Document Detail

URINARY BLADDER PARAGANGLIOMA IN CHILDHOOD: A case report and review of the literature.
MedLine Citation:
PMID:  21385006     Owner:  NLM     Status:  Publisher    
Abstract Bladder paraganglioma (BP) is a rare entity, and is exceedingly uncommon in childhood. Pheochromocytomas/Paragangliomas are components of several hereditary cancer syndromes, and up to 30% may be associated with germ line mutations of genes including VHL, RET and SDH. We present a 16-year-old female who was admitted with macroscopic hematuria and anemia. A cystoscopy demonstrated a polypoid and hemorrhagic mass arising from the floor of the bladder. She underwent a transurethral resection of clinically suspected urothelial papilloma. A histologic examination of the tumor showed large polygonal cells with eosinophilic cytoplasm, arranged in a zellballen pattern, surrounded by a fibrous network. Immunohistochemical studies showed a strong expression of neuroendocrine markers and lack of reactivity for epithelial markers. The diagnosis of BP was established; eight months later, a recurrence was observed and the patient underwent a partial cystectomy. Our case is the first BP in childhood reported in the literature with absent SDHB staining by immunohistochemistry. We discuss the clinical and pathologic findings and present a review of BP in childhood.
Olga L Bohn; Edgar Pardo-Castillo; Mariana Fuertes-Camilo; Nina P Rios-Luna; Andres Martinez; Sergio Sanchez-Sosa
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-3-8
Journal Detail:
Title:  Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society     Volume:  -     ISSN:  1093-5266     ISO Abbreviation:  -     Publication Date:  2011 Mar 
Date Detail:
Created Date:  2011-3-9     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9809673     Medline TA:  Pediatr Dev Pathol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
1 MetroHealth Medical Center.
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