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Most children with liver tumors present with abdominal distension, a palpable abdominal mass, or both.
Fetal and neonatal presentations include hydramnios, fetal hydrops, congestive heart failure, and respiratory distress. Laboratory studies are performed to assess baseline CBC count, electrolyte levels, liver enzyme levels, liver synthetic function, and I± -fetoprotein (AFP) levels. The initial workup for hepatic masses includes radiographic assessment using ultrasonography to confirm the location and to characterize the consistency as cystic or solid. Hemangiomas are the most common benign liver tumors in children and commonly occur within the first 6 months of life. Afflicted infants generally present with abdominal distension and cutaneous hemangiomas (10% of cases) that suggest the diagnosis. Laboratory abnormalities associated with this tumor include anemia, elevated aspartate transaminase levels, hyperbilirubinemia, and occasionally an elevated I±-fetoprotein (AFP) level.
The natural history for hemangiomas is spontaneous regression in the first 2 years of life; however, treatment is required if cardiac failure or platelet consumption occurs. More recently, the use of propranolol has been shown to help with resolution of these lesions; however, it does require several months to have an effect. Other treatment options include aminocaproic acid, vincristine, and cyclophosphamide.[8, 9, 10] Aminocaproic acid may be used in addition to cryoprecipitate to ameliorate the coagulopathy associated with Kasabach-Merritt syndrome, and antineoplastics may inhibit the proliferation and subsequent extension of the hemangioma. Focal lesions are treated with complete surgical excision or with selective hepatic artery embolization. Radiation therapy is usually avoided because angiosarcomatous degeneration of benign hemangiomas following radiation and spontaneously occurring have been reported. Signs and symptoms include a palpable mass with smooth borders and evidence of venous enlargement on physical examination.
CT scanning reveals a well-circumscribed, multilocular cystic mass with solid septae and stroma (see the images below).
Signs and symptoms may be absent or are nonspecific and include abdominal pain and mass symptoms.
Enhanced axial CT scan through the liver in the arterial phase in a 38-year-old woman referred for gallbladder scanning.
Differentiating FNH from adenomas may require a technetium sulphur colloid scan, which reveals uniform uptake by FNH lesions. Hepatoblastoma (HB) is the most common primary hepatic malignancy in childhood, accounting for 43% of all pediatric liver tumors. Associated laboratory finding abnormalities include an elevated I±-fetoprotein (AFP) level and thrombocytosis. The workup begins with an abdominal ultrasonography to localize the mass and estimate the extent of tumor within the liver.
CT scan showing a hepatoblastoma present in multiple liver segments that is not amenable to surgical resection at the time of presentation. The serum tumor marker, AFP, is obtained during the workup; more than 90% of patients with HB have elevated AFP levels. Future COG protocols plan to introduce a risk-based determination of treatment, with low, intermediate, and high-risk categories. COG protocols have introduced a risk-based determination of treatment with very low-, low-, intermediate-, and high-risk categories. The right anterior consists of segments 5 and 8, and the right posterior consists of segments 6 and 7. Sectors of the liver with tumor location based on the International Society of Pediatric Oncology on Childhood Liver Tumors (SIOPEL) study. Approximately 50% of tumors are deemed unresectable at diagnosis and require chemotherapy before definitive resection can be performed.
Resection of tumors that are multifocal or have major venous involvement should be considered only in selected centers with the capabilities for appropriate postoperative care and the ability to provide for transplant, should that option become necessary. AFP is considered an early marker for recurrence, and elevated levels should prompt thorough investigation. HCC accounts for 23% of pediatric hepatic malignancies and typically presents in 2 incidence peaks: the first is at age 0-4 years and the second is at age 10-14 years.
Patients with HCC typically present with abdominal pain caused by the large size of the lesion (see the image below). More than 70% of these tumors are considered unresectable at the time of presentation and, unlike HB, respond poorly to chemotherapy.
The overall survival rate remains poor, with a recent Surveillance, Epidemiology, and End-Results (SEER) database review showing 5-, 10-, and 20-year survival rates of 24%, 23%, and 8%, respectively.[26] Children with initially resectable disease have a much better prognosis than those who present with advanced or disseminated disease.
