Extrarenal malignant rhabdoid tumors (MRTs) are rare tumors with a poor prognosis. Five-year overall survival for patients with MRTs is poor at approximately 20%.(1) There are 5 case reports of histologically confirmed primary MRT of the bladder in pediatric patients. Herein, we report a case of an MRT of the bladder in a 14-year-old boy and discuss the preoperative evaluation, treatment options, and possible etiologies of metastasis after radical surgery.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4646707PMC
http://dx.doi.org/10.1016/j.urology.2014.11.011DOI Listing

Publication Analysis

Top Keywords

malignant rhabdoid
8
mrt bladder
8
rapid progression
4
progression disease
4
disease surgical
4
surgical excision
4
excision malignant
4
rhabdoid tumor
4
tumor bladder
4
bladder extrarenal
4

Similar Publications

Paediatric renal tumours: an update on challenges and recent developments.

Virchows Arch

January 2025

Histology Laboratory, Children's Health Ireland, Dublin, Ireland.

Paediatric renal tumours include a broad range of neoplasms which largely differ, but also overlap to a smaller extent, with adult kidney cancer. These include the embryonal tumour nephroblastoma, which accounts for the majority of cases of kidney cancer in the first decade of life and, despite boasting a cure in ~ 90% cases, still presents clinical challenges in a small proportion of cases. A variety of less common mesenchymal tumours, including the mostly indolent congenital mesoblastic nephroma, clear cell sarcoma of kidney which continues to be associated with poor outcomes for higher stage disease, and the typically lethal malignant rhabdoid tumour, form the bulk of the remaining presentations in the first decade.

View Article and Find Full Text PDF

Background: Atypical teratoid rhabdoid tumor (ATRT) is the most common malignant brain tumor in infants, and more than 60% of children with ATRT die from their tumor. ATRT is associated with mutational inactivation/deletion of , a member of the SWI/SNF chromatin remodeling complex, suggesting that epigenetic events play a critical role in tumor development and progression. Moreover, disruption of SWI/SNF allows unopposed activity of epigenetic repressors, which contribute to tumorigenicity.

View Article and Find Full Text PDF

SMARCA4-deficient undifferentiated cervical carcinoma is an extremely rare and aggressive malignancy, and effective treatment options are lacking. We experienced a rare case involving a patient with SMARCA4-deficient undifferentiated cervical carcinoma who was successfully managed in the long term. A woman in her 40s presented with a chief complaint of abnormal vaginal bleeding.

View Article and Find Full Text PDF

Background: The role of fine needle aspiration cytology (FNAC) in the diagnosis of renal malignancies is established and has been getting more precise and important over a period of time. Knowledge of the pathology of uncommon renal neoplasms along with radiological and clinical correlations often aids in correct diagnosis.

Aims: The present study aims to describe the cytomorphological and immunohistochemical findings in the varied spectrum of renal tumors, other than renal cell carcinomas (RCC).

View Article and Find Full Text PDF

Malignant rhabdoid tumor is a rare highly aggressive neoplasm that affects young children. It is composed of stromal and epithelial components and commonly arises from the kidney. The clinical presentation is usually nonspecific, and the common signs are palpable abdominal mass, hematuria, fever, anemia, and hypercalcemia.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!