Urinary Bladder Paraganglioma in Childhood: A Case Report and Review of the Literature

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 represents the 1st 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.

Pelvic and Bladder Catecholamine-Producing Tumors: A Review of the Literature

“…In decreasing order of frequency, PGLs may develop: (i) in the Zuckerkandl body, a vestigial chromaffin ganglion located at the root of the upper mesenteric artery; (ii) in the sympathetic plexus of the urinary bladder, the kidneys, and the heart; or (iii) in sympathetic ganglia in the mediastinum, the head or the neck. Most head and neck PGL are non-secreting [83], as in our second case report.…”

“…PGLs can be classified as functional and non-functional types [83], depending on the capacity to produce different types of catecholamines (e.g. epinephrine, norepinephrine, and dopamine) and their metabolites.…”

Micturition-triggered severe headache associated with bladder paraganglioma: A case report

“…These tumors are highly prevalent in young adult females, with the common symptoms and signs being hypertension and hematuria (10). The pathogenic factors underlying bladder paragangliomas remain unclear and may be related to certain gene mutations (11). Although some patients show a full recovery after complete tumor resection, some experience recurrence, metastases, or even death from the tumor.…”

Malignant Urinary bladder paraganglioma in 12 year old boy

“…Paragangliomas of the urinary bladder are rare tumors that can present at any age (range 11-84 years) with a mean age of 45 years and with slight female sex predilection [1]. In the pediatric population they are extremely rare, with just over 12 cases reported in the literature [6]. The commonest site within the bladder is the trigone and the posterior wall [8].The lateral wall has also been cited as a common site [7].As many as 50% of the paragangliomas are hereditary and may be associated with familial paraganglioma, neurofibromatosis type 1, von Hippel-Lindau disease, and the Carney triad [9].Histologically, they are characterized by cells arranged in discrete nests separated by a prominent sinusoidal network.…”