Other primary liver tumors include undifferentiated sarcoma, biliary rhabdomyosarcoma, angiosarcoma, and rhabdoid tumors. Hepatic metastases are more common in the pediatric population than primary tumors and may arise from various primary malignancies, including neuroblastoma, Wilms tumor, rhabdomyosarcoma, rhabdoid tumor, non-Hodgkin lymphoma, and adrenal cortical carcinoma. Complete surgical resection of malignant hepatic tumors is considered a key part of attempt at cure.[28] Planning a major hepatic resection begins with adequate imaging studies to ensure resectability.
Resection is typically performed through a bilateral subcostal incision, and, occasionally, a right thoracoabdominal approach is necessary for large lesions arising high in the right lobe. Intraoperative ultrasonography used to assess resectability of a right lobe hepatoblastoma. Laparoscopic and robotic resections of both benign and malignant liver tumors have been described. Unresectability is usually determined by involvement of hilar structures or all hepatic veins, multicentricity, and invasion of inferior vena cava (IVC) or portal vein. The most frequently performed procedure is a right hepatectomy (60%) because hepatoblastomas (HBs) occur 3 times more often in the right lobe than in the left.
In an extended right hepatectomy, the middle hepatic vein is ligated and segment 4 is resected.
Left hepatic lobectomy begins the same way right hepatectomy, with division of the left hepatic artery and left branch of the portal vein.
Major intraoperative complications include hemorrhage, air embolism, tumor embolus, and bile duct injury. Orthotopic liver transplantation was first described in 1968 by Starzl.[29] Hepatoblastoma (HB) now constitutes an indication for 3% of all pediatric liver transplantations. The survival rate after liver transplantation in children with malignant tumors (ie, HB and HCC) at a single center has been reported as 91% at 1 year and 5 years and 82% at 14 years, respectively.[32] More generally, the 5-year survival rate for patients transplanted for HB is 70%. A study of the United Network for Organ Sharing (UNOS) database reported 135 patients undergoing 135 transplants for HB and 43 transplants for HCC with 1-year, 5-year, and 10-year survival of 79%, 69%, and 66% for HB, respectively, and 86%, 63%, and 58% for HCC, respectively.[33] The primary cause of death for both groups was metastatic disease.
The availability of donor organs has increased with the use of split-liver grafting and other "technical variant" techniques, along with living-related liver transplant techniques. Liver transplantation for hepatic hemangioma has been studied in 59 patients in Europe with 1-year, 5-year, and 10-year patient survival rates of 93%, 83%, and 72%, respectively. Early failure of liver transplant (< 30 d) is usually due to vascular complications or primary nonfunction.
Bcl-2 appears to play a role in the anti-apoptotic mechanisms of some hepatoblastoma (HB) subtypes. The Wnt signaling and mutations in the beta-catenin gene have been shown to be present in HB specimens.
Hepatoblastoma found to be invading the inferior vena cava at the time of surgical exploration. Medscape's clinical reference is the most authoritative and accessible point-of-care medical reference for physicians and healthcare professionals, available online and via all major mobile devices.
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Our Drug Interaction Checker provides rapid access to tens of thousands of interactions between brand and generic drugs, over-the-counter drugs, and supplements. On ultrasounds it is usually seen as hyperechoic although in some cases it may be appear as hypoechoic. On MRI we have several imaging sequences to identify the fat: it is characteristic its high signal on T1-weighted images and the loss of signal on fat suppression sequences and opposed-phase gradient-echo sequences. The characteristic finding on any imaging technique is the presence of fat and prominent central vessels. However it can have various appearances due to the variant content of the three components. On ultrasound they usually appear as a hetero or homogeneous echogenic mass commonly in right hepatic lobe. On CT they appear as a well-defined mass with heterogeneous attenuation due to presence of the three components.
On MRI the lesions with high proportion of fat tissue show high signal intensity on T1 and T2-weighted images with a loss of signal on fat suppress sequences.
However 50% of hepatic angiomyolipoma lack considerable fat content so it is difficult to make an acute radiological diagnosis with the other hepatic tumors. On CT lipomas are homogenous and show fat attenuation (-20 to -70 HU) without contrast enhancement. On MRI lipomas show typical characteristics of a fatty lesion (high signal on T1-weighted images and fat suppression on fat suppressed sequences).
The combination of in-phase and out-of-phase T1-weighted gradient-echo imaging is an excellent technique to differentiate focal hepatic steatosis from metastases.
