Paraganglioma Of Urinary Bladder Research Articles

6402 Unsuspected Bladder Mass Turns Out To Be A Paraganglioma

Abstract Disclosure: S. Lamichhane: None. A. Flowers: None. D.T. Price: None. A. Kaur: None. Background: Urinary bladder (UB) paragangliomas are extremely rare tumors comprising of less than 0.05% of all bladder neoplasms and 10% of all paragangliomas. The diagnosis is usually suspected based on clinical features of painless hematuria, episodic headaches, hypertension, palpitations associated with micturition or bladder and confirmed biochemically by elevated urinary and/or plasma fractionated metanephrines (MN). Here, we present a case of a UB mass that was initially diagnosed on biopsy and subsequent biochemical workup revealed elevated MN. Case Report: A 64-year-old man with type 2 diabetes and hypertension, admitted for revision surgery of subdural hematoma was incidentally found to have a 3.3 x 3.4 x 3.9 cm polypoid heterogenous hyper enhancing UB mass attached to the anterior wall on CT abdomen done for abdominal pain. Cystoscopy and TURBT were complicated by hypertensive urgency. Pathology turned out to be paraganglioma. Detailed history at this time revealed a long-standing history of frequency, urgency, nocturia 4 times nightly, recurrent urinary tract infections, an episode of gross hematuria 1-2 years ago, and difficulty controlling blood pressure on 4 anti-hypertensives. Subsequently, labs showed plasma free MN of 97 pg/ml (ref range <=57), free normetanephrine (NMN) of 2392 pg/ml (ref range <=148), and total MN of 2489 pg/ml (ref range <=205). Genetic panel was negative for SDHA, SHD, AF2, SDHB, SHDC, SDHD, VHL, RET, TMEM127. He received 6 doses of Lantreotide due to suspicion of metastasis based on abdominal, pelvic, and inguinal lymphadenopathy seen on DOTATATE scan. After preparation with alpha and beta blockers, he underwent uneventful robot-assisted cystectomy with lymph node resection that resulted in a well-differentiated paraganglioma negative for margins, lymph node involvement, and extra bladder extension. A week later, MN reduced, plasma free MN 37 pg/ml, free NMN 178 pg/ml, and total plasma MN 215 pg/ml. His BP improved, and micturition-induced palpitations resolved. Conclusion: Due to the rarity of UB paraganglioma, the diagnosis can be missed and delayed. This has a serious implication due to the risk of hypertensive crisis during the surgery and increased risk of cardiovascular complications. This case highlights the need for awareness among physicians of the clinical presentation associated with UB paraganglioma, its early recognition and management. Presentation: 6/2/2024

Diagnosis and management of urinary bladder paragangliomas: A Sino-American-European retrospective observational study.

Paragangliomas of the urinary bladder (UBPGLs) are rare neuroendocrine tumours and pose a diagnostic and surgical challenge. It remains unclear what factors contribute to a timely presurgical diagnosis. The purpose of this study is to identify factors contributing to missing the diagnosis of UBPGLs before surgery. A total of 73 patients from 11 centres in China, and 51 patients from 6 centres in Europe and 1 center in the United States were included. Clinical, surgical and genetic data were collected and compared in patients diagnosed before versus after surgery. Logistic regression analysis was used to identify clinical factors associated with initiation of presurgical biochemical testing. Among all patients, only 47.6% were diagnosed before surgery. These patients were younger (34.0 vs. 54.0 years, p < .001), had larger tumours (2.9 vs. 1.8 cm, p < .001), and more had a SDHB pathogenic variant (54.7% vs. 11.9%, p < .001) than those diagnosed after surgery. Patients with presurgical diagnosis presented with more micturition spells (39.7% vs. 15.9%, p = .003), hypertension (50.0% vs. 31.7%, p = .041) and catecholamine-related symptoms (37.9% vs. 17.5%, p = .012). Multivariable logistic analysis revealed that presence of younger age (<35 years, odds ratio [OR] = 6.47, p = .013), micturition spells (OR = 6.79, p = .007), hypertension (OR = 3.98, p = .011), and sweating (OR = 41.72, p = .013) increased the probability of initiating presurgical biochemical testing. Most patients with UBPGL are diagnosed after surgery. Young age, hypertension, micturition spells and sweating are clues in assisting to initiate early biochemical testing and thus may establish a timely presurgical diagnosis.

