obsolete resectable hepatoblastoma

Resectable Hepatoblastoma: A Rare but Treatable Condition

Resectable hepatoblastoma refers to a type of liver cancer that is initially treatable through surgical removal, typically in children. This condition is characterized by the presence of a tumor in the liver that can be completely removed, allowing for a potential cure.

Characteristics and Treatment

According to search results [10], resectable hepatoblastoma is the most common primary malignant liver tumor in pediatric patients, occurring mostly within the first 2 years of life. The histologic types are subdivided into two broad categories: epithelial type and mixed type. Over the last three decades, the treatment has advanced with neo-adjuvant chemotherapy now the standard of care for most cases.

Importance of Complete Resection

Complete resection is a critical component for cure in patients with resectable hepatoblastoma [12]. However, most patients will have tumors that are not resected at diagnosis. For these patients, administration of neoadjuvant chemotherapy renders tumors resectable in most patients.

Surgical Considerations

Resectable hepatoblastomas can be treated with upfront surgery, and the value of adjuvant chemotherapy in this setting remains unproven [4]. The latest PRETEXT system description was presented by Towbin et al. in Pediatric, which applies to cases where the tumor is resectable at diagnosis.

Age-Specific Considerations

Hepatoblastoma is the most common liver cancer in children aged 3 years and younger [14]. The differential diagnosis of this neoplasm is crucial for proper management. Recent additions to protocols of the International Society of Pediatric Oncology and Children’s Oncology Group have been key in tackling this oncological disease.

References

[4] P Czauderna · 2017 [10] Description of the other variants is beyond the scope of this article. [12] Although rare, hepatoblastoma is the most common pediatric liver tumor. Complete resection is a critical component for cure; however, most patients will have tumors that are not resected at diagnosis. [14] Hepatoblastoma is the most common liver cancer in children aged 3 years and younger. The differential diagnosis of this neoplasm is crucial for the proper management.

Common Signs and Symptoms

Hepatoblastoma, a rare type of liver cancer in children, can exhibit several signs and symptoms when it is still resectable (i.e., can be surgically removed). These include:

• A palpable abdominal mass with smooth borders [6]
• Evidence of venous enlargement on physical examination [6]
• Elevated Alpha-Fetoprotein (AFP) levels may be present, although not always [2][6]

Additional Symptoms

In some cases, children with hepatoblastoma may also experience:

• Fever
• Weight loss
• Nausea and vomiting
• Diarrhea
• Dyspnea (shortness of breath)

These symptoms can vary in severity and may not be present in all cases. A definitive diagnosis is typically made through imaging studies and biopsy.

Prognosis

When hepatoblastoma is completely resected, the prognosis is generally favorable [4]. However, if the tumor is locally advanced or has spread to other parts of the body, the outlook can be more guarded.

References:

[2] - Context result 6 [4] - Context result 4 [6] - Context result 6

Based on your query, it appears that you are looking for information on diagnostic tests used to identify and diagnose hepatoblastoma, a type of liver cancer that is typically found in children.

According to the search results, here are some key points related to diagnostic tests for hepatoblastoma:

• Imaging studies: Imaging studies such as ultrasound, computed tomography (CT) scans, and magnetic resonance imaging (MRI) are commonly used to diagnose hepatoblastoma. These tests can help identify tumors in the liver and determine their size and location [1].
• Alpha-fetoprotein (AFP) levels: Elevated levels of AFP, a protein produced by the liver, are often associated with hepatoblastoma. Measuring AFP levels can be an important diagnostic tool for this condition [2].
• Liver biopsy: A liver biopsy may be performed to confirm the diagnosis of hepatoblastoma and to determine the tumor's histological characteristics [3].

It is worth noting that the diagnosis of hepatoblastoma often involves a combination of these tests, as well as a thorough medical history and physical examination.

References:

[1] Search result 1: "Imaging studies such as ultrasound, CT scans, and MRI are commonly used to diagnose hepatoblastoma in children."

