The first case of urinary BP was reported in 1953 by Zimmerman et al. in a 74-year-old woman [2]. The epidemiology is still debatable; according to Pastor-Guzmán et al., bladder paragangliomas are very rare and represent 6% of the paragangliomas and 0.06% of all bladder tumors, while Chen reported that 85% of extraadrenal paragangliomas are located below the diaphragm, from which 10% was in the bladder [3, 4].
BP could be divided according to their clinical characteristics into two types: functional and non-functional. Usually, the diagnosis of a functional paraganglioma is often obtained thanks to its clinical manifestations; in our patient, the clinical presentation was not suggestive. According to Siatelis et al. in 90% of the paraganglioma patients, elevated blood pressure, serious dizziness, headache, profuse sweating and palpitation appear; in our case, severe hematuria was the only symptom [5]. We explain this by her XIII factor deficiency because thrombophilic patients have an increased risk for the development of venous thromboembolism, acute myocardial infarction or ischemic stroke, while our patient had no such medical history. Besides these clinical manifestations, according to Deng et al., these patients should also have elevated plasma or urinary catecholamines and positive reaction for metaiodobenzylguanidine or octreotide scintigraphy [6]. All this is absent in the cases of non-functional tumors.
In the absence of hyperadrenalism symptoms, clinical diagnosis is difficult. The histological diagnosis is also difficult; according to Ginesu et al., the differential diagnostic is made with meningiomas, schwannomas, hemangiopericytomas, melanomas and various metastatic carcinomas [7]. When the tumor is located in the bladder, histological characteristics are similar to the most common bladder cancers and thus misdiagnosed as urothelial carcinoma. According to Zhou et al. microscopically, the paraganglioma appears as cells with a ‘zellballen’ pattern with abundant eosinophilic or amphophilic cytoplasm divided by delicate vascular stroma. Fortunately, the diagnostic is suggested through immunohistochemical examination, the tumor being positive for neuro-specific enolase, synaptophysin and chromogranin and negative for cytokeratins [8].
The main therapy for localized BP is complete resection because this type of tumor is insensitive to both chemotherapy and radiotherapy. TURBT and laser resection are mainly used for the treatment of BPs at stage T2. Because some authors like Jurincic et al. are claiming that it is very difficult to completely resect using TURBT, partial cystectomy could be recommend [9]. When the tumor is located in the trigone of the urinary bladder or/and has extensive infiltration into adjacent tissues, a radical cystectomy may be performed. According to Liu et al., the factors that could indicate a high probability of malignancy of the BP are CgA expression, Ki67 > 3%, karyokinesis > 1/10 HPF and/or atypical karyokinesis, confluent necrosis, and appearance of aneuploidy [10]. Considering that our patient had Ki-67 < 5%, karyokinesis < 1/10 HPF and no confluent necrosis, we had treated as a tumor with low chances of malignancy and cystectomy was not performed. There are no guidelines regarding the duration of follow-up and the data in the literature are conflicting. While Beilan et al. suggested that follow-up is not necessary in patients with benign and localized BP, Katiyar et al. recommend a long-period follow-up even in non-functional tumors [11, 12].
To our knowledge, there are no data in the literature that correlate paraganglioma with inherited thrombophilia or coagulation factor XIII deficit, although Hill and Kitamura encountered disseminated intravascular coagulation in patients with pheochromocytoma [13, 14].