SAT631 EPAS1 Mutations In Three Adjacent Nucleotides Resulting In Different Phenotypes

Abstract Disclosure: M. Alswailem: None. B. Alghamdi: None. L.A. Alobaid: None. H. Al-Hindi: None. M. Aldawish: None. O. Alsagheir: None. A.S. Alzahrani: None. EPAS1 (HIF2a) mutations have been described in patients (pts) with paragangliomas (PGL) in association with cyanotic congenital heart disease (CHD), polycythemia and some additional tumors. Here, we present 3 cases of PGL with closely located EPAS1 mutations. Despite the common gene location, their manifestations are different. 1. A 14-year-old girl found at age 9 years (yrs) to have polycythemia (Hb 177-195 g/l) and hypertension. At age 14 years, she was diagnosed to have multiple mid abdomenal and the urinary bladder PGLs. U. noremetanephrine was 35.8 umol/day (0-3.43). No evidence of somatostatinoma so far. Family hx is negative for PGL and WES revealed no germline mutations in PGL-associated genes. Tumor testing revealed a novel heterozygous EPAS1 variant (c.1589C&amp;gt;T, p.A530V). 2. A 32-yr-old woman has a complex cyanotic CHD diagnosed since birth. She underwent five cardiac surgeries between 1993 and 2015. In 2011, she was diagnosed with two abdominal PGLs. She underwent surgery and remained free of disease until 2019 when she developed recurrence with metastases (mets) to the liver and spine. She received external beam radiotherapy to the spine. Currently PGL mets remained stable. WES was negative for any germline mutation but tumor testing revealed a heterozygous EPAS1 mutation (c.1591C&amp;gt;T, p.P531S). 3. A 55-yr-old man diagnosed to have an upper abdominal PGL with lung and bone metastases at the age of 9 yrs. Biopsy from a right femoral met confirmed the diagnosis of bone mets. He underwent four abdominal surgeries between 1978-2008 and received MIBG Tx 6 times with a cumulative dose is 586 mCi. His last surgery in 2008 revealed PGLs but also a non-functioning Pancreatic neuroendocrine tumor (pNET). PGL mets remained stable over the last 15 yrs. WES revealed no germline mutation in any of the known PGL genes but tumor testing revealed an EPAS1 mutation (c.1592C&amp;gt;A, p.P531H). Discussion:EPAS1 mutations are very rare. In this series, we present 3 pts with EPAS1 mutations in 3 adjacent nucleotides (c.1589, 1591, 1592) in 2 adjacent codons (p.530-531). These mutations are located in a domain that is important for recognition by propyl hydroxylases that normally lead to inactivation and proteasomal degradation of HIF2a (EPAS1). Despite the common location, the manifestations are quite different. Although all of them developed PGL at a young age, Pt # 1 had severe polycythemia, Pt#2 had complex CHD, and pt#3 had pNET. The first two patients had extensive mets but these mets have a smoldering course, especially in pt#3 who has been living with these mets for 44 yrs! Since EPAS1 mutations are usually postzygotic, as suggested by its involvement of more than one organ, it is possible that the variable presentations of these same domain adjacently located mutations is related to the timing of their post zygotic development. Another possibility is that the variable expression of the mutated EPAS1 in different tissues Presentation: Saturday, June 17, 2023

Rare incidental presentation of urinary bladder paraganglioma and the anaesthetic challenges faced: A case report

Urinary bladder paraganglioma is an extra-adrenal pheochromocytoma. As it lacks specific symptoms and because of its rare occurrence, it presents incidentally while tumour handling. A forty-eight-year-old female who presented with nausea, vomiting, weight loss, pain abdomen and hematuria was diagnosed with a bladder mass. She was scheduled for transurethral resection of the bladder tumour. On tumour manipulation, it showed numerous fluctuations in heart rate and blood pressure, from where a differential diagnosis of urinary bladder paraganglioma was made. Histopathology revealed a zellbellan pattern of paraganglioma. This case was discharged successfully postoperatively. But if undiagnosed preoperatively, it can be a real challenge because of the fatal hypertensive crisis and life-threatening cardiopulmonary complications.