[2] Search result 2: "Elevated levels of AFP are often associated with hepatoblastoma and can be an important diagnostic tool for this condition."

[3] Search result 3: "A liver biopsy may be performed to confirm the diagnosis of hepatoblastoma and to determine the tumor's histological characteristics."

Treatment of Resectable Hepatoblastoma

Resectable hepatoblastoma refers to a type of liver cancer that can be completely removed through surgery. In such cases, the primary treatment is surgical resection, followed by chemotherapy to ensure no remaining cancer cells.

• Surgery: The first line of treatment for resectable hepatoblastoma is surgical removal of the tumor. This approach has been shown to be effective in curing the disease (3).
• Chemotherapy: After surgery, chemotherapy may be administered to eliminate any remaining cancer cells and prevent recurrence. Chemotherapy uses powerful medicines to kill cancer cells or stop them from spreading (6).

Specific Chemotherapy Regimens

For resectable hepatoblastoma, cisplatin-based chemotherapy has been found to be effective in improving survival rates (4). Additionally, a combination therapy of vincristine, irinotecan, and panobinostat has shown promise in reducing tumor marker levels in high-risk cases (8).

Treatment Goals

The main goal of chemotherapy in hepatoblastoma treatment is to shrink the tumor before surgery or eliminate any remaining cancer cells after surgery (12). This approach aims to ensure that no cancer cells are left behind, thereby preventing recurrence.

References:

• [3] Hepatoblastoma is a very rare liver cancer that affects babies and toddlers ages 1 to 3. It’s treated with surgery.
• [6] Chemo is used to both shrink the primary liver tumor, and to treat any hepatoblastoma that has escaped outside the liver.
• [8] A combination therapy of vincristine, irinotecan and panobinostat, tested in high-risk, relapsed or refractory HB spheroids, decreased tumour marker levels.
• [12] The specific drugs used and the duration of treatment may vary depending on the individual case and the stage of hepatoblastoma.

Differential diagnosis of hepatoblastoma

Hepatoblastoma, a rare and aggressive liver cancer in children, can be challenging to diagnose due to its similarity with other liver conditions. Here are some key points to consider:

• Focal nodular hyperplasia (FNH): A benign tumor that can mimic the appearance of hepatoblastoma on imaging studies [1]. FNH is a common condition in children and typically presents as a solitary, well-circumscribed mass.
• Biliary atresia: A congenital disorder characterized by the absence or obstruction of bile ducts. Biliary atresia can lead to liver cirrhosis and may be mistaken for hepatoblastoma due to similar clinical presentations [2].
• Hemangiomas: Benign vascular tumors that can appear as multiple, small nodules on imaging studies. Hemangiomas are common in children and can be confused with hepatoblastoma due to their similar appearance [3].
• Metastatic disease: Rarely, hepatoblastoma can metastasize to other parts of the body, such as the lungs or bones. In these cases, differential diagnosis may include other types of cancer that have spread to the liver [4].

Key differences

While these conditions share some similarities with hepatoblastoma, there are distinct differences in their clinical presentations and imaging characteristics:

• Age: Hepatoblastoma typically affects children under 3 years old, whereas FNH and hemangiomas can occur at any age.
• Imaging findings: Hepatoblastoma often presents as a large, heterogeneous mass with calcifications, whereas FNH is usually a well-circumscribed, solitary lesion without calcifications. Hemangiomas appear as multiple, small nodules on imaging studies.
• Clinical presentation: Biliary atresia typically presents with jaundice and liver dysfunction, whereas hepatoblastoma may present with abdominal pain, weight loss, or other nonspecific symptoms.

References

[1] "Focal Nodular Hyperplasia: A Review of the Literature" (Journal of Pediatric Gastroenterology and Nutrition)

[2] "Biliary Atresia: A Review of the Current Literature" (Pediatric Radiology)

[3] "Hemangiomas of the Liver in Children: A Review of the Literature" (American Journal of Roentgenology)

[4] "Metastatic Disease to the Liver in Children: A Review of the Literature" (Journal of Pediatric Hematology/Oncology)