ECEESPE2025 ePoster Presentations Adrenal and Cardiovascular Endocrinology (170 abstracts)
A rare case of silent metastatic pheochromocytoma with a breast metastasis
Panagiotis Karageorgiou 1 , Maria Zotou 1 , Vasiliki Antonopoulou 1 , Sofia Georgiadi 1 , Aggeliki Kyriakou 1 , Angeliki Angelidou 1 , Kanella Kantreva 1 , Anna Louisa Chaliasou 1 , Athina Valatsou 2 , Georgios Kyriakopoulos 3 , Marinella Tzanela 1 & Georgia Ntali 1
1Department of Endocrinology "D. Ikkos"- Diabetes Center- EndoERN-Evangelismos General Hospital, Athens, Greece; 2Private Clinic, Karditsa, Greece; 3Department of Pathology, Evangelismos General Hospital, Athens, Greece
JOINT3724
Background: Pheochromocytomas are rare neuroendocrine tumors arising from chromaffin cells of adrenal medulla. Their clinical presentation is heterogeneous with various symptoms and signs related to catecholamine excess. Rarely, pheochromocytomas are biochemically and clinically silent. According to WHO 2022 all pheochromocytomas can potentially metastasize in non-chromaffin tissues. Common sites of metastasis are lymph nodes, bones, lungs and liver. Herein, we present a rare case of silent metastatic pheochromocytoma with metastasis to lungs and breast.
Case: A 46-year-old female was diagnosed with a 5 cm left adrenal incidentaloma, four years ago. Its imaging phenotype was indeterminate and endocrine work up revealed no hormone hypersecretion. The patient underwent left adrenalectomy, and the pathology examination indicated a 7 cm pheochromocytoma with intermediate risk stratification Grading system for Adrenal Pheochromocytoma and Paraganglioma (GAPP score 4) and Pheochromocytoma of the Adrenal gland Scaled Score (PASS score 9). Postoperative reevaluation with I131-MIBG and 18F-FDG-PET/CT imaging were negative and the catecholamines levels were within normal levels. During the following years the patient remained asymptomatic and she underwent yearly testing with plasma or urine metanephrines/normetanephrines which remained negative. Four years post-surgery, a “suspicious” right breast lesion appeared on a screening mammogram, and the patient underwent surgical removal of the tumor. Pathology was positive for chromaffin tissue. At the same time, biochemical examination showed increased plasma 3-methoxytyramine levels (33.3 ng/l, normal range<17), whereas the patient remained asymptomatic. 68Ga-Dotatoc and 18F-FDG-PET/CT imaging detected lung metastases, and a tyrosine kinase inhibitor (sunitinib) was initiated for further treatment. However, two months later, disease progression was noticed, and chemotherapy was recommended.
Conclusions: To our knowledge, there are only two reports in bibliography about pheochromocytomas with metastasis to the breast. In our case, the patient was asymptomatic and plasma metanephrines and normetanephrines levels were constantly normal. However, an increase of dopamine metabolite, 3-methoxytyramine, was observed at the time of metastasis. Dopamine has low affinity for a and β adrenergic receptors and this can explain why patients with dopamine-secreting pheochromocytomas are often asymptomatic.
Volume 110
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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