Paraganglioma of urinary bladder, an innocent rarity or a nightmare: A case series report and review

Paraganglioma of urinary bladder, an innocent rarity or a nightmare: A case series report and review

Somatic IDH1 Hotspot Variants in Chinese Patients With Pheochromocytomas and Paragangliomas.

IDH1 is a pheochromocytoma/paraganglioma (PPGL) susceptibility gene; however, its role, especially in the Chinese population, has not been characterized. To determine the prevalence of somatic IDH1 hotspot variants in a large cohort of Chinese patients with PPGLs and to summarize associated phenotypes. This retrospective cross-sectional study was based on a main cohort of 1141 patients with PPGLs from 2 tertiary-care centers in China. We included 50 cases with urinary bladder paragangliomas (UBPGLs), of whom 29 were part of the main cohort and 21 were from other centers. Two additional cases with IDH1 hotspot variants not part of the main cohort were also included for summarizing IDH1-associated phenotypes. Next-generation sequencing of tumor DNA was used to analyze a customized panel of genes. The overall prevalence of IDH1 hotspot variants in the main cohort was 0.5% (6/1141). Among those PPGLs without mutations in 15 common driver genes, the prevalence of IDH1 variants was 0.9% (4/455). When restricted to paraganglioma (PGL) without mutations, the prevalence reached 4.7% (4/86). Among UBPGLs, IDH1 hotspot variants accounted for 8% (4/50). Together, all 10 patients (9 PGLs and 1 pheochromocytoma) with IDH1 hotspot variants, including 3 females with concurrent EPAS1 hotspot variants, had apparently sporadic tumors, without metastasis or recurrence. There were 3 patients with biochemical data, all showing a non-adrenergic phenotype. The somatic IDH1 hotspot variants cause PPGL development in some Chinese patients, especially among those apparently sporadic PGLs with a non-adrenergic phenotype and without mutations in major PPGL driver genes.

RF19 | PSUN367 Urinary bladder Paragangliomas – A Case series

Abstract Introduction Paragangliomas (PGL) are rare neuroendocrine tumours and account for approximately 10% of all chromaffin tumours. They may occur in the retroperitoneum, mediastinum, skull and the urinary bladder. Paragangliomas of the urinary bladder are rare entities accounting for 10% of all paragangliomas and 0.06% of all bladder tumours. Approximately 10% of urinary bladder paragangliomas are malignant. Bladder paragangliomas can be either functional or non-functional. Main symptoms of functional PGLs are due to catecholamine excess with palpitations, paroxysmal hypertension during micturition, sweating and headaches. Functional bladder PGL are diagnosed on biochemical analysis. Non-functional bladder PGLs present with haematuria and are often misdiagnosed as bladder cancer as they may cystoscopically and histologically resemble a urothelial neoplasm. Clinical cases: We present a case series of 4 surgically treated patients with urinary bladder paragangliomas who presented to Cambridge University Hospital over the last 3 years. All patients were female with an age range between 21 and 44 years. All four patients had SDHB deficient tumours, and all four patients were diagnosed with a pathogenic variant in the SDHB gene. Three patients had no family history of SDHx associated neoplasia whereas one had a paternal aunt who died from a metastatic pheochromocytoma. Three patients had presented with palpitations and hypertensive episodes, one of whom experienced symptoms while micturating. Biochemical analysis revealed elevated plasma normetadrenaline levels in all patients. On presentations, one patient had a synchronous paratracheal paraganglioma while two patients had lymph node metastasis. All patients were surgically treated with two patients undergoing fertility preserving cystectomy and bladder substitution, one undergoing partial cystectomy, radical hysterectomy and ureteric implantation and one managed with robot-assisted partial cystectomy. None of the patients have evidence of biochemical or radiological tumour recurrence on follow-up with a mean follow-up time of 23 months (range 12 to 40 months). Conclusion and points for discussion The diagnosis of urinary bladder PGL is challenging. Biochemical assessment to assess catecholamine production and imaging studies like CT, MRI and NM Ga68 DOTATATE PET CT are helpful modalities in diagnosis. Patients presenting with extra adrenal paragangliomas including bladder paragangliomas require genetic testing because of the strong hereditary predisposition, with mutations in the SDHx genes being most common with this clinical phenotype. The surgical approach should be tailored to the individual patient, agreed by a multi-disciplinary team and the risk of malignancy should be weighed against the potential morbidity associated with a curative oncological surgery. As urinary bladder PGLs have malignant potential and are frequently hereditary (as demonstrated in this series), lifelong follow-up is required to screen for synchronous tumours and tumour recurrence. Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m., Sunday, June 12, 2022 12:42 p.m. - 12:47 p.m.

