) Hepatic sarcoma
Extremely rare and more often metastatic in nature when present.
7) Angiomyolipoma
Angiomyolipoma (AML) is a benign mesenchymal tumor predominantly occurring in the kidney. It has a low incidence of 0.07-0.03% in an unselected population.

8.2. Quiz 2

Which tumor markers are most helpful in establishing a diagnosis?
1) CEA
2) CA-125
3) AFP
4) HCG
5) CA 19-9
Correct answers:
1) CEA
Could be helpful to distinguish colorectal metastases from the more infrequently encountered upper GI malignancies.
CEA was normal in this case.
3) AFP
Elevated in 83-97% of cases of HCC. It can be predictive of response to treatment and therefore useful for follow-up purposes.
It is normal in this case.
5) CA 19-9
Elevated in upper GI malignancies (pancreas) and cholangiocarcinomas. It is normal in this patient.
Reference:
Williams R, Melia WM, Johnson PJ. Serum alpha-foetoprotein in hepatocellular carcinoma - - value in diagnosis, and prognosis. Ann Acad Med Singapore 1980;9:245-50.
Wrong answers:
2) CA-125
It is primarily used as a marker in ovarian carcinoma. It is non-specific in this setting.
4) HCG
It is elevated in testicular or other germ cell tumors. It is normal in this case.

8.3. Quiz 3

What is your next step in the management of this patient?
1) Further imaging
2) Fine needle aspiration
3) Core biopsy under ultrasound guidance
4) Laparoscopic biopsy
5) Exploratory laparotomy
Correct answer:
1) Further imaging
The first step in the management of this patient should be further imaging:
1. To better define the nature of the lesion;
2. To search for a primary in case this represents a metastatic lesion;
3. To rule out a hepatic hemangioma, the biopsy of which is relatively contraindicated.
FNA, core biopsy, laparoscopic biopsy or exploratory laparotomy are all options that will be useful in time. At the current moment, the best approach is to utilize appropriate non-invasive imaging modalities to try to establish a diagnosis or narrow the differential.

8.4. Quiz 4

Concerning hepatic hemangiomas, which of the following statement(s) is/are true?
1) They have a typical appearance on ultrasonography.
2) They are easily diagnosed using non-contrast CT scanning.
3) They are best diagnosed using MRI.
4) The agent of choice for radionuclide imaging of hepatic hemangiomas is Tc99m sulfur colloid.
5) Arteriography is the ideal modality for diagnosis.
Correct answer:
3) They are best diagnosed using MRI.
MRI is highly sensitive for the imaging of hepatic hemangiomas. T2 densities greater than 80 ms characterize hemangiomas.
Reference:
Birnbaum BA, Weinreb JC, Megibow AJ, Sanger JJ, Lubat E, Kanamuller H et al. Definitive diagnosis of hepatic hemangiomas: MR imaging versus Tc-99m-labeled red blood cell SPECT. Radiology 1990;176:95-101.
Wrong answers:
1) They have a typical appearance on ultrasonography.
Ultrasonographic appearance of hemangiomas varies greatly and overlaps with the appearance of other benign and malignant lesions.
2) They are easily diagnosed using non-contrast CT scanning.
Routine CT fails to discriminate hemangiomas from other hepatic lesions. Contrast-enhanced sequential scanning showing accumulation of the contrast in the lesion spreading from the periphery of the lesion towards its center is characteristic of cavernous hemangiomas.
4) The agent of choice for radionuclide imaging of hepatic hemangiomas is Tc99m sulfur colloid.
The ideal agent for radionuclide imaging of hepatic hemangiomas is Tc99m labelled red blood cells.
5) Arteriography is the ideal modality for diagnosis.
Arteriography can be helpful in establishing the diagnosis but it is invasive and rarely indicated for diagnostic purposes.

