MiT family translocation renal cell carcinoma with retroperitoneal metastasis in childhood: a case report
RCC accounts for only 0.1%–0.3% of all kidney tumors and 2%–6% of malignant kidney tumors in children. Accounting for approximately one-third of the total number of cases in children and adolescents with RCC, Xp11.2 tRCC is the most common subtype of the MiT family translocation renal cell carcinoma...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2023-07-01
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| Series: | Frontiers in Pediatrics |
| Subjects: | |
| Online Access: | https://www.frontiersin.org/articles/10.3389/fped.2023.1141223/full |
| _version_ | 1797741687098834944 |
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| author | Kaihang Yang Yuhao Ma Shuyang Dai Rui Dong |
| author_facet | Kaihang Yang Yuhao Ma Shuyang Dai Rui Dong |
| author_sort | Kaihang Yang |
| collection | DOAJ |
| description | RCC accounts for only 0.1%–0.3% of all kidney tumors and 2%–6% of malignant kidney tumors in children. Accounting for approximately one-third of the total number of cases in children and adolescents with RCC, Xp11.2 tRCC is the most common subtype of the MiT family translocation renal cell carcinoma, which is a group of rare childhood and adult tumors, characterized by recurrent gene rearrangements of TFE3. Here we report a rare case of a 6-year-old male patient with MiT family translocation renal cell carcinoma (MiTF tRCC) where the patient developed retroperitoneal metastasis. The patient underwent partial nephrectomy (PN), radical nephrectomy (RN), abdominal lymph node resection, and intestinal adhesion lysis. Microscopically, we detected focal and nest clump-shaped clusters of tumor cells whose cytoplasm was bright and clear. Immunohistochemistry (IHC) showed tumor cells diffusely expressed TFE3, and fluorescence in situ hybridization (FISH) demonstrated disruption of the TFE3 locus, confirming the diagnosis of Xp11.2 tRCC, the most common subtype of MiTF tRCC. Eventually, the patient obtained a good therapeutic result. This case can provide a good reference and guidance for pediatric urologists and oncologists to recognize and diagnose rare renal cell carcinoma in children. |
| first_indexed | 2024-03-12T14:30:19Z |
| format | Article |
| id | doaj.art-6e095ff89da346938fd467e9335f0f48 |
| institution | Directory Open Access Journal |
| issn | 2296-2360 |
| language | English |
| last_indexed | 2024-03-12T14:30:19Z |
| publishDate | 2023-07-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Pediatrics |
| spelling | doaj.art-6e095ff89da346938fd467e9335f0f482023-08-17T14:14:20ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602023-07-011110.3389/fped.2023.11412231141223MiT family translocation renal cell carcinoma with retroperitoneal metastasis in childhood: a case reportKaihang YangYuhao MaShuyang DaiRui DongRCC accounts for only 0.1%–0.3% of all kidney tumors and 2%–6% of malignant kidney tumors in children. Accounting for approximately one-third of the total number of cases in children and adolescents with RCC, Xp11.2 tRCC is the most common subtype of the MiT family translocation renal cell carcinoma, which is a group of rare childhood and adult tumors, characterized by recurrent gene rearrangements of TFE3. Here we report a rare case of a 6-year-old male patient with MiT family translocation renal cell carcinoma (MiTF tRCC) where the patient developed retroperitoneal metastasis. The patient underwent partial nephrectomy (PN), radical nephrectomy (RN), abdominal lymph node resection, and intestinal adhesion lysis. Microscopically, we detected focal and nest clump-shaped clusters of tumor cells whose cytoplasm was bright and clear. Immunohistochemistry (IHC) showed tumor cells diffusely expressed TFE3, and fluorescence in situ hybridization (FISH) demonstrated disruption of the TFE3 locus, confirming the diagnosis of Xp11.2 tRCC, the most common subtype of MiTF tRCC. Eventually, the patient obtained a good therapeutic result. This case can provide a good reference and guidance for pediatric urologists and oncologists to recognize and diagnose rare renal cell carcinoma in children.https://www.frontiersin.org/articles/10.3389/fped.2023.1141223/fullMiT family translocation renal cell carcinomaXp11.2 translocationTFE3partial nephrectomy (PN)retroperitoneal metastasis |
| spellingShingle | Kaihang Yang Yuhao Ma Shuyang Dai Rui Dong MiT family translocation renal cell carcinoma with retroperitoneal metastasis in childhood: a case report Frontiers in Pediatrics MiT family translocation renal cell carcinoma Xp11.2 translocation TFE3 partial nephrectomy (PN) retroperitoneal metastasis |
| title | MiT family translocation renal cell carcinoma with retroperitoneal metastasis in childhood: a case report |
| title_full | MiT family translocation renal cell carcinoma with retroperitoneal metastasis in childhood: a case report |
| title_fullStr | MiT family translocation renal cell carcinoma with retroperitoneal metastasis in childhood: a case report |
| title_full_unstemmed | MiT family translocation renal cell carcinoma with retroperitoneal metastasis in childhood: a case report |
| title_short | MiT family translocation renal cell carcinoma with retroperitoneal metastasis in childhood: a case report |
| title_sort | mit family translocation renal cell carcinoma with retroperitoneal metastasis in childhood a case report |
| topic | MiT family translocation renal cell carcinoma Xp11.2 translocation TFE3 partial nephrectomy (PN) retroperitoneal metastasis |
| url | https://www.frontiersin.org/articles/10.3389/fped.2023.1141223/full |
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