Pediatric Case Report Bladder Ganglioneuroma in a 5-Year-old Girl Presenting With a Urinary Tract Infection and Hematuria: Case Report and Review of the Literature Christopher Hartman, Alex K. Williamson, Ariella A. Friedman, Lane S. Palmer, and Ronnie G. Fine Ganglioneuromas are rare benign tumors arising from neural crest cells of the autonomic nervous system. These tumors may rarely localize to the bladder, and few cases have been reported in the adult literature. To date, however, bladder ganglioneuromas have not been reported in the pediatric literature. We report the case of a 5-year-old girl who presented with hematuria and a urinary tract infection and on workup was found to have a large bladder mass. Transurethral resection and pathologic examination revealed the mass to be a ganglioneuroma. The case is presented followed by a brief review of the literature. UROLOGY 85: 467e469, 2015.  2015 Elsevier Inc.


anglioneuromas are rare tumors of the autonomic nervous system, originating in the neural crest cells of the sympathetic nervous system.1 They may present anywhere sympathetic nervous tissue is found; however, these tumors most frequently occur in the abdomen. Although they may grow to a large size and exhibit a mass effect that causes local symptoms, these mature tumors are benign. We report the case of a 5-yearold girl who presented with gross hematuria and was found to have a large bladder ganglioneuroma after surgical resection.

CASE REPORT A severely autistic, nonverbal 5-year-old Chinese girl presented to her pediatrician with a 5-week history of hematuria, dysuria, increased aggression, and mild incontinence. She was initially diagnosed with a Proteus mirabilis urinary tract infection and treated accordingly with culture-specific antibiotics. Her symptoms recurred twice during the following month with cultures isolating the same organism; she was therefore placed on a 10-day suppressive course of amoxicillin-clavulanate and referred for pediatric urologic evaluation. General examination revealed an avoidant, nonverbal 5-year-old girl with normal physical development for her age. An abdominal examination did not elicit flank Financial Disclosure: The authors declare that they have no relevant financial interests. From the Department of Pediatric Urology, Cohen Children’s Medical Center of New York, Hofstra North Shore-LIJ School of Medicine, New Hyde Park, NY; and the Department of Anatomic Pathology, Hofstra North Shore-LIJ School of Medicine, NY Address correspondence to: Christopher Hartman, M.D., The Arthur Smith Institute for Urology, 450 Lakeville Road, Suite M41, New Hyde Park, NY 11040. E-mail: [email protected] Submitted: July 31, 2014, accepted (with revisions): October 21, 2014

ª 2015 Elsevier Inc. All Rights Reserved

tenderness, and her bladder and kidneys were nonpalpable. Genitourinary examination showed Tanner stage 1 normal external genitalia. A basic metabolic panel was normal, including creatinine. A urinalysis obtained, while the patient was not experiencing hematuria revealed no nitrites, leukocyte esterase, white blood cells, or red blood cells. Renal and bladder ultrasonography was performed, demonstrating normal kidneys without evidence of hydronephrosis. However, a 3.8-cm well-defined ovoid mass was seen arising from the left lateral bladder wall and protruding into the bladder lumen (Fig. 1). Urine cytology revealed reactive and degenerated urothelial cells but no evidence of malignancy. To further characterize the mass seen in the bladder and to rule out extravesical extension, a magnetic resonance imaging (MRI) was performed. This showed a 3.6  3.5  2.8 cm mass with heterogeneous enhancement on the left side of the bladder, without evidence of lymphadenopathy or hydroureteronephrosis. The mass did not extend beyond the bladder and had a smooth capsule (Fig. 2). On the basis of these findings, transurethral resection of the bladder tumor was performed. A frozen section sent during the case indicated that the tumor was a benign spindle cell neoplasm, and the final pathology revealed a ganglioneuroma, maturing subtype (Fig. 3).

PATHOLOGIC FINDINGS On gross inspection, the resected tissue appeared as tanwhite to pink and gray soft tissue, ranging from 0.2 to 2.5 cm in the greatest individual dimension and aggregating to approximately 6  5  2 cm. Intraoperative frozen section performed on 2 smaller pieces revealed fibroconnective tissue and a spindle cell neoplasm, which was favored to represent a ganglioneuroma. 0090-4295/15


Figure 1. Ultrasonography image of a 3.6-cm left lateral wall bladder mass.

Figure 2. T1-weighted coronal magnetic resonance image revealing a large mass emanating from the left lateral bladder wall.

Permanent histologic sections demonstrated mature ganglioneuromatous tissue comprised of abundant Schwannian stroma containing individual and focally clustered mature ganglion cells (Fig. 3B). In one level, there was a microscopic cluster of differentiating neuroblasts and maturing ganglion cells, and rare maturing ganglion cells were distributed throughout the tumor as well. The tumor appeared to arise within or adjacent to the bladder wall, as some sections showed neural tumor pushing into, but not invading, smooth muscle (confirmed by desmin and S100 immunostains, Fig. 3C). Bladder mucosa focally present in some sections showed slight and nonspecific chronic inflammation. Evidence of bladder invasion or mucosal involvement was not identified (Fig. 3D). Histologic features of a paraganglioma, neurofibroma, leiomyoma, or malignant neoplasm were not identified.

COMMENT In the present case, a severely autistic 5-year-old girl presented with gross hematuria, dysuria, and mild incontinence. Workup revealed a large bladder mass, and cystoscopy with transurethral resection confirmed a bladder ganglioneuroma. To our knowledge, this is the first report of a bladder ganglioneuroma in a pediatric patient. 468

Pediatric bladder tumors are exceedingly rare, with fewer than 150 cases of bladder transitional cell carcinoma reported in the literature. Additionally, fewer than 20% of these cases occurred in children under the age of 10 years.2 Presentation of bladder tumors in children mimics that in adults, with hematuria being the most common presenting symptom.3 In contrast to adults, however, among pediatric patients who present with gross hematuria,

Bladder ganglioneuroma in a 5-year-old girl presenting with a urinary tract infection and hematuria: case report and review of the literature.

Ganglioneuromas are rare benign tumors arising from neural crest cells of the autonomic nervous system. These tumors may rarely localize to the bladde...
853KB Sizes 0 Downloads 5 Views