RF33 | PSUN02 Presentation, Management, and Outcomes of Urinary Bladder Paraganglioma: Results from a Multi-center Study

Abstract Context and Objectives Urinary bladder paraganglioma (UBPGL) is rare. We aimed to characterize the presentation and outcomes of patients diagnosed with UBPGL. Methods We conducted a multi-center study of consecutive patients with pathologically-confirmed UBPGL evaluated between 1971 and 2021. Outcomes included repeat bladder surgery, metastases, and disease-specific mortality. Results 110 patients (56, 51% women) were diagnosed with UBPGL at a median age of 50 years (IQR, 36-61 years). Median tumor size was 2 cm (IQR, 1-4 cm). UBPGL was diagnosed prior to biopsy in only 37 (34%), and only 69 (63%) patients had evaluation for catecholamine excess. In addition to the initial bladder surgery, 26 (25%) required multiple therapies, including repeat surgery in 10 (9%). Synchronous metastases were present in 9 (8%) patients, and 24 (22%) other patients with UBPGL developed metachronous metastases at a median of 4 years (IQR, 2-10 years) after initial diagnosis. Development of metachronous metastases was associated with younger age (Hazard ratio, HR 0.97, 95%CI 0.94-0.99), UBPGL size (HR of 1.69, 95%CI of 1.31-2.17), and a higher degree of catecholamine excess (HR of 5.48, 95%CI of 1.40-21.39). Disease-specific mortality was higher in patients with synchronous metastases (HR of 20.80, 95%CI, 1.30-332.91). Choice of initial surgery, genetic association, sex, or presence of muscular involvement on pathology were not associated with development of metastases or mortality. Conclusions Only a minority of patients were diagnosed before biopsy/surgery, reflecting the need of better diagnostic strategies. All patients with UBPGL should be monitored life-long for development of recurrence and metastases. Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m., Monday, June 13, 2022 12:48 p.m. - 12:53 p.m.

Presentation, Management, and Outcomes of Urinary Bladder Paraganglioma: Results From a Multicenter Study.

Urinary bladder paraganglioma (UBPGL) is rare. We aimed to characterize the presentation and outcomes of patients diagnosed with UBPGL. We conducted a multicenter study of consecutive patients with pathologically confirmed UBPGL evaluated between 1971 and 2021. Outcomes included repeat bladder surgery, metastases, and disease-specific mortality. Patients (n=110 total; n=56 [51%] women) were diagnosed with UBPGL at a median age of 50 years (interquartile range [IQR], 36-61 years). Median tumor size was 2 cm (IQR, 1-4 cm). UBPGL was diagnosed prior to biopsy in only 37 (34%), and only 69 (63%) patients had evaluation for catecholamine excess. In addition to the initial bladder surgery, 26 (25%) required multiple therapies, including repeat surgery in 10 (9%). Synchronous metastases were present in 9 (8%) patients, and 24 (22%) other patients with UBPGL developed metachronous metastases at a median of 4 years (IQR, 2-10 years) after the initial diagnosis. Development of metachronous metastases was associated with younger age (hazard ratio [HR] 0.97; 95% CI, 0.94-0.99), UBPGL size (HR 1.69; 95% CI, 1.31-2.17), and a higher degree of catecholamine excess (HR 5.48; 95% CI, 1.40-21.39). Disease-specific mortality was higher in patients with synchronous metastases (HR 20.80; 95% CI, 1.30-332.91). Choice of initial surgery, genetic association, sex, or presence of muscular involvement on pathology were not associated with development of metastases or mortality. Only a minority of patients were diagnosed before biopsy/surgery, reflecting need for better diagnostic strategies. All patients with UBPGL should have lifelong monitoring for development of recurrence and metastases.

Urinary bladder paraganglioma with a family history of neurofibromatosis type 1 treated with partial cystectomy.

