Twenty children with non-Wilms renal tumors from a reference center in Central Anatolia, Turkey

Ünal, Ekrem; Yılmaz, Ebru; Özcan, Alper; Karakükçü, Musa; Turan, Cüneyt; Akgün, Hülya; Öztürk, Figen; Çoşkun, Abdulhakim; Özdemır, Mehmet Akif; Patıroğlu, Türkan

doi:10.3906/sag-1902-106

Cited by 5 publications

(7 citation statements)

References 30 publications

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“…NWRTs that occur in children include CMN, CCSK, RCC, MRTK, lymphoma, angiomyolipoma, and other rare entities [2]. Different studies have reported that the proportion of NWRTs in renal tumors varies from 13.6-18.7% [3][4]. Preoperative imaging, which can be performed to determine the anatomic location and extent of the mass, is often su cient to presume a diagnosis [12].…”

Section: Discussionmentioning

confidence: 99%

Non-wilms' Renal Tumors in Children: Experience With 139 Cases Treated at a Single Center

Fang

Song

Sun

et al. 2022

Preprint

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Introduction: To determine the clinical characteristics, management and prognosis of children with Pediatric non-Wilms renal tumors (NWRTs).Methods: Medical records of all patients (n = 139) treated for NWRTs over a 12-year period (2008.01–2019.10) at a single center were reviewed retrospectively.Results: The histopathological groups of NWRTs included malignant rhabdoid tumor of the kidney (MRTK) (n: 30, 21.6%), renal cell cancer (RCC) (n: 26,18.7%), clear cell sarcoma of the kidney (CCSK) (n: 24,17.3%), congenital mesoblastic nephroma (CMN) (n: 21,15.1%), cystic nephroma (CN) (n: 16,11.5%), metanephric tumors (n: 12, 8.6%), renal angiomyoliporma (RAML) (n: 3, 2.2%), renal primitive neuroectodermal tumor (rPNET) (n: 2, 1.4%), renal hemangioma (n: 2, 1.4%), inflammatory myofibroblastic tumor (IMT) (n: 2, 1.4%), ossifying renal tumor of infancy (ORTI) (n: 1, 0.7%). The distribution of all malignant NWRTs, including MRTK, CCSK, RCC and PNET, according to stage was as follows: stages I (n=26), II (n=16), III (n=29), and IV (n=11). The summary table shows the treatment offered to children with NWRTs. A total of 123 children were followed up for an average of 42 months. Sixteen children were lost to follow-up. Tumor-free survival was observed in 94 children. One patient who suffered from RCC is currently receiving targeted therapy and survives with the tumor. Twenty-eight children (22.8%) died.Discussion: Pediatric NWRTs comprise 19.1% of all renal tumors in our single center. The mainstay treatment of malignant NWRTs, including MRTK, CCSK, RCC and PNET, is comprehensive treatment. The mainstay treatment of benign NWRTs, including RAML, CN, ORTI, CMN, metanephric tumors, and renal hemangioma, is surgical resection alone.

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Section: Discussionmentioning

confidence: 99%

Non-wilms' Renal Tumors in Children: Experience With 139 Cases Treated at a Single Center

Fang

Song

Sun

et al. 2022

Preprint

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“…NWRTs that occur in children include CMN, CCSK, RCC, MRTK, lymphoma, angiomyolipoma, and other rare entities [ 2 ]. Different studies have reported that the proportion of NWRTs in renal tumors varies from 13.6 to 18.7% [ 3 , 4 ]. In our single center, pediatric NWRTs comprise 19.1% of all renal tumors.…”

Section: Discussionmentioning

confidence: 99%

Non-Wilms' renal tumors in children: experience with 139 cases treated at a single center

et al. 2022

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Background Pediatric non-Wilms renal tumors (NWRTs), which comprise a small proportion of renal tumors, are a heterogeneous group of neoplasms with variable malignant potential, mortality, and response to treatment. We performed this study to determine the clinical characteristics, management and prognosis of children with Pediatric NWRTs. Methods Medical records of all patients (n = 139) treated for NWRTs over a 12-year period (2008.01–2019.10) at a single center were reviewed retrospectively. Results The histopathological groups of NWRTs included malignant rhabdoid tumor of the kidney (MRTK) (n: 30, 21.6%), renal cell cancer (RCC) (n: 26,18.7%), clear cell sarcoma of the kidney (CCSK) (n: 24,17.3%), congenital mesoblastic nephroma (CMN) (n: 21,15.1%), cystic nephroma (CN) (n: 16,11.5%), metanephric tumors (n: 12, 8.6%), renal angiomyoliporma (RAML) (n: 3, 2.2%), renal primitive neuroectodermal tumor (n: 2, 1.4%), renal hemangioma (n: 2, 1.4%), inflammatory myofibroblastic tumor (n: 2, 1.4%), ossifying renal tumor of infancy (ORTI) (n: 1, 0.7%). The distribution of all malignant NWRTs, including MRTK, CCSK, RCC and PNET, according to stage was as follows: stages I (n = 26), II (n = 16), III (n = 29), and IV (n = 11). The summary table shows the treatment offered to children with NWRTs. A total of 123 children were followed up for an average of 42 months. Sixteen children were lost to follow-up. Tumor-free survival was observed in 94 children. One patient who suffered from RCC is currently receiving targeted therapy and survives with the tumor. Twenty-eight children (22.8%) died. Conclusions Pediatric NWRTs comprise 19.1% of all renal tumors in our single center. Most NWRTs can readily be distinguished using a range of immunohistochemical markers. Molecular genetic profiling has allowed much progress in the understanding of this group of tumors, making diagnosis and classification less difficult. The mainstay treatment of malignant NWRTs, including MRTK, CCSK, RCC and PNET, is comprehensive treatment. The mainstay treatment of benign NWRTs, including RAML, CN, ORTI, CMN, metanephric tumors, and renal hemangioma, is surgical resection alone and when the tumor diameter is smaller than 7 cm and the tumor locates in one pole, NSS can be performed.

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“…surveyed 20 children with nonWilm's tumors in Turkey and reported the case of a 2.5-month-old girl infant with bilateral renal PNET. [ 3 ]…”

Section: Discussionmentioning

confidence: 99%

“…Major differentials to be considered for small round cell tumors from kidney, all with similar morphological features, include malignant lymphoma, rhabdomyosarcoma, renal neuroblastoma, Wilms' tumor, small-cell osteosarcoma, desmoplastic small-cell tumor, synovial sarcoma, small-cell neuroendocrine carcinoma, and PNET. [ 3 ]…”

Section: Discussionmentioning

confidence: 99%

Renal Primitive Neuroectodermal Tumor Mimicking Wilms' Tumor in a Young Boy

Jayaraman

Babu

et al. 2022

Journal of Indian Association of Pediatric Surgeons

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Twenty children with non-Wilms renal tumors from a reference center in Central Anatolia, Turkey

Cited by 5 publications

References 30 publications

Non-wilms' Renal Tumors in Children: Experience With 139 Cases Treated at a Single Center

Non-wilms' Renal Tumors in Children: Experience With 139 Cases Treated at a Single Center

Non-Wilms' renal tumors in children: experience with 139 cases treated at a single center

Renal Primitive Neuroectodermal Tumor Mimicking Wilms' Tumor in a Young Boy

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