Malignant Paraganglioma With Calvarial Metastases Presenting With Recurrent Catecholamine-Induced Cardiomyopathy
Background/Objective: Cranial metastases rarely occur in malignant paragangliomas (PGLs) or pheochromocytomas, which usually metastasize to the liver, bone, lungs, and lymph nodes. Early detection and intervention with a multidisciplinary approach are crucial given the critical location. Case Report...
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Elsevier
2025-01-01
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| Series: | AACE Clinical Case Reports |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S237606052400107X |
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| author | Beatrice A. Brumley, MD Run Yu, MD, PhD Shadfar Bahri, MD Jane Rhyu, MD |
| author_facet | Beatrice A. Brumley, MD Run Yu, MD, PhD Shadfar Bahri, MD Jane Rhyu, MD |
| author_sort | Beatrice A. Brumley, MD |
| collection | DOAJ |
| description | Background/Objective: Cranial metastases rarely occur in malignant paragangliomas (PGLs) or pheochromocytomas, which usually metastasize to the liver, bone, lungs, and lymph nodes. Early detection and intervention with a multidisciplinary approach are crucial given the critical location. Case Report: Our patient was a 31-year-old man diagnosed with periaortic PGL and succinate dehydrogenase subunit B pathogenic variant at the age of 9 years with cardiac arrest. He developed intra-abdominal and skeletal metastatic disease by the age of 14 years and treated with surgery, chemotherapy, and radiation. After being lost to follow-up, the patient presented emergently with headache, palpitations, hypertensive crisis, type 2 non-ST-elevation myocardial infarction, and catecholamine-induced cardiomyopathy, with plasma free metanephrine level of 61.0 pg/mL (0.0-88.0 pg/mL) and elevated serum free normetanephrine level of 662.9 pg/mL (0.0-210.1 pg/mL). Imaging showed a right frontal calvarial lesion, with 4.9-cm intracranial dural and 4.9-cm extracranial components, and a 1.5-cm occipital bone lesion. Following adrenergic blockade, the patient underwent resection of the frontal lesion with pathology showing metastatic PGL. Discussion: A multidisciplinary team was consulted. Because of potential neurotoxicity, radiology advised against radiotherapy. Oncology advised monitoring. Seven months postoperatively, gallium-68 dodecane tetraacetic acid–octreotate positron emission tomography/computed tomography showed no recurrence at the surgical site, stable occipital lesion, and additional skeletal metastases. The patient is planned for peptide receptor radionuclide therapy. Conclusion: Our case highlights the importance of active surveillance in PGL and pheochromocytoma to allow early intervention for metastatic disease and reviews the controversial management of rare calvarial or cerebral metastases, including peptide receptor radionuclide therapy. |
| format | Article |
| id | doaj-art-8b46f2c16d4649c69af8a5b15b876b88 |
| institution | Kabale University |
| issn | 2376-0605 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | Elsevier |
| record_format | Article |
| series | AACE Clinical Case Reports |
| spelling | doaj-art-8b46f2c16d4649c69af8a5b15b876b882025-01-15T04:11:44ZengElsevierAACE Clinical Case Reports2376-06052025-01-011112428Malignant Paraganglioma With Calvarial Metastases Presenting With Recurrent Catecholamine-Induced CardiomyopathyBeatrice A. Brumley, MD0Run Yu, MD, PhD1Shadfar Bahri, MD2Jane Rhyu, MD3Division of Endocrinology, Diabetes, and Metabolism, University of California, Los Angeles (UCLA), David Geffen School of Medicine, Los Angeles, CaliforniaDivision of Endocrinology, Diabetes, and Metabolism, University of California, Los Angeles (UCLA), David Geffen School of Medicine, Los Angeles, CaliforniaDivision of Nuclear Medicine, Department of Molecular and Medical Pharmacology, University of California, Los Angeles (UCLA), David Geffen School of Medicine, Los Angeles, CaliforniaDivision