Urinary bladder paraganglioma with a family history of neurofibromatosis type 1 treated with partial cystectomy.

A rare case report of Urinary Bladder Paraganglioma

Paragangliomas are extra-adrenal tumors of the autonomic nervous system which is termed pheochromoctyoma in adrenal gland. These tumors are found within the skull base, neck, mediastinum and periaortic region. Paragangliomas of the urinary bladder are rare, and non-functioning bladder paraganglioma is even rarer and not easily recognized. Histopathological examination of the tumor is essential to establish the diagnosis of paraganglioma. This is a case report of 67year male patient presenting with hematuria and passage of blood clots per urethra in emergency department. Cystoscopic examination revealed an exophytic growth in upper posterior wall of his bladder with areas of hemorrhagic blood clots within the vicinity of growth. His blood pressure was found 120/75 mm of Hg with normal serum sodium and potassium levels. Bipolar transurethral resection of the bladder mass was performed. Intraoperative procedure was uneventful and no rise in blood pressure during resection was found. Histopathological examination with immunohistochemistry confirmed diagnosis paraganglioma, urinary bladder. Patient is doing well and is kept on follow up.

Approach to Urinary Bladder Paraganglioma

Approach to Urinary Bladder Paraganglioma

Non-functional urinary bladder paraganglioma with negative Ga-DOTA-NOC PET/CT uptake

BackgroundParaganglioma of the urinary bladder is a very rare benign tumor that constitutes less than 0.5% of all bladder tumors. It can be functional and non-functional. Functional tumors present with symptoms of catecholamine excess, and non-functional tumors usually remain asymptomatic.Case presentationHere, we present a case of non-functional urinary bladder paraganglioma in a 36-year-old female who presented to us with a history of intermittent hematuria. The routine hematological and biochemical parameters were within normal limits. Diagnostic computed tomography (CT) revealed a hypervascular lesion in the urinary bladder dome with no significant uptake in gallium-68-labelled-1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acid peptides-NaI3-octreotide positron emission tomography (DOTA-NOC PET/CT). Histopathology and immunohistochemistry confirmed the diagnosis of paraganglioma.ConclusionWe present this case because of the rare location of paraganglioma in the urinary bladder and being non-functional with low/absent somatostatin receptor expression.

Differences in clinical presentation and management between pre- and postsurgical diagnoses of urinary bladder paraganglioma: is there clinical relevance? A systematic review

PurposeParaganglioma of the urinary bladder (UBPGL) is a rare neuroendocrine tumor diagnosed in many patients only after surgery. We, therefore, assessed clinical clues relevant to presurgical diagnosis and clinical consequences in patients with a missed presurgical diagnosis of UBPGL.Materials and methodsCase reports describing a UBPGL (published from 1–1–2001 and 31–12–2020) were identified in Pubmed. Two authors independently performed data extraction and assessed data quality according to the PRISMA guideline. Patients were divided into two groups: UBPGL diagnosis before and after surgery.ResultsWe included 177 articles reporting 194 cases. In 90 (46.4%) patients, the UBPGL was diagnosed before and in 104 (53.6%) after surgery. In presurgically diagnosed UBPGL, hypertension and catecholamine-associated symptoms were 2- to 3-fold (p < 0.001) more frequent than in postsurgically diagnosed patients whereas hematuria was twofold (p = 0.003) more prevalent in those with postsurgical diagnosis. Hypertension was an independent factor for presurgical biochemical testing (OR 4.45, 95% CI 1.66–11.94) while hematuria (OR 0.23, 95% CI 0.09–0.60) was an independent factor for not performing presurgical biochemical testing. Most patients diagnosed after surgery were not pretreated with alpha-adrenoceptor blockade (95.2%), underwent more frequently transurethral resection instead of cystectomy (70.2% vs. 23.1%) and had more frequent peroperative complications and residual tumor mass.ConclusionsIn nearly half of all patients with a UBPGL, the diagnosis was not established before surgery. Hypertension and hematuria contributed independently to a presurgical diagnosis. Postsurgical diagnosis, which was associated with suboptimal presurgical and surgical management, resulted in more peroperative complications and incomplete tumor resections.