Focal hepatic steatosis is isointense or hyperintense to liver on in-phase and loses signal on out-of-phase images. In 20% of cases they have the characteristic “spoke-wheel” pattern of prominent arterial flow on Color and Power Doppler examination. On CT and MRI focal nodular hyperplasia shows intense contrast enhancement except the central scar which is unenhanced in the arterial phase. Intratumoral steatosis may be or not associated with diffuse steatosis and is better observed on MRI. The present of glycogen and fat is the responsible for the high signal on T1-weighted images. Chemical shift MR imaging can confirm the presence of fat by showing a loss signal on out-of-phase imaging.
Mendez Montero et al suggested that fat-fluid levels inside hydatid cysts derives from the lipid elements in bile caused by a communicating rupture in the cyst wall. Langerhans cell histiocytosis is a multisystemic disorder characterized by uncontrolled monoclonal proliferation of Langerhan cells (distinctive cells of monocyte-macrophage lineage). Hepatic involvement may be from metastases although isolated cases of primary hepatic liposarcomas have been reported.
The signal intensity drop on chemical shift images could be very useful in the detection of lipomatous nodules in cirrhotic liver; however benign regenerative nodules can also contain fat.
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CT imaging of presurgical evaluation of potential renal transplant donors: What do surgeons should know? Confirmation of diagnosis of multiple liver adenomas with signs of recent bleedingCase DiscussionHepatic adenoma is a benign liver tumor that can degenerate into a malignant form.Causes of hepatic adenoma are not entirely known. Auf dieser Seite finden Sie alles wissenswerte uber unser Angebot und unseren vielseitigen Service. Auf dieser Seite finden Sie alles wissenswerte über unser Angebot und unseren vielseitigen Service. The liver is the third-most-common site for intra-abdominal malignancy in children, following adrenal neuroblastoma and Wilms tumor. AFP levels are elevated in 50%-70% of children with hepatic neoplasms, and multiple studies confirm that AFP is a valuable surveillance marker in children who have previously undergone hepatic resection for malignancy. They have endothelial-lined vascular spaces and vary from small incidentally found masses to large cavernous hemangiomas that are distinguished by large vascular spaces and lack of cellularity.
Most hemangiomas are incidentally discovered on imaging studies.[3] As many as 50% of these infants have high-output cardiac failure at initial presentation.
The significance of an elevated AFP level is unknown because the levels can be elevated in healthy neonates and does not decrease to normal adult levels until age 6 months. Several treatment options are available, and all are associated with potential severe complications and poor outcome. Supportive care may include liberal use of diuretics and digitalis to improve the cardiac function in cases of failure.[6] Correction of anemia and coagulopathy is performed with blood product replacement. As much as 50% regression has occurred in some reports; however, the response time is slow, and lesions can rebound once the drug is stopped. Selective hepatic artery embolization may not be as successful for multifocal lesions as it is for focal lesions.
Rarely, liver transplantation may be indicated for diffuse disease that is unresponsive to steroid and interferon therapy. They can be considered to be more of a malformation than true tumors, although they present as masses with their organ of origin. Both of these benign lesions have an association with a high estrogen environment and frequently occur in adolescent girls. Hepatocellular carcinomas (HCCs) and hepatoblastomas (HBs) have been reported after a benign diagnosis on imaging studies.[3] Open biopsy may be required for definitive diagnosis in rare circumstances, especially if observation is considered (see the image below). Many surgeons advocate elective resection to prevent spontaneous rupture and hemorrhage; however, other surgeons follow these lesions with serial ultrasonography monitoring.
Doppler evaluation can be used to evaluate the patency of the inferior vena cava, the hepatic veins, and the portal vein.
In one series, the pure fetal hepatoblastoma subtype demonstrated 50% survival in compared with those who had fetal and embryonal histology (30% survival).[1] A recent Children's Oncology Group (COG) study has shown that complete resection of stage I patients with pure fetal histology are cured with surgery alone and require no chemotherapy. In the United States, staging of tumors is based on the extent of tumor and outcome of surgical resection as critical criteria.
The left lateral consists of segments 2 and 3, and left medial consists of segments 4a and 4b. Characteristics of an unresectable tumor include multicentricity, invasion of the IVC or portal vein, or distant metastases. Major complications following resection have been reported to be as much as 20-30%, with complications following resection of HB more prevalent than complications after resection of hepatocellular carcinoma (HCC).