8.5. Quiz 5

A central stellate scar on CT scan or Tc99M sulfur colloid scanning is typical of which lesion?
1) Hepatic adenoma
2) Focal Nodular Hyperplasia (FNH)
3) Hepatocellular carcinoma (HCC)
4) Hemangioma
5) Metastatic lesions
Correct answer:
2) Focal Nodular Hyperplasia (FNH)
FNH is present in up to 4% of the population. It is most common in women near the age of 40. Rarely symptomatic (<10%), it has a typical tan to dark brown appearance with prominent vessels. It may be multiple in up to 20% of cases. It is unusual for this lesion to enlarge or cause complications.
Reference:
Nagorney DM. Benign hepatic tumors: focal nodular hyperplasia and hepatocellular adenoma. World J Surg 1995;1:13-8.

8.6. Quiz 6

Rapid growth during pregnancy and involution after stopping oral contraceptive therapy is characteristic of which lesion?
1) HCC
2) Hemangioma
3) Hepatic adenoma
4) Metastatic lesion
5) Focal nodular hyperplasia
Correct answer:
3) Hepatic adenoma
Hepatic adenoma is a lesion most commonly found in women using oral contraceptives. It is symptomatic in up to 80% of patients. It is difficult to distinguish from FNH on the basis of radiologic criteria alone but typically appears as a hypodense solid lesion on CT scan. Radionuclide scanning reveals a cold nodule due to the lack of Kupffer's cells and bile ducts. The typical gross appearance is one of a soft fleshy tumor, yellow to tan in color. If the diagnosis of adenoma is suspected in a lesion <5cm, a trial of discontinuing oral contraceptives should be considered as it leads to regression of some lesions. Due to the 10% risk of presence of HCC in adenomas as well as their propensity to be symptomatic and to bleed, surgical resection is advocated. For small, centrally located lesions, a period of observation with close follow-up may be considered.
Reference:
Nagorney DM. Benign hepatic tumors: focal nodular hyperplasia and hepatocellular adenoma. World J Surg 1995;1:13-8.

9. CT scan

The patient undergoes a CT scan, revealing a large hepatic lesion measuring approximately 15 cm in diameter replacing most of the left lobe of the liver.
It also shows a suspect left adrenal lesion and possible retroperitoneal adenopathy.

Figure 9.a

Figure 9.b

Figure 9.c

Figure 9.d

10. MRI

The lesion does not have the characteristics of a hemangioma. It has a heterogeneous appearance with areas of varying enhancement.

Figure 10.a

Figure 10.b

11. Quizzes

11.1. Quiz 1

How does the detection of a possible left adrenal lesion on CT and MRI affect your management?
1) Not at all
2) FNA of the adrenal lesion is indicated to establish a diagnosis.
3) A preoperative hormonal workup is indicated.
4) The presence of an adrenal metastasis contraindicates surgical resection.
Correct answer:
3) A preoperative hormonal workup is indicated.
The presence of a possible adrenal lesion mandates a preoperative hormonal workup to rule out a functioning malignant pheochromocytoma with liver metastases. Although remote, this scenario would mandate preoperative preparation with alpha or calcium channel blockade in case of a surgical intervention and must be kept in mind. The hormonal workup was negative in this case.

11.2. Quiz 2

What is your next step in the management of this patient?
1) Fine needle aspiration
2) Core biopsy under ultrasound guidance
3) Laparoscopic biopsy
4) Exploratory laparotomy with resection
Correct answers:
1) Fine needle aspiration
2) Core biopsy under ultrasound guidance
3) Laparoscopic biopsy
4) Exploratory laparotomy with resection
The priority now should be one of establishing a tissue diagnosis to guide therapy. All of the above options are valid approaches. We chose to perform a diagnostic laparoscopy to confirm the hepatic origin of the lesion and rule out unresectability.