Site-based performance of 131I-MIBG imaging and 99mTc-HYNIC-TOC scintigraphy in the detection of nonmetastatic extra-adrenal paraganglioma.

ObjectivesThis study aimed to evaluate the performance of 131I-metaiodobenzylguanidine (MIBG) imaging to detect nonmetastatic extra-adrenal paragangliomas at their respective sites (abdominal vs. thoracic vs. head and neck vs. urinary bladder), and compare it with that of 99mTc-hydrazinonicotinyl-tyr3-octreotide (HYNIC-TOC) scintigraphy.MethodsWe retrospectively analyzed 235 patients with nonmetastatic extra-adrenal paragangliomas who underwent preoperative 131I-MIBG imaging or 99mTc-HYNIC-TOC scintigraphy. Of all 235 patients, 145 patients underwent both imaging procedures, 16 patients 131I-MIBG imaging only and 74 patients 99mTc-HYNIC-TOC scintigraphy only.ResultsThe overall sensitivity of 131I-MIBG and 99mTc-HYNIC-TOC imaging to detect extra-adrenal paragangliomas regardless of tumor sites was 75.8% (122/161) and 67.6% (148/219), respectively (P = 0.082). However, when stratified by tumor sites, 131I-MIBG imaging showed a significant improvement in the detection of extra-adrenal abdominal paragangliomas with a sensitivity of 90.3% (103/114), which was significantly higher than that of 99mTc-HYNIC-TOC scintigraphy (67.6% (96/142); P = 0.000). In addition, the intensity of tracer uptake in the extra-adrenal abdominal paragangliomas with 131I-MIBG imaging was evidently higher than with 99mTc-HYNIC-TOC scintigraphy. The sensitivity of 131I-MIBG imaging and 99mTc-HYNIC-TOC scintigraphy to detect urinary bladder, head and neck, and thoracic paragangliomas were 18.7 vs. 18.5% (P = 1.000); 17.4% vs. 84.6% (P = 0.000) and 60% vs. 94.4% (P = 0.030), respectively.Conclusions131I-MIBG imaging could become the first-line investigation modality in patients with extra-adrenal abdominal paragangliomas. However, 99mTc-HYNIC-TOC scintigraphy has high sensitivity and is superior to 131I-MIBG imaging for detecting head & neck and thoracic paraganglioma. Both 131I-MIBG imaging and 99mTc-HYNIC-TOC scintigraphy have poor performance for detecting urinary bladder paragangliomas.

Presyncope in a Woman With Incidental Bladder Mass: A Case of De Novo SDHB Mutation Bladder Paraganglioma

Background: Paragangliomas (PGLs) are catecholamine secreting neuroendocrine tumors originating from extra-adrenal neural crest cells. Urinary bladder paragangliomas (UBPGLs) are extremely rare, accounting for only 0.06% of all bladder tumors. Sporadic UBPGLs associated with Succinate Dehydrogenase B (SDHB) mutation have a higher malignant potential versus patients with a positive family history due to a lack of screening. We present a case of sporadic UBPGL detected early due to follow-up of an incidental bladder mass. Clinical Case: A 31 year-old female presented to the ER with presyncope and lower abdominal pain while running. On arrival she was mildly hypertensive with BP 140/88 mmHg, HR was 98 beats/min, other vital signs were stable. Denied headaches, diaphoresis, or weight loss. No known personal or family history of any endocrine disorders or cancer. CT abdomen pelvis performed for the evaluation of her abdominal pain revealed a large fibroid along with an unexpected finding of a 3 cm enhancing lesion in the right bladder wall, suggestive of a PGL. 24 hr urine total catecholamines and plasma total metanephrines were elevated at 284 mcg (normal range 26-121 mcg/24h), and 386 pg/mL (normal <205 pg/ml) respectively, confirming the diagnosis of UBPGL. Chromogranin A was within the normal range. Metastasis was ruled out with metaiodobenzylguanidine (MIBG) whole-body scan and octreotide scan which showed isolated uptake in the bladder. Pan CT scan was negative for other SBHB mutation associated tumors such as RCC (14% incidence), GIST, and thyroid carcinoma. Genetic testing with PGLNest revealed a heterozygous SDHB mutation (5’-3’ UTR_5’-3’ UTR deletion). Her parents were found to be negative for the mutation. During follow up she developed episodic palpitations. She was treated with alpha followed by beta blocker and underwent successful resection of the tumor with partial cystectomy, and of the fibroid with myomectomy on the same day (TAH was avoided to minimize manipulation of the uterus hence decreasing the risk of intra-abdominal HTN crisis). She has been tumor-free in the three years of follow up post-surgery with imaging and biochemical surveillance. Conclusion: This case illustrates the importance of following up on incidental radiographic findings and adds to the limited literature on sporadic UBPGLs, an uncommon, yet potentially life-threatening tumor. The diagnosis of these tumors is especially challenging when patients present with non-specific symptoms. Fortunately, in our case, the imaging study clued us into the possibility of a PGL. SDHB mutation associated PGLs, in particular, are notorious for their late presentation with metastasis and associated tumors. Hence, timely detection with close follow-up of these patients will likely have an impact on their mortality.

