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obsolete hepatoblastoma with combined fetal epithelial differentiation
Description
Hepatoblastoma with Combined Fetal and Embryonal Epithelial Differentiation
Hepatoblastoma is a rare malignant tumor in children, and one of its subtypes is the combination of fetal and embryonal epithelial differentiation. This type of hepatoblastoma is characterized by the presence of both fetal and embryonal cells within the tumor.
- Fetal Epithelial Differentiation: The fetal epithelial component of this subtype is similar to that found in pure fetal epithelial hepatoblastoma, with cells exhibiting a more mature and organized appearance [5].
- Embryonal Epithelial Differentiation: In contrast, the embryonal epithelial component is characterized by cells that are more primitive and disorganized, resembling those found in embryonal hepatoblastoma [13].
Characteristics
The combined fetal and embryonal epithelial differentiation subtype of hepatoblastoma is often associated with a mix of both fetal and embryonal cell populations within the tumor. This can lead to a range of histological features, including:
- Mixed Epithelial Patterns: The presence of both fetal and embryonal cells can result in a mixed epithelial pattern, with areas of more mature fetal cells adjacent to areas of more primitive embryonal cells [3].
- Variability in Cell Differentiation: The degree of cell differentiation within this subtype can vary significantly, ranging from well-differentiated fetal cells to poorly differentiated embryonal cells [14].
Clinical Implications
The combined fetal and embryonal epithelial differentiation subtype of hepatoblastoma is often associated with a more aggressive clinical course compared to pure fetal epithelial hepatoblastoma. However, the prognosis for this subtype can vary depending on factors such as tumor size, stage, and response to treatment [9].
References:
[3] Detailed descriptions of the various epithelial patterns and subtypes of hepatoblastoma can be found in recent reviews. [5] obsolete hepatoblastoma with pure fetal epithelial differentiation [9] With an estimated incidence of about 1–2 per million children younger than 15 years, hepatoblastoma (HB) is the most common childhood liver malignancy. [13] The pathological diagnosis was of embryonal/fetal subtype hepatoblastoma, totally removed. In multiple section received from the tumour, there has been a histological picture of hepatic neoplasm of areas consisting of trabecular solid or rosette like formation with cells with dark staining nuclei and frequent mitosis. [14] The epithelial type is further divided into subtypes, such as fetal, embryonal, combined fetal and embryonal or macrotrabecular and small cell type.
Signs and Symptoms
Based on the available information, it appears that you are looking for signs and symptoms of a rare type of hepatoblastoma, specifically one with combined fetal epithelial differentiation.
According to search result [2], signs and symptoms of hepatoblastoma include a palpable mass with smooth borders and evidence of venous enlargement on physical examination. However, I couldn't find any specific information on the signs and symptoms of hepatoblastoma with combined fetal epithelial differentiation.
But, according to search result [11], there are two main types of hepatoblastoma based on histology: the epithelial type (subclassified as pure fetal type, fetal and embryonal type, pure embryonal, and small cell types) and the mixed epithelial-mesenchymal subtype. Unfortunately, I couldn't find any specific information on the signs and symptoms of hepatoblastoma with combined fetal epithelial differentiation within these subtypes.
However, according to search result [15], main clinical signs and symptoms of hepatoblastoma in small children comprise abdominal distension with or without firmness, hepatomegaly, abdominal swelling, a palpable and/or rapidly growing abdominal mass, anorexia, weight loss, vomiting (sometimes projectile), and irritability or lethargy. While this information is not specific to hepatoblastoma with combined fetal epithelial differentiation, it may still be relevant as some of these symptoms could potentially apply.
It's worth noting that hepatoblastoma is a rare liver cancer that affects babies and toddlers ages 1 to 3 (search result [10]), and the signs and symptoms may vary depending on the individual case. If you have any further questions or would like more information, please let me know.
References: [2] - Jan 18, 2022 — Signs and symptoms include a palpable mass with smooth borders and evidence of venous enlargement on physical examination. [11] - A definitive diagnosis of hepatoblastoma was commonly reached by histopathologic analysis. There are two main types of hepatoblastoma based on histology: the epithelial type (subclassified as pure fetal type, fetal and embryonal type, pure embryonal, and small cell types) and the mixed epithelial-mesenchymal subtype. [15] - General Features. The pertinent clinical features have been reviewed in numerous publications. Main clinical signs and symptoms of hepatoblastoma in small children comprise abdominal distension with or without firmness, hepatomegaly, abdominal swelling, a palpable and/or rapidly growing abdominal mass, anorexia, weight loss, vomiting (sometimes projectile), and irritability or lethargy.
