Primary leiomyosarcoma of the inferior vena cava in a pediatric case: a case report and literature review

Abstract Background Leiomyosarcoma is classified as a soft tissue sarcoma. In adults, leiomyosarcoma is the most common malignancy affecting the vascular system; however, vascular leiomyosarcoma in children is extremely rare as most pediatric soft tissue tumors are rhabdomyosarcomas. The survival ra...

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Main Authors: Kazuki Yoshizawa, Yasunari Ohno, Takashi Kurata, Yuki Takagi, Tomoko Kasai, Momoko Takizawa, Yuji Soejima
Format: Article
Language:English
Published: SpringerOpen 2023-04-01
Series:Surgical Case Reports
Subjects:
Online Access:https://doi.org/10.1186/s40792-023-01630-x
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author Kazuki Yoshizawa
Yasunari Ohno
Takashi Kurata
Yuki Takagi
Tomoko Kasai
Momoko Takizawa
Yuji Soejima
author_facet Kazuki Yoshizawa
Yasunari Ohno
Takashi Kurata
Yuki Takagi
Tomoko Kasai
Momoko Takizawa
Yuji Soejima
author_sort Kazuki Yoshizawa
collection DOAJ
description Abstract Background Leiomyosarcoma is classified as a soft tissue sarcoma. In adults, leiomyosarcoma is the most common malignancy affecting the vascular system; however, vascular leiomyosarcoma in children is extremely rare as most pediatric soft tissue tumors are rhabdomyosarcomas. The survival rate is very low, and incomplete resection is a poor prognostic factor. There is also a high rate of distant recurrence, with the lungs and liver being the most common sites of metastasis. There is no established effective chemotherapy, and complete surgical resection is the only potentially curative treatment for leiomyosarcoma. Case presentation A 15-year-old female patient with no significant medical history presented with severe upper abdominal pain and was admitted. Abdominal contrast-enhanced computed tomography and magnetic resonance imaging showed a large retroperitoneal tumor protruding into the lumen of the inferior vena cava behind the liver and multiple small nodules, and metastasis to the liver was suspected. The tumor was 6 × 4 × 5 cm in diameter, located just behind the hepatic hilar structures, and was suspected to infiltrate into the right portal vein. The tumor was diagnosed as a leiomyosarcoma through an open tumor biopsy. As the multiple liver metastases were located only in the right lobe of the liver on imaging, we performed tumor resection with right hepatectomy and replacement of the inferior vena cava (IVC). The postoperative course was uneventful; however, on postoperative day 51, distant metastatic recurrences were found in the remaining liver and right lung. The patient was immediately started on chemotherapy and trabectedin proved to be the most effective drug in the treatment regimen; however, severe side effects, such as hepatotoxicity, prevented timely administration, and the patient passed away 19 months after surgery. Conclusions IVC resection and reconstruction combined with right hepatectomy were able to be safely performed even in a pediatric case. To improve the prognosis of leiomyosarcoma with multiple metastases, an effective treatment strategy combining surgical treatment and chemotherapy, including molecularly targeted drugs, should be established as early as possible.
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spelling doaj.art-848be25a66364e31821221feea0ca3d12023-04-09T11:25:34ZengSpringerOpenSurgical Case Reports2198-77932023-04-01911810.1186/s40792-023-01630-xPrimary leiomyosarcoma of the inferior vena cava in a pediatric case: a case report and literature reviewKazuki Yoshizawa0Yasunari Ohno1Takashi Kurata2Yuki Takagi3Tomoko Kasai4Momoko Takizawa5Yuji Soejima6Division of Gastroenterological, Hepato-Biliary-Pancreatic, Transplantation and Pediatric Surgery, Department of Surgery, Shinshu University School of MedicineDivision of Gastroenterological, Hepato-Biliary-Pancreatic, Transplantation and Pediatric Surgery, Department of Surgery, Shinshu University School of MedicineDepartment of Hematology/Oncology, Nagano Children’s HospitalDivision of Cardiovascular Surgery, Department of Surgery, Shinshu University School of MedicineDepartment of Surgery, Nagano Children’s HospitalDepartment of Laboratory Medicine, Shinshu University HospitalDivision of Gastroenterological, Hepato-Biliary-Pancreatic, Transplantation and Pediatric Surgery, Department of Surgery, Shinshu University School of MedicineAbstract Background Leiomyosarcoma is classified as a soft tissue sarcoma. In adults, leiomyosarcoma is the most common malignancy affecting the vascular system; however, vascular leiomyosarcoma in children is extremely rare as most pediatric soft tissue tumors are rhabdomyosarcomas. The survival rate is very low, and incomplete resection is a poor prognostic factor. There is also a high rate of distant recurrence, with the lungs and liver being the most common sites of metastasis. There is no established effective chemotherapy, and complete surgical resection is the only potentially curative treatment for leiomyosarcoma. Case presentation A 15-year-old female patient with no significant medical history presented with severe upper abdominal pain and was admitted. Abdominal contrast-enhanced computed tomography and magnetic resonance imaging showed a large retroperitoneal tumor protruding into the lumen of the inferior vena cava behind the liver and multiple small nodules, and metastasis to the liver was suspected. The tumor was 6 × 4 × 5 cm in diameter, located just behind the hepatic hilar structures, and was suspected to infiltrate into the right portal vein. The tumor was diagnosed as a leiomyosarcoma through an open tumor biopsy. As the multiple liver metastases were located only in the right lobe of the liver on imaging, we performed tumor resection with right hepatectomy and replacement of the inferior vena cava (IVC). The postoperative course was uneventful; however, on postoperative day 51, distant metastatic recurrences were found in the remaining liver and right lung. The patient was immediately started on chemotherapy and trabectedin proved to be the most effective drug in the treatment regimen; however, severe side effects, such as hepatotoxicity, prevented timely administration, and the patient passed away 19 months after surgery. Conclusions IVC resection and reconstruction combined with right hepatectomy were able to be safely performed even in a pediatric case. To improve the prognosis of leiomyosarcoma with multiple metastases, an effective treatment strategy combining surgical treatment and chemotherapy, including molecularly targeted drugs, should be established as early as possible.https://doi.org/10.1186/s40792-023-01630-xLeiomyosarcomaSoft tissue tumorChildhoodInferior vena cavaMetastasisHepatectomy
spellingShingle Kazuki Yoshizawa
Yasunari Ohno
Takashi Kurata
Yuki Takagi
Tomoko Kasai
Momoko Takizawa
Yuji Soejima
Primary leiomyosarcoma of the inferior vena cava in a pediatric case: a case report and literature review
Surgical Case Reports
Leiomyosarcoma
Soft tissue tumor
Childhood
Inferior vena cava
Metastasis
Hepatectomy
title Primary leiomyosarcoma of the inferior vena cava in a pediatric case: a case report and literature review
title_full Primary leiomyosarcoma of the inferior vena cava in a pediatric case: a case report and literature review
title_fullStr Primary leiomyosarcoma of the inferior vena cava in a pediatric case: a case report and literature review
title_full_unstemmed Primary leiomyosarcoma of the inferior vena cava in a pediatric case: a case report and literature review
title_short Primary leiomyosarcoma of the inferior vena cava in a pediatric case: a case report and literature review
title_sort primary leiomyosarcoma of the inferior vena cava in a pediatric case a case report and literature review
topic Leiomyosarcoma
Soft tissue tumor
Childhood
Inferior vena cava
Metastasis
Hepatectomy
url https://doi.org/10.1186/s40792-023-01630-x
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