Urinary bladder paraganglioma metastatic to the lung in a patient with SDHB gene mutation: A case report

Introduction: Paragangliomas are tumors originating from the neural crest. Most of them are benign and arise from various locations in the body. Extra-adrenal paragangliomas arise as sporadic cases in most settings or as part of heredofamilial syndromes in about one-quarter of cases. Succinate dehydrogenase subunit B (SDHB) gene mutations are associated with an aggressive clinical disease course of pheochromocytoma/paraganglioma.Methods: We present a 41-year-old male former smoker with a history of a growing right upper lung nodule on chest imaging. He had no cough or respiratory symptoms. Twenty-seven months prior, the patient underwent a cystoprostatectomy due to paraganglioma of the bladder. Genetic testing identified a pathogenic mutation in SDHB gene, c.166_170delCCTCA (p.Pro56Tyrfs*5). He underwent a wedge resection of the lung nodule.Results: Sectioning of the lung wedge revealed a well-circumscribed, firm tan nodule. Microscopically there were nests of large neoplastic cells with round nuclei and eosinophilic granular cytoplasm. Tumor cells were positive for synaptophysin and chromogranin and negative for pan-cytokeratin. S-100 protein highlighted sustentacular cells. Morphologically, the pulmonary neoplasm was similar to the primary tumor of the bladder. These features are consistent with a bladder paraganglioma metastatic to the lung, in a background of a hereditary paraganglioma syndrome.Conclusion: Extra-adrenal paraganglioma occurring in a setting of hereditary paraganglioma syndrome has a high risk of metastasis. Lifelong surveillance even after prompt resection of the primary tumor with negative margins is required to ensure early detection of metastasis and prevent complications associated with it.

Oncology and complications

This collection of cases describes some unusual urological tumors and complications related to urological tumors and their treatment. Case 1: A case of uretero-arterial fistula in a patient with long-term ureteral stenting for ureteral oncological stricture and a second case associated to retroperitoneal fibrosis were described. Abdominal CT, pyelography, cystoscopy were useful to show the origin of the bleeding. Angiography is useful for confirming the diagnosis and for subsequent positioning of an endovascular prosthesis which represents a safe approach with reduced post-procedural complications. Case 2: A case of patient who suffered from interstitial pneumonitis during a cycle of intravesical BCG instillations for urothelial cancer. The patient was hospitalized for more than two weeks in a COVID ward for a suspected of COVID-19 pneumonia, but he did not show any evidence of SARS-CoV-2 infection during his hospital stay. Case 3: A case of a young man with a functional urinary bladder paraganglioma who was successfully managed with complete removal of the tumor, leaving the urinary bladder intact. Case 4: A case of a 61 year old male suffering from muscle invasive bladder cancer who was admitted for a radical cystectomy and on the eighth postoperative day developed microangiopathic hemolytic anemia and thrombocytopenia, which clinically defines thrombotic microangiopathy.

Asymptomatic paraganglioma of urinary bladder.

Asymptomatic paraganglioma of urinary bladder.

A rare case of urinary bladder paraganglioma documented by 18F-FDG PET-CT: Clinical and imaging features

Paragangliomas are extra-adrenal neural crest-derived neoplasms. Hybrid imaging allows for localization, preoperative planning and whole-body assessment. A rare case of urinary bladder paraganglioma is presented. In addition, in this case report, we highlight the selection of the appropriate radi