The survival rate has steadily improved over the last 3 decades, and the overall survival rate is currently 85%-92%. Predisposing conditions include hepatic fibrosis and cirrhosis secondary to metabolic liver disease, viral hepatitis, extrahepatic biliary atresia, total parenteral nutrition, and chemotherapy-induced fibrosis. Multiple lesions, intravascular spread and metastases are more common in HCC compared with HB. Combination chemotherapy, as is used for HB, has been administered to patients with HCC but has been largely ineffective in shrinking tumors to the point of respectability and in eradicating metastases. Doppler ultrasonography used in combination with MRI provides valuable information regarding the vascular and biliary anatomy. The hilar plate is divided, exposing the bifurcation of the hepatic artery and portal vein. The left and middle hepatic veins are identified after dissection through the sinus venosus. Postoperative complications include hemorrhage, bile leak, abscess formation, pulmonary complications, and wound problems.
Additionally, successful transplantation has been used for hepatocellular carcinoma (HCC) and benign lesions such as diffuse hepatic hemangiomas. The criteria currently used to evaluate adult transplant candidates may not be applicable for pediatric patients.
Late failure is usually more a result of infection, posttransplant lymphoproliferative disease, chronic rejection, biliary complications, or recurrence of malignant disease. Resolution of medically resistant hypothyroidism after liver transplantation for hepatic hemangioendothelioma.


Efficacy of propranolol treatment in thyroid dysfunction associated with severe infantile hepatic hemangioma.
Cyclophosphamide and radiation therapy in the treatment of hemangioendothelioma with disseminated intravascular clotting. Treatment of severe coagulopathy in the Kasabach-Merritt syndrome with aminocaproic acid and cryoprecipitate. Focal nodular hyperplasia and hepatic adenoma: a review of eight cases in the pediatric age group.
Hepatoblastoma with a low serum alpha-fetoprotein level at diagnosis: the SIOPEL group experience. 2005 PRETEXT: A revised staging system for primary malignant liver tumours of childhood developed by the SIOPEL group. Surgical treatment of primary liver tumors in children: outcomes analysis of resection and transplantation in the SEER database. Orthotopic liver transplantation for unresectable hepatoblastoma: a single center's experience. Liver transplantation for childhood hepatic malignancy: a review of the United Network for Organ Sharing (UNOS) database. The place of liver transplantation in the treatment of hepatic epitheloid hemangioendothelioma: report of the European liver transplant registry. Sonogram shows mass effect from the tumor, as demonstrated by the arching of the portal vein anteriorly.
Topics are richly illustrated with more than 40,000 clinical photos, videos, diagrams, and radiographic images. The articles assist in the understanding of the anatomy involved in treating specific conditions and performing procedures.
Check mild interactions to serious contraindications for up to 30 drugs, herbals, and supplements at a time. The incidence of primary malignant liver tumors per year is 1-1.5 per million children in the United States.
CT scanning and MRI (MRI) of the abdomen and chest are used for indeterminate or solid lesions to further delineate the location, extent, and multiplicity of the lesions and to detect metastases. Hemangioendothelioma is a subtype of hemangioma that is typically found in infants (see the images below).
Platelet sequestration and consumptive coagulopathy are rarely evident in these children (see Kasabach-Merritt Syndrome), and they may present with hemorrhage and respiratory distress. This regimen is discontinued if no response is observed, in order to avoid steroid-induced complications. Case reports have described spastic diplegia in very young children who have received interferon, but the actual risk of this therapy is unknown.
They are typically diagnosed when the patient is younger than 2 years.[12] They usually grow during the first few months of life, then may stabilize, grow, or regress. A 3-phase CT scan is the optimal study to make the diagnosis of FNH, including an arterial phase, portal venous phase, and delayed images.
If the lesions are symptomatic or rapidly enlarging, complete surgical resection, embolization, or hepatic artery ligation may be used for treatment. An increased risk of HB is noted in association with hemihypertrophy and Beckwith-Wiedemann syndrome, which indicates possible involvement of a chromosome 11 deletion.[18] In addition, an increased incidence of HB is associated with familial adenomatous polyposis syndrome as well as case series of HB associated with trisomy 18. CT scanning of the abdomen and chest is used to assess resectability and evaluate for the presence of pulmonary metastasis (see the images below). Already, they have discovered that the very lowa€“risk group is cured with surgery alone and no longer requires adjuvant chemotherapy.