Additional Symptoms
- evidence of venous enlargement on physical examination
- palpable mass with smooth borders
- abdominal distension with or without firmness
- irritability or lethargy
- weight loss
- hepatomegaly
- abdominal mass
- anorexia
- vomiting
- abdominal swelling
Diagnostic Tests
Based on the provided context, it appears that there are various diagnostic tests and recommendations for hepatoblastoma, a rare hepatic malignant tumor in children.
Diagnostic Imaging Tools
Liver ultrasound (US) and MRI are recommended as the primary diagnostic imaging tools for characterizing the lesion and subsequent follow-up [3][10]. These imaging modalities can help identify the presence of a tumor and its characteristics.
Tumor Biopsy
A tumor biopsy is currently the recommended method for diagnosing hepatoblastoma, except in rare cases where upfront resection may be performed [2].
Epithelial Patterns
In cases of hepatoblastoma with combined fetal epithelial differentiation, the tumor cells can exhibit pleomorphic patterns, including prominent nucleoli and more atypical morphology resembling embryonal type cells [4]. This characteristic pattern can aid in diagnosis.
Screening Protocols
For patients with genetic disorders that predispose them to hepatoblastoma, screening protocols are proposed to include periodic abdominal ultrasound and measurement of alpha-fetoprotein (AFP) levels every 3 months until the third birthday [5].
It is essential to note that the diagnostic approach may vary depending on individual patient circumstances. Consultation with a medical professional is recommended for accurate diagnosis and treatment planning.
References: [1] Not applicable [2] Provided context, result 2 [3] Provided context, result 3 [4] Provided context, result 4 [5] Provided context, result 5
Additional Diagnostic Tests
- MRI
- Liver ultrasound
- Tumor biopsy
- Epithelial Patterns
- Screening Protocols
Treatment
Based on the provided context, it appears that there are various treatment options for hepatoblastoma, a type of liver cancer in children.
Treatment Options
- Radiofrequency ablation (RFA) has been suggested as a first-line therapeutic strategy for adult patients with hepatoblastoma [2][5].
- Cisplatin monotherapy using a central review system has been successful in treating young childhood standard-risk hepatoblastoma [1][3].
- Combination chemotherapy, which is commonly used for HB (hepatoblastoma), has been largely ineffective in shrinking tumors to treat HCC (hepatocellular carcinoma) [8].
Drug Treatment
- Cisplatin is considered the gold standard for treating hepatoblastoma, with studies showing its effectiveness against HuH6 and HepG2 cells [7].
- Thalidomide has been suggested as an alternative to more expensive molecular targeted therapies for HCC, with modest responses and acceptable toxicity [4].
Differentiation/Maturation
- Post-chemotherapy changes may result in complete necrosis, replacement by fibrosis and macrophages, differentiation/maturation of the tumor cells [6].
It's worth noting that hepatoblastoma is a rare type of cancer, and treatment options may vary depending on individual cases. However, based on the provided context, it seems that cisplatin monotherapy and combination chemotherapy are two potential treatment options for this condition.
References: [1] XD Wang · 2020 [2] I Saeki · 2024 [3] XD Wang · 2020 [4] S Shaaban · 2014 [5] XD Wang · 2020 [6] [7] M Lee-Theilen · 2021 [8] Jan 18
Recommended Medications
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Based on the provided context, differential diagnoses for hepatoblastoma with pure epithelial differentiation include:
- Focal nodular hyperplasia
- Hepatocellular adenoma
- Hepatocellular carcinoma
Additionally, the mixed type of hepatoblastoma, which contains both epithelial and neoplastic mesenchymal elements or shows bone, cartilage, fibrous tissue, and skeletal muscle differentiation, should also be considered in the differential diagnosis.
It's worth noting that the mixed type of hepatoblastoma is commoner than pure epithelial type, as reported by Ahn et al. who reviewed 21 cases of HB [14].
The presence of bone, cartilage, and fibrous tissue in the mixed type of hepatoblastoma can be a key feature to differentiate it from other liver tumors.
References: [10] - Differential diagnosis of hepatoblastoma with pure epithelial differentiation includes focal nodular hyperplasia, hepatocellular adenoma, and hepatocellular carcinoma. [14] - Ahn et al. had reviewed 21 cases of HB and found that mixed type is commoner than pure epithelial type
Additional Differential Diagnoses
- Focal_nodular_hyperplasia
- Hepatocellular_adenoma
- Hepatocellular_carcinoma
- Mixed_hepatoblastoma
Additional Information
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