Endocrine Abstracts

JOINT335

Background: Co-existence of Paraganglioma (Phaeochromocytoma) and Parkinson’s Disease (PD) in the same patient is exceptional and it is hypothesised that genetic predisposition to the two is contradictory. Only around five previous cases are recorded in the literature, all with paraganglioma found incidentally in either an adrenal or extra-adrenal location (never both). Clinically both are associated with supine hypertension and orthostatic hypotension (OH) making the management of blood pressure (BP) changes of PD in the context of paraganglioma that much more complicated.

Purpose: To highlight a) the unusual nature of this combination of illnesses and b) the difficulties of diagnosis and management when they co-exist.

Case description: A 75-year-old white British gentleman with a history of PD, hypertension, diabetes mellitus and gout was admitted to the hospital having been found on the floor at home. After further assessment for ischaemic-looking toes, a CT-angiogram revealed three incidental abdominal masses that were avidly contrast-enhancing raising the possibility of adrenal mass with further related mesenteric nodules. Biochemistry showed raised serum chromogranin A and B, urinary 24-hour normetanephrine and 3-methoxytyramine (3-MT). As 3-MT is a metabolite of dopamine it is raised in the presence of iatrogenic levodopa making interpretation confusing. Urinary 24-hour metanephrines were normal. MIBG (metaiodobenzylguanidine) scan supported a diagnosis of phaeochromocytoma with extra-adrenal secondaries. Although treatment of hypertension in paraganglioma depends on alpha and beta-blockers in our patient, who also suffered from problematic OH, such vaso-active medications were undesirable. He was discharged on pyridostigmine for OH with monitoring of hypertension and further oncology review. He was subsequently re-started on ramipril for control of symptomatic high systolic BP.

Conclusions: This case describes for the first time in literature, as far as we are aware, the presence of both adrenal and extra-adrenal paragangliomas in a patient with PD. It also highlights the complexities of diagnosis and management.

References: 1. Matsumura-Matsuda E, Sekiya M, Omoto-Inuzuka M, Santo K, Shikama A, Kuba M, Sugano Y, Iwasaki H, Yatoh S, Sato T, Hara H, Takekoshi K, Suzuki H, Shimano H. A Rare Coexistence of Pheochromocytoma and Parkinson’s Disease With Diagnostic Challenges. Intern Med. 2018 Apr 1;57(7):979-985. doi: 10.2169/internalmedicine.9242-17. Epub 2017 Dec 21. PMID: 29269645; PMCID: PMC5919857 2. Holder AC, Dylewski A, Brown JN. Pyridostigmine for the Management of Neurogenic Orthostatic Hypotension: A Systemic Review. J Geriatr Psychiatry Neurol. 2024 Jul 23:8919887241266800. doi: 10.1177/08919887241266800. Epub ahead of print. PMID: 39043171.