A Case of Wilms Tumor with a Tumor Thrombus in a Boy with WAGR Syndrome
Intravascular extension of Wilms tumor (WT) can occur in approximately 4-10% of patients. In general, it does not cause any clinical problems because most of these tumors are small. Although there is no standard treatment currently, preoperative chemotherapy and delayed nephrectomy is generally reco...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
| Published: |
The Korean Society of Pediatric Hematology-Oncology
2020-10-01
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| Series: | Clinical Pediatric Hematology-Oncology |
| Subjects: | |
| Online Access: | https://doi.org/10.15264/cpho.2020.27.2.134 |
| _version_ | 1818266823783612416 |
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| author | Soojung Lee Hyo Jin Kim In-sang Jeon |
| author_facet | Soojung Lee Hyo Jin Kim In-sang Jeon |
| author_sort | Soojung Lee |
| collection | DOAJ |
| description | Intravascular extension of Wilms tumor (WT) can occur in approximately 4-10% of patients. In general, it does not cause any clinical problems because most of these tumors are small. Although there is no standard treatment currently, preoperative chemotherapy and delayed nephrectomy is generally recommended for children with WT accompanied by tumor thrombus. We report a rare case of WT, aniridia, genitourinary anomalies, and mental retardation (WAGR) syndrome in a boy who also had a huge inferior vena cava thrombus, 7 cm length. The prevalence of bilateral WT and tumor thrombus in WAGR has not been identified. The patient was successfully treated with neoadjuvant chemotherapy to decrease the size of the tumor thrombus with WT and delayed nephrectomy following chemotherapy without any invasive intervention and did not show complications. |
| first_indexed | 2024-12-12T20:12:50Z |
| format | Article |
| id | doaj.art-60e5aa60437b4a9892aa50ecb464df23 |
| institution | Directory Open Access Journal |
| issn | 2233-5250 |
| language | English |
| last_indexed | 2024-12-12T20:12:50Z |
| publishDate | 2020-10-01 |
| publisher | The Korean Society of Pediatric Hematology-Oncology |
| record_format | Article |
| series | Clinical Pediatric Hematology-Oncology |
| spelling | doaj.art-60e5aa60437b4a9892aa50ecb464df232022-12-22T00:13:27ZengThe Korean Society of Pediatric Hematology-OncologyClinical Pediatric Hematology-Oncology2233-52502020-10-0127213413710.15264/cpho.2020.27.2.134cpho.2020.27.2.134A Case of Wilms Tumor with a Tumor Thrombus in a Boy with WAGR SyndromeSoojung Lee0Hyo Jin Kim1In-sang Jeon2Department of Pediatrics, Gil Medical Center, Gachon University College of Medicine, Incheon, KoreaDepartment of Pediatrics, Gil Medical Center, Gachon University College of Medicine, Incheon, KoreaDepartment of Pediatrics, Gil Medical Center, Gachon University College of Medicine, Incheon, KoreaIntravascular extension of Wilms tumor (WT) can occur in approximately 4-10% of patients. In general, it does not cause any clinical problems because most of these tumors are small. Although there is no standard treatment currently, preoperative chemotherapy and delayed nephrectomy is generally recommended for children with WT accompanied by tumor thrombus. We report a rare case of WT, aniridia, genitourinary anomalies, and mental retardation (WAGR) syndrome in a boy who also had a huge inferior vena cava thrombus, 7 cm length. The prevalence of bilateral WT and tumor thrombus in WAGR has not been identified. The patient was successfully treated with neoadjuvant chemotherapy to decrease the size of the tumor thrombus with WT and delayed nephrectomy following chemotherapy without any invasive intervention and did not show complications.https://doi.org/10.15264/cpho.2020.27.2.134wagr syndromewilms tumortumor thrombusinferior vena cava |
| spellingShingle | Soojung Lee Hyo Jin Kim In-sang Jeon A Case of Wilms Tumor with a Tumor Thrombus in a Boy with WAGR Syndrome Clinical Pediatric Hematology-Oncology wagr syndrome wilms tumor tumor thrombus inferior vena cava |
| title | A Case of Wilms Tumor with a Tumor Thrombus in a Boy with WAGR Syndrome |
| title_full | A Case of Wilms Tumor with a Tumor Thrombus in a Boy with WAGR Syndrome |
| title_fullStr | A Case of Wilms Tumor with a Tumor Thrombus in a Boy with WAGR Syndrome |
| title_full_unstemmed | A Case of Wilms Tumor with a Tumor Thrombus in a Boy with WAGR Syndrome |
| title_short | A Case of Wilms Tumor with a Tumor Thrombus in a Boy with WAGR Syndrome |
| title_sort | case of wilms tumor with a tumor thrombus in a boy with wagr syndrome |
| topic | wagr syndrome wilms tumor tumor thrombus inferior vena cava |
| url | https://doi.org/10.15264/cpho.2020.27.2.134 |
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