Teratoid Wilms Tumor and Classical Wilms Tumor: A Retrospective Ten-year Single-center Study

Abstract BackgroundWilms tumor (WT) is the most common renal tumor in the pediatric population. Nevertheless, teratoid Wilms tumor (TWT) is a rare category of WT characterized by different tissue types, and fewer than 40 cases have been reported in the literature to date. Methods A total of 67 WT patients admitted in our hospital from 2010 to 2020, including 5 patients with TWT, were enrolled in this study. The clinical features, preoperative and postoperative chemotherapeutic regimens, intraoperative findings, histopathological information, as well as prognoses of the WT patients were evaluated. ResultsTWT cases were matched to classical WT cases at 1:3 for the comparison of various variables. 7.46% (5/67) of WT patients were diagnosed with TWT. the tumor volume was dramatically larger for patients with TWT than for those with classical WT (203.30 ± 109.89 cm3 vs. 104.30 ± 66.97 cm3). However, the tumor weight of the two groups was similar (471.00 ± 80.65 vs. 432.67 ± 109.25). ConclusionsOur data with the first reported Chinese children with TWT, preliminarily demonstrated that chemotherapy combined with surgery might be the appropriate treatment option for patients with WT, and prognoses of these patients differed sharply due to various stages. The density of TWT might be lower than that of classical WT. Future studies with more basic research to understand the biologic nature and theranostic markers of teratoid WT, may help us optimize the patient-tailored therapy of this rare type of WT.

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Familial synchronous bilateral teratoid Wilms tumor with elevated alpha-fetoprotein level

Tumori Journal ◽

10.1177/030089161209800634 ◽

2012 ◽

Vol 98 (6) ◽

pp. e179-e182 ◽

Cited By ~ 4

Author(s):

Arzu Okur ◽

Faruk Guclu Pinarli ◽

Ceyda Karadeniz ◽

Aylar Poyraz ◽

Kibriya Fidan ◽

...

Keyword(s):

Tumor Volume ◽

Age Of Onset ◽

Wilms Tumor ◽

Alpha Fetoprotein ◽

Rare Entity ◽

Rare Type ◽

Specific Tumor ◽

Serum Alpha Fetoprotein ◽

First Time ◽

Specific Tumor Marker

Familial Wilms tumor is a rare entity that accounts for only 1–2% of all Wilms tumor cases, with an earlier age of onset and an increased frequency of bilateral tumors. Teratoid Wilms tumor is a variant of nephroblastoma with a predominance of heterologous tissues comprising more than 50% of the tumor volume. Wilms tumor does not usually secrete any specific tumor marker and all teratoid Wilms tumor cases previously reported were sporadic non-secreting neoplasms. Here we describe an infant with familial synchronous bilateral teratoid Wilms tumor whose serum alpha-fetoprotein level was elevated. To our knowledge, this extremely rare type of case is reported for the first time in the literature.

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Wilms Tumor (Nephroblastoma), Version 2.2021, NCCN Clinical Practice Guidelines in Oncology

Journal of the National Comprehensive Cancer Network ◽

10.6004/jnccn.2021.0037 ◽

2021 ◽

Vol 19 (8) ◽

pp. 945-977

Author(s):

Frank Balis ◽

Daniel M. Green ◽

Clarke Anderson ◽

Shelly Cook ◽

Jasreman Dhillon ◽

...

Keyword(s):

Radiation Therapy ◽

Renal Tumor ◽

Wilms Tumor ◽

Renal Tumors ◽

Nccn Guidelines ◽

Favorable Histology ◽

Tumor Focus ◽

Appropriate Treatment ◽

Pediatric Oncologist

The NCCN Guidelines for Wilms Tumor focus on the screening, diagnosis, staging, treatment, and management of Wilms tumor (WT, also known as nephroblastoma). WT is the most common primary renal tumor in children. Five-year survival is more than 90% for children with all stages of favorable histology WT who receive appropriate treatment. All patients with WT should be managed by a multidisciplinary team with experience in managing renal tumors; consulting a pediatric oncologist is strongly encouraged. Treatment of WT includes surgery, neoadjuvant or adjuvant chemotherapy, and radiation therapy (RT) if needed. Careful use of available therapies is necessary to maximize cure and minimize long-term toxicities. This article discusses the NCCN Guidelines recommendations for favorable histology WT.

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Is it always Wilms’ tumor? Localized cystic disease of the kidney in an infant. An extremely rare case report and review of the literature

Pediatric Reports ◽

10.4081/pr.2020.8483 ◽

2020 ◽

Vol 12 (1) ◽

Author(s):

Christos Kaselas ◽

Charikleia Demiri ◽

Vasilios Mouravas ◽

Eleni Koutra ◽

Kleanthis Anastasiadis ◽

...

Keyword(s):

Case Report ◽

Rare Case ◽

Wilms Tumor ◽

Pediatric Population ◽

Benign Disease ◽

Cystic Disease ◽

Proper Treatment ◽

Review Of The Literature ◽

Rare Case Report ◽

Renal Malignancies

Localized Cystic Disease of the Kidney (LCDK) is an extremely rare benign disease in pediatric population. Although its management is conservative and generally requires no treatment, the unfamiliarity with the disease can expose such patients to misdiagnosis as renal malignancies or uncertainty for proper treatment. We report such a case in an infant and review the current literature.

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Is partial nephrectomy appropriate treatment for unilateral Wilms' tumor?

