SFEBES2023 Poster Presentations Adrenal and Cardiovascular (78 abstracts)
Royal United Hospital of Bath, Bath, United Kingdom
We present the case of a 32-year-old gentleman who was reviewed in the endocrinology clinic for hypogonadotropic hypogonadism and he was investigated for persistent HTN. His only symptoms were anxiety and palpitations. We have controlled his blood pressure with doxazosin 10 mg BD and bisoprolol 1.25 mg OD. Investigations: bloods (Jan 23) normetanephrine:3,460 picomole/l, metanephrine:120 picomole/l, aldo/renin ratio: 92, TSH 2.3, prolactin 307, IGF-1: 26,6, testosterone: 5,9 nmol/l. CT Adrenal was normal. NET MDT recommended a MIBG scan and it showed possible mass near the bladder. MRI pelvis prostate showed a partially cystic lobulated lesion measuring 3.9 x 4.2 x 3.2 cm which is closely related to the left superior bladder, but is extra mucosal/perivesical. This has some malignant features and in the context of MIBI uptake is entirely consistent with a pheochromocytoma. Genetic testing has revealed that our patient was heterozygous for a pathogenic FH variant which causes hereditary paragangliomas/pheochromocytomas (HPP). In his case its important to test his relatives for HPP. He underwent surgical resection in July 23 and his histology report is still awaited.
Conclusion: Primary paraganglioma of the urinary bladder is very rare, making up less than 0.05% of all bladder malignancy [1]. It is thought to arise from embryonic rests of chromaffin cells within the bladder wall, and it can present with various complications like paroxysmal hypertension, hematuria, therefore many are misdiagnosed due to lack of specificity. [2] A lesson learnt, to be open to look for other conditions while presenting with another diagnosis, hypoandrogenism in this case.
References: 1. Cheng L, Leibovich BC, Cheville JC. Paraganglioma of the urinary bladder: can biologic potential be predicted? Cancer. 2000; 2. Diagnosis and treatment of a rare tumor-bladder paraganglioma Hang Li,1,2 Jiaodi Xie,1 Zebo Chen, Mol Clin Oncol. 2020 Oct;