Diagnosis and Treatment > Medication
Search for other papers by Stephanie Teasdale in
Google Scholar
PubMed
Search for other papers by Elham Reda in
Google Scholar
PubMed
Summary
We present two cases of adrenal phaeochromocytoma in patients with a previous diagnosis of neurofibromatosis type 1 (NF1). One had an adrenergic phenotype. The other had a more noradrenergic phenotype. Both had large primary tumours, which increases the likelihood of malignancy. Both also had elevated plasma-free methoxytyramine, which has been linked with malignancy even in non-SDHB phaeochromocytomas.
Learning points
-
Phaeochromocytoma can have varied clinical presentations.
-
Methoxytyramine can be useful in the biochemical work-up of both SDHB-positive and SDHB-negative phaeochromocytoma.
-
The utility of methoxytyramine as a marker of malignancy in NF1-related phaeochromocytoma is unclear, and cases with elevated titres warrant longer follow-up.
