Extrarenal Wilms' Tumour of the Ovary

Albiroty, Khalil A.; Sabahi, Amal Al; Shabibi, Saud Al; Al'Ajmi, Zakyia I.; Hinai, Karima Al; Al-Mashaikhi, Nawal

doi:10.18295/squmj.8.2021.117

Cited by 2 publications

(2 citation statements)

References 8 publications

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“…Even more unusual cases are those of extrarenal Wilms tumors located to the gynaecological tract 8–11. The case we reported combines these two uncommon characteristics of the Wilms tumor: the possibility of a late recurrence (in this case 16 years after the primary diagnosis) and the genital system location.…”

Section: Discussionmentioning

confidence: 64%

“…A Wilms tumor is defined extrarenal when it is histologically confirmed as nephroblastoma and when it is located in an extrarenal site 13. According to Connheims’ cell rest theory, extrarenal Wilms tumors have been suggested to arise from persistent mesonephric duct remnants in the wall of the uterine cervix and vagina, ovary and inguinal regions, or from cells with persistent embryonic potential 7 8. In our case, the atypical location of the tumor relapse, linked to an unclear initial histological definition, made it difficult to define the best therapeutic approach and delayed the surgical treatment.…”

Section: Discussionmentioning

confidence: 88%

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First-reported case of vaginal late recurrence of Wilms tumor treated with complete pelvic exenteration

Pizzuti,

Quijano,

Vicente

et al. 2023

BMJ Case Rep

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Late relapses of Wilms tumour are extremely uncommon but still represent possible events. Even more rarely Wilms tumours present as extrarenal neoplasms, for which it could be difficult to diagnose and treat them promptly.We present a unique case of late recurrence of Wilms tumour 16 years after the primary diagnosis, with location in the gynaecological system. The relapse presented as a vaginal mass, and it gradually raised up to involve the majority of pelvic organs. We accurately studied the tumour extension, even realising a 3D preoperative reconstruction, and we managed to evaluate the patient with a multidisciplinary team involving general surgeons, urologists, gynaecologists and plastic surgeons. We finally decided for an extended surgical approach and realised a complete pelvic exenteration. Three months after surgery, the patient is in a good general condition, without major surgical complications and with no radiological signs of pelvic tumour relapse.

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Section: Discussionmentioning

confidence: 64%

Section: Discussionmentioning

confidence: 88%

First-reported case of vaginal late recurrence of Wilms tumor treated with complete pelvic exenteration

Pizzuti,

Quijano,

Vicente

et al. 2023

BMJ Case Rep

View full text Add to dashboard Cite

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Pediatric Extra-Renal Nephroblastoma (Wilms’ Tumor): A Systematic Case-Based Review

Karim

Shaikhyzada

Abulkhanova

et al. 2023

Cancers

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Wilms Tumor (WT) is one of the most common renal tumors in the pediatric population. Occasionally, WT can primarily develop outside the kidneys (Extra-Renal Wilms Tumor, ERWT). Most pediatric ERWTs develop in the abdominal cavity and pelvis, whereas the occurrence of this tumor in other extra-renal sites represents a minor part of ERWT cases. In addition to describing a case of spinal ERWT (associated with spinal dysraphism) in a 4-year boy (to add a further clinical experience on this very rare pediatric tumor), we performed a case-based systematic literature review on pediatric ERWT. We retrieved 72 papers providing enough information on the diagnosis, treatment, and outcomes of 98 ERWT pediatric patients. Our research highlighted that a multimodal approach involving both chemotherapy and radiotherapy, after partial or complete tumor resection in most cases, was typically used, but there is no standardized therapeutic approach for this pediatric malignancy. However, this tumor may be potentially treated with a better success rate if the diagnostic confirmation is not delayed, the mass can be totally resected, and an appropriate and, possibly, tailored multimodal treatment can be promptly established. In this regard, an international agreement on a unique staging system for (pediatric) ERWT is definitely needed, as well as the development of international research, which may be able to gather several children diagnosed with ERWT and, possibly, lead to clinical trials which should also include developing countries.

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Extrarenal Wilms' Tumour of the Ovary

Cited by 2 publications

References 8 publications

First-reported case of vaginal late recurrence of Wilms tumor treated with complete pelvic exenteration

First-reported case of vaginal late recurrence of Wilms tumor treated with complete pelvic exenteration

Pediatric Extra-Renal Nephroblastoma (Wilms’ Tumor): A Systematic Case-Based Review

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