TREATMENT/MANAGEMENT OF OTHER HEPATIC TUMORS
Pediatric liver neoplasias are rare, comprising 1–4% of all solid childhood tumors. More than half of these masses are due to hepatoblastoma and hepatocellular carcinomas, making remaining various liver tumors much more rarer.Hepatic mesenchymal hamartoma (HMH) is a benign tumor observed in children...
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| Format: | Article |
| Language: | English |
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Elsevier
2022-10-01
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| Series: | Hematology, Transfusion and Cell Therapy |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S253113792201330X |
| _version_ | 1797997432316887040 |
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| author | Dildar Bahar Genc |
| author_facet | Dildar Bahar Genc |
| author_sort | Dildar Bahar Genc |
| collection | DOAJ |
| description | Pediatric liver neoplasias are rare, comprising 1–4% of all solid childhood tumors. More than half of these masses are due to hepatoblastoma and hepatocellular carcinomas, making remaining various liver tumors much more rarer.Hepatic mesenchymal hamartoma (HMH) is a benign tumor observed in children less than five- year-old. It is located in the right lobe of the liver more often. It has a cystic structure, and AFP is generally normal or slightly elevated. The treatment is complete resection. In rare cases, HMH can transform into undifferentiated embryonal sarcoma.Focal nodular hyperplasia is a well-circumscribed benign lesion, which is usually discovered incidentally as well as with oral contraceptive use, postchemotherapy status, and hereditary hemorrhagic telengiectasia. Typical imaging finding is a central scar in the mass lesion, which might overlap with fibrolamellar hepatocellular carcinoma. Asymptomatic cases might be managed with an expectant approach by careful monitoring whereas symptomatic cases might be treated with surgery or ablation therapy.Infantile hemangioma is the most common benign hepatic tumor in infancy. Symptoms at admission may include abdominal distention due to hepatomegaly, congestive heart failure, feeding problems, anemia, thrombocytopenia and consumptive coagulopathy, jaundice. Many of these hemangiomas are discovered incidentally and are localized. In most cases, these lesions are small enough to be of no clinical significance. In severe cases, propranolol, corticosteroids, mTOR inhibitors or cytotoxic agents can be used for treatment.Undifferentiated embryonal sarcoma of the liver (UESL) is the third most common malignant liver tumor in children. The median age of diagnosis of UESL is 10,5 years, in comparison to that of liver rhabdomyosarcoma which is 3.6 years. UESL is an aggressive malignancy that should be treated with multimodal therapy. Most important prognostic factor is the completeness of the resection. The survival is reported to range between 70% and 90% in various case series.Rhabdomyosarcomas are observed in the biliary tract. The usual sign in admission is obstructive jaundice with or without abdominal mass. Treatment is multimodal therapy. With CWS protocols, the 5-year overall (OS) and event free survival (EFS) rates were 58% and 47%, respectively.Hepatic angiosarcoma is a rare but highly aggressive malignancy of endothelial cells, previously known as infantile hemangioendothelioma type 2. There is a girl predominance. Despite multiagent aggressive treatment approaches including liver transplantation, the overall prognosis is still poor. |
| first_indexed | 2024-04-11T10:32:53Z |
| format | Article |
| id | doaj.art-bc210319ac7e46348f465eb4009d3028 |
| institution | Directory Open Access Journal |
| issn | 2531-1379 |
| language | English |
| last_indexed | 2024-04-11T10:32:53Z |
| publishDate | 2022-10-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Hematology, Transfusion and Cell Therapy |
| spelling | doaj.art-bc210319ac7e46348f465eb4009d30282022-12-22T04:29:23ZengElsevierHematology, Transfusion and Cell Therapy2531-13792022-10-0144S11S12TREATMENT/MANAGEMENT OF OTHER HEPATIC TUMORSDildar Bahar Genc0University of Health Sciences, Sisli Hamidiye Etfal Research and Training Hospital, Department of Pediatric Hematology and OncologyPediatric liver neoplasias are rare, comprising 1–4% of all solid childhood tumors. More than half of these masses are due to hepatoblastoma and hepatocellular carcinomas, making remaining various liver tumors much more rarer.Hepatic mesenchymal hamartoma (HMH) is a benign tumor observed in children less than five- year-old. It is located in the right lobe of the liver more often. It has a cystic structure, and AFP is generally normal or slightly elevated. The treatment is complete resection. In rare cases, HMH can transform into undifferentiated embryonal sarcoma.Focal nodular hyperplasia is a well-circumscribed benign lesion, which is usually discovered incidentally as well as with oral contraceptive use, postchemotherapy status, and hereditary hemorrhagic telengiectasia. Typical imaging finding is a central scar in the mass lesion, which might overlap with fibrolamellar hepatocellular carcinoma. Asymptomatic cases might be managed with an expectant approach by careful monitoring whereas symptomatic cases might be treated with surgery or ablation therapy.Infantile hemangioma is the most common benign hepatic tumor in infancy. Symptoms at admission may include abdominal distention due to hepatomegaly, congestive heart failure, feeding problems, anemia, thrombocytopenia and consumptive coagulopathy, jaundice. Many of these hemangiomas are discovered incidentally and are localized. In most cases, these lesions are small enough to be of no clinical significance. In severe cases, propranolol, corticosteroids, mTOR inhibitors or cytotoxic agents can be used for treatment.Undifferentiated embryonal sarcoma of the liver (UESL) is the third most common malignant liver tumor in children. The median age of diagnosis of UESL is 10,5 years, in comparison to that of liver rhabdomyosarcoma which is 3.6 years. UESL is an aggressive malignancy that should be treated with multimodal therapy. Most important prognostic factor is the completeness of the resection. The survival is reported to range between 70% and 90% in various case series.Rhabdomyosarcomas are observed in the biliary tract. The usual sign in admission is obstructive jaundice with or without abdominal mass. Treatment is multimodal therapy. With CWS protocols, the 5-year overall (OS) and event free survival (EFS) rates were 58% and 47%, respectively.Hepatic angiosarcoma is a rare but highly aggressive malignancy of endothelial cells, previously known as infantile hemangioendothelioma type 2. There is a girl predominance. Despite multiagent aggressive treatment approaches including liver transplantation, the overall prognosis is still poor.http://www.sciencedirect.com/science/article/pii/S253113792201330X |
| spellingShingle | Dildar Bahar Genc TREATMENT/MANAGEMENT OF OTHER HEPATIC TUMORS Hematology, Transfusion and Cell Therapy |
| title | TREATMENT/MANAGEMENT OF OTHER HEPATIC TUMORS |
| title_full | TREATMENT/MANAGEMENT OF OTHER HEPATIC TUMORS |
| title_fullStr | TREATMENT/MANAGEMENT OF OTHER HEPATIC TUMORS |
| title_full_unstemmed | TREATMENT/MANAGEMENT OF OTHER HEPATIC TUMORS |
| title_short | TREATMENT/MANAGEMENT OF OTHER HEPATIC TUMORS |
| title_sort | treatment management of other hepatic tumors |
| url | http://www.sciencedirect.com/science/article/pii/S253113792201330X |
| work_keys_str_mv | AT dildarbahargenc treatmentmanagementofotherhepatictumors |