Extrarenal teratoid wilms tumor

Unny, Ashitha K; Subramanian, Balagopal; Srinivasan, Arathi; Rabia, Seyed

doi:10.4103/jiaps.jiaps_190_21

Cited by 2 publications

(1 citation statement)

References 3 publications

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“…There are about a hundred cases of ERWT presented in literature. The most common locations of ERWT are the retroperitoneum, inguinal area, lumbosacral and pelvic region, female genital organs (including the uterus, cervix, vagina, and ovaries), mediastinum and chest wall, and spermatic cord and paratesticular region (1,(3)(4)(5)(6)(7)(8)(9)(10)(11)(12).…”

Section: Introductionmentioning

confidence: 99%

Extrarenal Wilms tumor mimicking funicular hydrocele

Mijović,

Rašić,

Miličković

et al. 2024

Medicinska istraživanja

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Introduction/Objective: Extrarenal Wilms' tumor (ERWT) comprises 0.5% to 1% of all nephroblastoma cases. The most common locations of ERWT are the retroperitoneum, inguinal, lumbosacral and pelvic region, female genital organs, mediastinum and chest wall, spermatic cord and paratesticular region. ERWT most likely originates from the ectopic nephrogenic rest that undergoes a malignant transformation. The exclusion of primary renal tumor is necessary to establish the diagnosis of ERWT. Patient Review: A 15-month-old male was operated on because of a left-sided inguinal mass that clinically resembled a funicular hydrocele. The surgical exploration of the inguinal canal revealed a solid tumor located in the area of the external inguinal ring, measuring around 2cm in diameter, with no ingvinal hernia present. Complete surgical resection of the mass was performed. The histological structure of the tumor corresponded to non-anaplastic nephroblastoma (Wilms tumor), mixed type. After abdominal CT scan primary renal tumor was excluded and the diagnosis of ERWT was made. Because of microscopic tumor rests on the resection margin, the patient was treated according to the high-risk SIOP Wilms tumor protocol. The patient made a full recovery. During the 10-year follow-up, the boy has remained disease-free. Conclusion: ERWT is mostly diagnosed after the surgical removal of the specimen. The clinical resemblance of the ERWT located in the inguinal region to common benign inguinal conditions in children may lead to the omission of detailed preoperative work-up. Most children with ERWT in the inguinal region have an excellent prognosis, although most of them require adjuvant chemotherapy and/or radiotherapy after surgery.

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Section: Introductionmentioning

confidence: 99%

Extrarenal Wilms tumor mimicking funicular hydrocele

Mijović,

Rašić,

Miličković

et al. 2024

Medicinska istraživanja

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Mediastinal Teratoma with Nephroblastomatous Elements: Case Report, Literature Review, and Comparison with Maturing Fetal Glomerulogenic Zone/Definitive Zone Ratio and Nephrogenic Rests

Alfawaz,

Koujok,

Eamer

et al. 2024

IJMS

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Extrarenal teratoid Wilms’ tumor (TWT) is a variant of Wilms’ tumor with fewer than 30 cases reported in the literature. It comprises more than 50% heterologous tissue and presents a significant diagnostic challenge due to its complex histology. We report an unusual case of mediastinal teratoma with nephroblastomatous elements in an 8-year-old female. The patient presented with respiratory distress, fever, weight loss, and a large anterior mediastinal mass. Imaging revealed a heterogeneous tumor containing fat, fluid, and calcification, suggestive of a teratoma. Surgical resection confirmed a mature cystic teratoma with foci of nephroblastoma. Pathological analysis demonstrated a mixture of ectodermal, mesodermal, and endodermal tissues alongside nephroblastomatous components. Immunohistochemistry was positive for Wilms Tumor 1 and other relevant markers, confirming the diagnosis. The patient had an uneventful postoperative course and was discharged after three days. This case adds to the growing body of research on extrarenal TWT, particularly its occurrence in the mediastinum, a rare site for such tumors. A literature review highlighted that extrarenal TWT often affects children, typically presenting in the retroperitoneum or sacrococcygeal regions, with varying recurrence rates and long-term outcomes. This case underscores the importance of histopathological and immunohistochemical analysis in diagnosing TWT and differentiating it from other mediastinal tumors to ensure appropriate treatment planning, emphasizing the need for long-term follow-up due to the potential for recurrence or metastasis. This paper also provides an in-depth look at nephron development and nephrogenic rests, highlighting the structural and functional aspects of nephrogenesis and the factors that disrupt it in fetal kidneys.

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Extrarenal teratoid wilms tumor

Abstract: Teratoid Wilms tumor (TWT) is a variant of Wilms tumor. 30 cases have been reported so far. The extrarenal TWT variant has only been 5 in number. We are reporting a case of retroperitoneal TWT in a 6-year-old female child with diagnostic and therapeutic challenges.

Cited by 2 publications

References 3 publications

Extrarenal Wilms tumor mimicking funicular hydrocele

Extrarenal Wilms tumor mimicking funicular hydrocele

Mediastinal Teratoma with Nephroblastomatous Elements: Case Report, Literature Review, and Comparison with Maturing Fetal Glomerulogenic Zone/Definitive Zone Ratio and Nephrogenic Rests

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