12. Laparoscopic exploration

The patient undergoes a laparoscopic exploration to confirm resectability. This video illustrates the laparoscopic findings.
Having ruled out diffuse carcinomatosis, we decided to proceed with a laparotomy, left lobectomy and retroperitoneal LN dissection. There were multiple celiac and retroperitoneal adenopathies. The suspected image of the adrenal tumor represented in fact a retroperitoneal enlarged LN. This agressive approach was justified in our mind by the young age of the patient.
video

Video 12

13. Pathologic findings

Fibrolamellar carcinoma, metastatic to regional LN and peritoneal surface.

14. Quizzes

14.1. Quiz 1

Concerning fibrolamellar carcinoma, which of the following statement(s) is/are true?
1) It is typically associated with a marked elevation of liver enzymes.
2) It is more common in older patients.
3) It typically presents as a large hepatic mass in adolescents and young adults with no underlying liver disease.
4) It is typically associated with an elevated Alpha Feto-Protein (AFP) level.
Correct answer:
3) It typically presents as a large hepatic mass in adolescents and young adults with no underlying liver disease.
Fibrolamellar carcinoma is an uncommon variant of hepatocellular carcinoma, typically seen in young adults without underlying liver disease. Physical signs are minimal and laboratory values are non-contributory. It is usually not associated with any elevation of any of the tumor markers.
References:
Schlitt HJ, Neipp M, Weimann A, Olfhafer KJ, Schmoll E, Boeker K et al. Recurrence patterns of hepatocellular and fibrolamellar carcinoma after liver transplantation. J Clin Oncol 1999;1:324-31.
McLarney JK, Rucker PT, Bender GN, Goodman ZD, Kashitani N, Ros PR. Fibrolamellar carcinoma of the liver: radiologic-pathologic correlation. Radiographics 1999;2:453-71.

14.2. Quiz 2

Concerning fibrolamellar carcinoma, which of the following statement(s) is/are true?
1) It has a more aggressive course than hepatocellular carcinoma (HCC).
2) It is more common in female patients.
3) It typically arises in a background of cirrhosis or chronic hepatitis (B or C).
4) It has a typical homogeneous appearance on imaging modalities.
5) It has a central scar on T2-weighted MRI in 80% of cases.
Correct answer:
5) It has a central scar on T2-weighted MRI in 80% of cases.
Individuals with fibrolamellar carcinoma generally have a better survival than those with HCC. There is no gender predominance in fibrolamellar carcinoma in most reports. Known risk factors for HCC such as cirrhosis, alcohol abuse, metabolic disease and viral hepatitis are typically absent in fibrolamellar carcinoma. The typical appearance on CT and MRI is one of a heterogeneous solid lesion with a central scar. Therefore it must be differentiated from FNH, which requires no surgical therapy.
Reference:
Ichikawa T, Federle MP, Grazioli L, Madariaga J, Nalesnik M, Marsh W. Fibrolamellar hepatocellular carcinoma: imaging and pathologic findings in 31 recent cases. Radiology 1999;2:352-61.

14.3. Quiz 3

Concerning fibrolamellar carcinoma, which of the following statement(s) is/are false?
1) Metastatic unresectable fibrolamellar hepatoma has a median survival of 14 months.
2) Surgical therapy remains the gold standard for treatment.
3) Adjuvant therapies are very effective in prolonging survival.
4) The most common cause of death is liver failure.
Correct answer:
3) Adjuvant therapies are very effective in prolonging survival.
Surgical therapy remains the only hope for cure in fibrolamellar carcinoma. The Johns Hopkins group experience demonstrates a median survival of 14 month in unresectable metastatic fibrolamellar carcinoma. Adjuvant therapies including radiation therapy, chemotherapy, hepatic artery embolization and radiolabelled antibodies have been used with limited efficacy. The most common cause of death in fibrolamellar carcinoma is liver failure followed by metastatic disease.
Reference:
Epstein BE, Pajak TF, Haulk TL, Herpst JM, Order SE, Abrams RA. Metastatic ono-resectable fibrolamellar hepatoma: prognostic features and natural history. Am J Clin Oncol 1999;1:22-8.

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