of Endocrinology, Diabetes, and Metabolism, University of California, Los Angeles (UCLA), David Geffen School of Medicine, Los Angeles, California; Address correspondence to Dr Jane Rhyu, Division of Endocrinology, Diabetes, and Metabolism, UCLA David Geffen School of Medicine, 10833 Le Conte Ave-CHS 57-145, Los Angeles, CA 90095.Background/Objective: Cranial metastases rarely occur in malignant paragangliomas (PGLs) or pheochromocytomas, which usually metastasize to the liver, bone, lungs, and lymph nodes. Early detection and intervention with a multidisciplinary approach are crucial given the critical location. Case Report: Our patient was a 31-year-old man diagnosed with periaortic PGL and succinate dehydrogenase subunit B pathogenic variant at the age of 9 years with cardiac arrest. He developed intra-abdominal and skeletal metastatic disease by the age of 14 years and treated with surgery, chemotherapy, and radiation. After being lost to follow-up, the patient presented emergently with headache, palpitations, hypertensive crisis, type 2 non-ST-elevation myocardial infarction, and catecholamine-induced cardiomyopathy, with plasma free metanephrine level of 61.0 pg/mL (0.0-88.0 pg/mL) and elevated serum free normetanephrine level of 662.9 pg/mL (0.0-210.1 pg/mL). Imaging showed a right frontal calvarial lesion, with 4.9-cm intracranial dural and 4.9-cm extracranial components, and a 1.5-cm occipital bone lesion. Following adrenergic blockade, the patient underwent resection of the frontal lesion with pathology showing metastatic PGL. Discussion: A multidisciplinary team was consulted. Because of potential neurotoxicity, radiology advised against radiotherapy. Oncology advised monitoring. Seven months postoperatively, gallium-68 dodecane tetraacetic acid–octreotate positron emission tomography/computed tomography showed no recurrence at the surgical site, stable occipital lesion, and additional skeletal metastases. The patient is planned for peptide receptor radionuclide therapy. Conclusion: Our case highlights the importance of active surveillance in PGL and pheochromocytoma to allow early intervention for metastatic disease and reviews the controversial management of rare calvarial or cerebral metastases, including peptide receptor radionuclide therapy.http://www.sciencedirect.com/science/article/pii/S237606052400107Xmetastatic pheochromocytoma/paraganglioma (PCC/PGL)calvarial metastasescatecholamine-induced cardiomyopathypeptide receptor radionuclide therapy (PRRT) |
| spellingShingle | Beatrice A. Brumley, MD Run Yu, MD, PhD Shadfar Bahri, MD Jane Rhyu, MD Malignant Paraganglioma With Calvarial Metastases Presenting With Recurrent Catecholamine-Induced Cardiomyopathy AACE Clinical Case Reports metastatic pheochromocytoma/paraganglioma (PCC/PGL) calvarial metastases catecholamine-induced cardiomyopathy peptide receptor radionuclide therapy (PRRT) |
| title | Malignant Paraganglioma With Calvarial Metastases Presenting With Recurrent Catecholamine-Induced Cardiomyopathy |
| title_full | Malignant Paraganglioma With Calvarial Metastases Presenting With Recurrent Catecholamine-Induced Cardiomyopathy |
| title_fullStr | Malignant Paraganglioma With Calvarial Metastases Presenting With Recurrent Catecholamine-Induced Cardiomyopathy |
| title_full_unstemmed | Malignant Paraganglioma With Calvarial Metastases Presenting With Recurrent Catecholamine-Induced Cardiomyopathy |
| title_short | Malignant Paraganglioma With Calvarial Metastases Presenting With Recurrent Catecholamine-Induced Cardiomyopathy |
| title_sort | malignant paraganglioma with calvarial metastases presenting with recurrent catecholamine induced cardiomyopathy |
| topic | metastatic pheochromocytoma/paraganglioma (PCC/PGL) calvarial metastases catecholamine-induced cardiomyopathy peptide receptor radionuclide therapy (PRRT) |
| url | http://www.sciencedirect.com/science/article/pii/S237606052400107X |
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