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A case report of infantile cystic nephroblastoma

BACKGROUND: Nephroblastoma (NB) is a malignant embryonal neoplasm derived from nephrogenic blastemal cells. NB usually forms a solid mass, but in extremely rare cases, it may show cystic changes. CASE PRESENTATION: A six-month-old girl with persistent high fevers was found to have pyuria and bacteri...

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Autores principales: Kurose, Nozomu, Takenaka, Michiho, Yamashita, Manabu, Shimaguchi, Chie, Nakano, Mariko, Britni, Bryant, Guo, Xin, Futatsuya, Chizuru, Shioya, Akihiro, Yamada, Sohsuke
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2018
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Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6204273/
https://www.ncbi.nlm.nih.gov/pubmed/30368245
http://dx.doi.org/10.1186/s13000-018-0761-5
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author Kurose, Nozomu
Takenaka, Michiho
Yamashita, Manabu
Shimaguchi, Chie
Nakano, Mariko
Britni, Bryant
Guo, Xin
Futatsuya, Chizuru
Shioya, Akihiro
Yamada, Sohsuke
author_facet Kurose, Nozomu
Takenaka, Michiho
Yamashita, Manabu
Shimaguchi, Chie
Nakano, Mariko
Britni, Bryant
Guo, Xin
Futatsuya, Chizuru
Shioya, Akihiro
Yamada, Sohsuke
author_sort Kurose, Nozomu
collection PubMed
description BACKGROUND: Nephroblastoma (NB) is a malignant embryonal neoplasm derived from nephrogenic blastemal cells. NB usually forms a solid mass, but in extremely rare cases, it may show cystic changes. CASE PRESENTATION: A six-month-old girl with persistent high fevers was found to have pyuria and bacteriuria. Ultrasonography revealed multilocular cysts in the right kidney. Right nephrectomy was performed with cyst wall rupture during surgery. An intraoperative rapid diagnosis, based on peritoneal fluid cytology, confirmed three components of blastemal, stromal, and epithelial cells. The blastemal cells were dyshesive, with scant to no cytoplasm and were the predominant cell type. The spindle-shaped stromal cells were arranged in fascicles. The epithelial cells demonstrated tubular structures. Macroscopically, the resected cystic tumor measured 80 mm in maximum diameter with a prominently thin cyst wall, but solid areas were also apparent. Histologically, the tumor was diagnosed as cystic NB (blastemal-predominant) displaying a triphasic pattern. Hyperchromatic nuclei and apoptotic bodies were found. The clinical stage classification of Japan Wilms Tumor Study group was 3. The patient was treated with chemotherapy and radiotherapy. Tumor recurrence and metastasis have not been observed in the 8 months since surgery. CONCLUSION: This is an extremely rare case of infantile cystic NB. We diagnosed the NB cells that appeared in the peritoneal fluid by intraoperative rapid cytology. Cytological examination proved to be a very useful technique for determining the clinical stage of NB. Additionally, we propose that massive tumor degeneration and necrosis be considered as a pathogenic mechanism of cyst formation in NB.
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spelling pubmed-62042732018-10-31 A case report of infantile cystic nephroblastoma Kurose, Nozomu Takenaka, Michiho Yamashita, Manabu Shimaguchi, Chie Nakano, Mariko Britni, Bryant Guo, Xin Futatsuya, Chizuru Shioya, Akihiro Yamada, Sohsuke Diagn Pathol Case Report BACKGROUND: Nephroblastoma (NB) is a malignant embryonal neoplasm derived from nephrogenic blastemal cells. NB usually forms a solid mass, but in extremely rare cases, it may show cystic changes. CASE PRESENTATION: A six-month-old girl with persistent high fevers was found to have pyuria and bacteriuria. Ultrasonography revealed multilocular cysts in the right kidney. Right nephrectomy was performed with cyst wall rupture during surgery. An intraoperative rapid diagnosis, based on peritoneal fluid cytology, confirmed three components of blastemal, stromal, and epithelial cells. The blastemal cells were dyshesive, with scant to no cytoplasm and were the predominant cell type. The spindle-shaped stromal cells were arranged in fascicles. The epithelial cells demonstrated tubular structures. Macroscopically, the resected cystic tumor measured 80 mm in maximum diameter with a prominently thin cyst wall, but solid areas were also apparent. Histologically, the tumor was diagnosed as cystic NB (blastemal-predominant) displaying a triphasic pattern. Hyperchromatic nuclei and apoptotic bodies were found. The clinical stage classification of Japan Wilms Tumor Study group was 3. The patient was treated with chemotherapy and radiotherapy. Tumor recurrence and metastasis have not been observed in the 8 months since surgery. CONCLUSION: This is an extremely rare case of infantile cystic NB. We diagnosed the NB cells that appeared in the peritoneal fluid by intraoperative rapid cytology. Cytological examination proved to be a very useful technique for determining the clinical stage of NB. Additionally, we propose that massive tumor degeneration and necrosis be considered as a pathogenic mechanism of cyst formation in NB. BioMed Central 2018-10-27 /pmc/articles/PMC6204273/ /pubmed/30368245 http://dx.doi.org/10.1186/s13000-018-0761-5 Text en © The Author(s). 2018 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Kurose, Nozomu
Takenaka, Michiho
Yamashita, Manabu
Shimaguchi, Chie
Nakano, Mariko
Britni, Bryant
Guo, Xin
Futatsuya, Chizuru
Shioya, Akihiro
Yamada, Sohsuke
A case report of infantile cystic nephroblastoma
title A case report of infantile cystic nephroblastoma
title_full A case report of infantile cystic nephroblastoma
title_fullStr A case report of infantile cystic nephroblastoma
title_full_unstemmed A case report of infantile cystic nephroblastoma
title_short A case report of infantile cystic nephroblastoma
title_sort case report of infantile cystic nephroblastoma
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6204273/
https://www.ncbi.nlm.nih.gov/pubmed/30368245
http://dx.doi.org/10.1186/s13000-018-0761-5
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