Involvement of the caudate lobe (segment 1) is given separate staging consideration, as are extrahepatic disease, tumor focus, tumor rupture, distant metastasis, lymph node involvement, portal, hepatic, and inferior vena cava (IVC) involvement. Isolated pulmonary metastases that persist after neoadjuvant chemotherapy may be treated with pulmonary metastasectomy.[22] Adequate response to chemotherapy is observed in 70% of patients who then go on to complete resection followed by additional postoperative chemotherapy. Vincristine, cisplatin, 5-fluorouracil (5-FU), and doxorubicin have had little impact on the progression of this disease. Current criteria for resection of these hepatic metastases include control of the primary tumor, a solitary or limited number of metastases, and a reasonable expectation of prolonged survival.
The PRETEXT system was developed by the International Society of Pediatric Oncology on Childhood Liver Tumors (SIOPEL) group to identify suitable candidates for primary resection.[21] This system is being adopted internationally to provide a universal language for surgeons and will be helpful for those who see such cases infrequently. Intraoperative ultrasonography has been widely applied to determine the exact location of the tumor relative to the vessels.
Only 20% of the liver is necessary to maintain hepatic function; thus, postoperative insufficiency is rare. The main indication for transplantation is nonmetastatic, unresectable lesions.[30] Transplantation may also be used in selected cases of tumor recurrence but is much less successful when used for salvage therapy.
Because no good medical therapy for pediatric HCC has been identified, liver transplantation should be carefully evaluated as front-line therapy.
CT images show various focal regions of low attenuation in the liver, a finding consistent with focal geographic fatty infiltration. CT shows a low-attenuation change of the hepatic parenchyma caused by severe hepatic steatosis.
Axial fat-suppressed T2-weighted fast spin-echo sequence (1), axial T1-weighted in-phase MRI (2), axial T1-weighted out-of-phase MRI (3).
Axial fat-suppressd T2-weighted fast spin-echo sequence (1), axial T1-weighted in phase MRI (2), axial T1-weighted out-of-phase MRI (3).
These modalities facilitate surgical planning and may determine resectability; however, definitive diagnosis can be proven only through biopsy findings. These tumors are more common in the right lobe of the liver.[3] They are often multicystic, heterogeneous, confined to one lobe, and asymptomatic. During the arterial phase, an FNH lesion appears as an early contrast-enhanced homogenous lesion that becomes isodense with the normal liver parenchyma on delayed images.
Future whole-genome sequencing may elucidate the combination of genetic and epigenetic changes that drive tumorigenesis in HB.
Hepatic angiography or MRI angiography is frequently helpful preoperatively to determine resectability because it delineates the vascular anatomy more precisely. Proponents of this therapy argue that the cumulative toxicity of chemotherapy can be reduced, some agents can be entirely avoided, and a reduction of in vivo development of tumor resistance may also occur. Liver function test findings are routinely elevated; the AFP level is elevated in approximately half of the cases.
Once deemed resectable, the resection is marked out, and various tools may then be used to perform the resection; electrocautery, bipolar devices such as LigaSure, and argon beam coagulation for hemostasis have been used. The right hepatic vein is identified and ligated before any division of the hepatic parenchyma. Postoperative care consists of adequate fluid replacement, intravenous albumin supplementation, vitamin K, and clotting factors for the first 3-4 days. In addition, liver transplantation may be an option in children with unresectable primary tumors, without metastatic disease, after neoadjuvant chemotherapy and pulmonary metastasectomy, if necessary. The discovery of a hepatic adenoma requires periodic checks.When more than ten adenomas are present the term adenomatosis is used. Of these malignant tumors, hepatoblastoma (HB) and hepatocellular carcinoma (HCC) are the most common and account for two thirds of all hepatic neoplasms.
An opportunity to delay resection until after neoadjuvant therapy is observed in patients with stage III and IV tumors.
Newer therapeutic strategies have included chemoembolization, intra-arterial chemotherapy, and intraoperative cryotherapy.
The liver function test results generally normalize within the first 2 weeks, and hepatic insufficiency is reasonably rare. Postoperative monitoring consists of frequent ultrasonography, chest radiography, and serial I± -fetoprotein (AFP) level measurements, generally at 3-month to 6-month intervals.



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