Journal of Pediatric Surgery ◽

10.1016/s0022-3468(98)90425-0 ◽

1998 ◽

Vol 33 (2) ◽

pp. 165-170 ◽

Cited By ~ 58

Author(s):

Catharina G.M Moorman-Voestermans ◽

Daniel C Aronson ◽

Chris R Staalman ◽

Jan F.M Delemarre ◽

Jan de Kraker

Keyword(s):

Partial Nephrectomy ◽

Wilms Tumor ◽

Appropriate Treatment

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Resolution of congenital nasolacrimal duct obstruction with conservative management

Journal of Chitwan Medical College ◽

10.3126/jcmc.v6i1.16573 ◽

2017 ◽

Vol 6 (1) ◽

pp. 12-15

Author(s):

J B Shrestha

Keyword(s):

Congenital Anomaly ◽

Conservative Management ◽

Pediatric Population ◽

Clinical Signs ◽

Nasolacrimal Duct ◽

Nasolacrimal Duct Obstruction ◽

Congenital Nasolacrimal Duct Obstruction ◽

Duct Obstruction ◽

Topical Antibiotics ◽

Appropriate Treatment

Congenital nasolacrimal duct obstruction is the commonly encountered congenital anomaly in pediatric population occurring in as many as 30% of new borns. Conservative management of such condition with topical antibiotics and properly performed massage of the nasolacrimal sac is appropriate treatment during the first few months of age. The purpose of this study was to determine the rate of resolution of nasolacrimal duct obstruction with conservative management in infants up to 10 months of age. A total of 181 infants of age up to 10 months old with the diagnosis of Congenital nasolacrimal duct obstruction were advised nasolacrimal duct massage with or without the prescription of topical antibiotics. Resolution of nasolacrimal duct obstruction was assessed at 3 month and 6 month and was defined as the absence of all clinical signs of nasolacrimal duct obstruction. At the 6-month examination, 163 eyes (83%) of 181 children showed resolution with conservative management. The overall success rate of Congenital nasolacrimal duct obstruction with conservative management was high and this form of management can be considered as one of the best options in infants.

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Postchemotherapy tumor volume as a prognostic indicator in Wilms tumor: A single‐center experience from South India

Pediatric Blood & Cancer ◽

10.1002/pbc.29454 ◽

2021 ◽

Author(s):

Leenu Lizbeth Joseph ◽

Deepthi Boddu ◽

Hema Nalapullu Srinivasan ◽

Soumya Susan Regi ◽

Belavendra Antonisamy ◽

...

Keyword(s):

South India ◽

Tumor Volume ◽

Wilms Tumor ◽

Prognostic Indicator ◽

Single Center ◽

Single Center Experience

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Papillary tumor of the pineal region in a 15-month-old boy

Journal of Neurosurgery Pediatrics ◽

10.3171/2011.2.peds10434 ◽

2011 ◽

Vol 7 (5) ◽

pp. 534-538 ◽

Cited By ~ 16

Author(s):

Jessica Li ◽

Pablo F. Recinos ◽

Brent A. Orr ◽

Peter C. Burger ◽

George I. Jallo ◽

...

Keyword(s):

Pediatric Population ◽

Proton Beam Therapy ◽

Pineal Region ◽

Biological Behavior ◽

Imaging Diagnosis ◽

Papillary Tumor ◽

Distinct Entity ◽

Pineal Region Tumor ◽

Appropriate Treatment

The papillary tumor of the pineal region (PTPR) is a distinct entity that is particularly rare in the pediatric population. The authors document the youngest reported patient with this clinicopathological entity to date. A case of PTPR in a 15-month-old boy is described. Initially thought to be a tectal glioma, the tumor was later identified as a pineal region tumor after demonstrating growth on routine imaging. Diagnosis of PTPR was established by histopathological evaluation of biopsy samples, which revealed papillary, cystic, and solid tumor components. The patient's postoperative course was complicated by tumor growth despite several debulking procedures and chemotherapy, as well as persistent hydrocephalus requiring 2 endoscopic third ventriculostomies and eventual ventriculoperitoneal shunt placement. After a 15-month follow-up period, the patient has received proton-beam therapy and has a stable tumor size. The PTPR is a recently described tumor of the CNS that must be included in the differential diagnosis of pineal region masses. The biological behavior, prognosis, and appropriate treatment of PTPR have yet to be fully defined.

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The Effects of Hypertension on Cognitive Function in Children and Adolescents

International Journal of Pediatrics ◽

10.1155/2012/891094 ◽

2012 ◽

Vol 2012 ◽

pp. 1-5 ◽

Cited By ~ 7

Author(s):

Stephen D. Cha ◽

Hiren P. Patel ◽

David S. Hains ◽

John D. Mahan

Keyword(s):

Children And Adolescents ◽

Pediatric Population ◽

Organ Damage ◽

Future Research ◽

Cognitive Effects ◽

Long Term Outcomes ◽

Future Studies ◽

End Organ Damage ◽

Anxiety Depression

Hypertension (HTN) is found in about 3-4% of the pediatric population with long-term risks of end organ damage if untreated or poorly controlled. Although children with HTN are being more frequently screened for end organ damage (i.e., LVH), the cognitive effects of HTN and methods to screen for cognitive dysfunction have not been extensively explored. In recent years, there have been a small number of studies that have provided important insights that can guide future research in this area. These studies show that HTN can be associated with headaches, restlessness, sleep disturbance, anxiety, depression, decreased attention, and also poor executive functioning. By increasing the utilization of cognitive tests in hypertensive children and adolescents, important cognitive defects secondary to HTN may be detected. More research is needed in the area, and the results of future studies could have far reaching implications for long-term outcomes in hypertensive children and adolescents.

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