Primary hepatic malignant triton tumor mimicking hepatocellular carcinoma by demonstrating arterial-phase hypervascularity and subsequent washout on dynamic contrast-enhanced imaging: a case report and literature review
BackgroundMalignant Triton tumor (MTT) is a relatively rare subtype of malignant peripheral nerve sheath tumor (MPNST) characterized by rhabdomyosarcoma differentiation. There are no distinct features of MTT, and it is easy to misdiagnose preoperatively.Case presentationHere, we describe a rare case...
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Frontiers Media S.A.
2024-03-01
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| Series: | Frontiers in Medicine |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fmed.2024.1361690/full |
| _version_ | 1797279275945033728 |
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| author | Bo Zhou Canyang Zhan Yang Tian Zhenzhen Gao Sheng Yan |
| author_facet | Bo Zhou Canyang Zhan Yang Tian Zhenzhen Gao Sheng Yan |
| author_sort | Bo Zhou |
| collection | DOAJ |
| description | BackgroundMalignant Triton tumor (MTT) is a relatively rare subtype of malignant peripheral nerve sheath tumor (MPNST) characterized by rhabdomyosarcoma differentiation. There are no distinct features of MTT, and it is easy to misdiagnose preoperatively.Case presentationHere, we describe a rare case of primary hepatic MTT in a 56-year-old male who presented with nonspecific abdominal pain for 1 day. Magnetic resonance imaging and abdominal computed tomography revealed an extremely large mass located in the right liver with intratumoral hemorrhage, arterial-phase hypervascularity and subsequent washout on dynamic contrast-enhanced imaging and the possibility of intrahepatic metastasis. Tumor marker levels revealed only an elevated level of alpha-fetoprotein (AFP: 5304.0 ng/mL). Then, he received transcatheter arterial chemoembolization combined with lenvatinib and pembrolizumab, and he was diagnosed with hepatocellular carcinoma. After 3 months of neoadjuvant therapy, we resected the hepatic cancer and adherent diaphragmatic pleura. MTT was confirmed by postoperative pathology and immunohistochemistry.ConclusionDespite the preoperative diagnosis of hepatocellular carcinoma with a rising serum AFP level, typical CT and MRI findings, histopathology assessment showing MPNST with rhabdomyosarcoma differentiation confirms the diagnosis of primary hepatic MTT. |
| first_indexed | 2024-03-07T16:22:56Z |
| format | Article |
| id | doaj.art-b280a685569c40f5abbd76a49ce917f7 |
| institution | Directory Open Access Journal |
| issn | 2296-858X |
| language | English |
| last_indexed | 2024-03-07T16:22:56Z |
| publishDate | 2024-03-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Medicine |
| spelling | doaj.art-b280a685569c40f5abbd76a49ce917f72024-03-04T04:22:31ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2024-03-011110.3389/fmed.2024.13616901361690Primary hepatic malignant triton tumor mimicking hepatocellular carcinoma by demonstrating arterial-phase hypervascularity and subsequent washout on dynamic contrast-enhanced imaging: a case report and literature reviewBo Zhou0Canyang Zhan1Yang Tian2Zhenzhen Gao3Sheng Yan4Department of Hepatobiliary and Pancreatic Surgery, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, ChinaDepartment of Neonatology, Children’s Hospital, School of Medicine, Zhejiang University, Hangzhou, ChinaDepartment of Hepatobiliary and Pancreatic Surgery, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, ChinaDepartment of Hepatobiliary and Pancreatic Surgery, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, ChinaDepartment of Hepatobiliary and Pancreatic Surgery, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, ChinaBackgroundMalignant Triton tumor (MTT) is a relatively rare subtype of malignant peripheral nerve sheath tumor (MPNST) characterized by rhabdomyosarcoma differentiation. There are no distinct features of MTT, and it is easy to misdiagnose preoperatively.Case presentationHere, we describe a rare case of primary hepatic MTT in a 56-year-old male who presented with nonspecific abdominal pain for 1 day. Magnetic resonance imaging and abdominal computed tomography revealed an extremely large mass located in the right liver with intratumoral hemorrhage, arterial-phase hypervascularity and subsequent washout on dynamic contrast-enhanced imaging and the possibility of intrahepatic metastasis. Tumor marker levels revealed only an elevated level of alpha-fetoprotein (AFP: 5304.0 ng/mL). Then, he received transcatheter arterial chemoembolization combined with lenvatinib and pembrolizumab, and he was diagnosed with hepatocellular carcinoma. After 3 months of neoadjuvant therapy, we resected the hepatic cancer and adherent diaphragmatic pleura. MTT was confirmed by postoperative pathology and immunohistochemistry.ConclusionDespite the preoperative diagnosis of hepatocellular carcinoma with a rising serum AFP level, typical CT and MRI findings, histopathology assessment showing MPNST with rhabdomyosarcoma differentiation confirms the diagnosis of primary hepatic MTT.https://www.frontiersin.org/articles/10.3389/fmed.2024.1361690/fullhepatic malignant triton tumorhepatocellular carcinomacomplete surgical removalprognosiscase report |
| spellingShingle | Bo Zhou Canyang Zhan Yang Tian Zhenzhen Gao Sheng Yan Primary hepatic malignant triton tumor mimicking hepatocellular carcinoma by demonstrating arterial-phase hypervascularity and subsequent washout on dynamic contrast-enhanced imaging: a case report and literature review Frontiers in Medicine hepatic malignant triton tumor hepatocellular carcinoma complete surgical removal prognosis case report |
| title | Primary hepatic malignant triton tumor mimicking hepatocellular carcinoma by demonstrating arterial-phase hypervascularity and subsequent washout on dynamic contrast-enhanced imaging: a case report and literature review |
| title_full | Primary hepatic malignant triton tumor mimicking hepatocellular carcinoma by demonstrating arterial-phase hypervascularity and subsequent washout on dynamic contrast-enhanced imaging: a case report and literature review |
| title_fullStr | Primary hepatic malignant triton tumor mimicking hepatocellular carcinoma by demonstrating arterial-phase hypervascularity and subsequent washout on dynamic contrast-enhanced imaging: a case report and literature review |
| title_full_unstemmed | Primary hepatic malignant triton tumor mimicking hepatocellular carcinoma by demonstrating arterial-phase hypervascularity and subsequent washout on dynamic contrast-enhanced imaging: a case report and literature review |
| title_short | Primary hepatic malignant triton tumor mimicking hepatocellular carcinoma by demonstrating arterial-phase hypervascularity and subsequent washout on dynamic contrast-enhanced imaging: a case report and literature review |
| title_sort | primary hepatic malignant triton tumor mimicking hepatocellular carcinoma by demonstrating arterial phase hypervascularity and subsequent washout on dynamic contrast enhanced imaging a case report and literature review |
| topic | hepatic malignant triton tumor hepatocellular carcinoma complete surgical removal prognosis case report |
| url | https://www.frontiersin.org/articles/10.3389/fmed.2024.1361690/full |
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