Two Cases of Composite Pheochromocytoma-Ganglioneuromas With Plasma Metanephrine Levels in the Subclinical Range Pheochromocytoma-Ganglioneuroma
Background/Objective: In <10% of cases, pheochromocytomas coexist with other tumors, most commonly ganglioneuromas, and are termed composite pheochromocytoma-ganglioneuromas. We present 2 cases of composite pheochromocytoma-ganglioneuromas and review the diagnosis and management of these rare tum...
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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/S2376060524001123 |
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| author | Mayssam El Najjar, MD Elie Naous, MD Caroline Blake Aysegul Bulut, MD Alexander Perepletchikov, MD Ann T. Sweeney, MD |
| author_facet | Mayssam El Najjar, MD Elie Naous, MD Caroline Blake Aysegul Bulut, MD Alexander Perepletchikov, MD Ann T. Sweeney, MD |
| author_sort | Mayssam El Najjar, MD |
| collection | DOAJ |
| description | Background/Objective: In <10% of cases, pheochromocytomas coexist with other tumors, most commonly ganglioneuromas, and are termed composite pheochromocytoma-ganglioneuromas. We present 2 cases of composite pheochromocytoma-ganglioneuromas and review the diagnosis and management of these rare tumors. Case Report: Patient 1 and patient 2 were 35-year-old and 45-year-old woman, respectively. Patient 1 presented with a history of controlled hypertension and symptoms of anxiety along with palpitations, diaphoresis, and flushing. Patient 2 complained of abdominal pain and underwent abdominal computed tomography (CT) imaging. Patient 1 and patient 2 had metanephrine levels of 76 pg/mL and 61 pg/mL (normal <57 pg/mL), respectively, and normetanephrine levels of 161 pg/mL and 116 pg/mL (normal < 148 pg/mL), respectively. CT scans depicted right adrenal masses in both cases: patient 1 had a 2.3 × 2.6 cm mass measuring 36 Hounsfield units on noncontrast CT imaging and patient 2 had a 4.5 × 3.5 cm right adrenal mass measuring 73 Hounsfield units on contrast CT imaging. Both patients underwent laparoscopic robotic adrenalectomies without complications. The pathologic analyses of both cases revealed composite pheochromocytoma-ganglioneuroma tumors. Surveillance at 1 year in both patients demonstrated no evidence of recurrence. Discussion: The clinical and radiological presentation of composite pheochromocytoma-ganglioneuromas mirrors pheochromocytomas. The diagnosis relies on histopathological analysis. Treatment of pheochromocytoma-ganglioneuromas is complete surgical excision in a high-volume center with adrenal expertise and is associated with an overall excellent prognosis. The probability of recurrence is low, and distant metastases have rarely been reported. Conclusion: Pheochromocytoma-ganglioneuromas may present with plasma metanephrine levels in the subclinical range. As with isolated pheochromocytomas, lifetime surveillance is critical for composite pheochromocytoma-ganglioneuromas. |
| format | Article |
| id | doaj-art-bc4f70d4474f420a9bb7bf3521d3e716 |
| institution | Kabale University |
| issn | 2376-0605 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | Elsevier |
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| series | AACE Clinical Case Reports |
| spelling | doaj-art-bc4f70d4474f420a9bb7bf3521d3e7162025-01-15T04:11:45ZengElsevierAACE Clinical Case Reports2376-06052025-01-011114548Two Cases of Composite Pheochromocytoma-Ganglioneuromas With Plasma Metanephrine Levels in the Subclinical Range Pheochromocytoma-GanglioneuromaMayssam El Najjar, MD0Elie Naous, MD1Caroline Blake2Aysegul Bulut, MD3Alexander Perepletchikov, MD4Ann T. Sweeney, MD5Department of Medicine, St Elizabeth’s Medical Center, Brighton, MassachusettsDepartment of Medicine, St Elizabeth’s Medical Center, Brighton, MassachusettsDepartment of Medicine, St Elizabeth’s Medical Center, Brighton, MassachusettsDepartment of Medicine, Division of Endocrinology, Boston Medical Center, Boston, MassachusettsProfessional Pathology Services, Professional Corporation, Hilton Head Hospital, Hilton Head Island, South CarolinaDepartment of Medicine, Division of Endocrinology, St Elizabeth’s Medical Center, Brighton, Massachusetts; Address correspondence to Dr Ann T. Sweeney, Department of Medicine, Division of Endocrinology, St Elizabeth’s Medical Center, 736 Cambridge Street, Brighton, MA 02135.Background/Objective: In <10% of cases, pheochromocytomas coexist with other tumors, most commonly ganglioneuromas, and are termed composite pheochromocytoma-ganglioneuromas. We present 2 cases of composite pheochromocytoma-ganglioneuromas and review the diagnosis and management of these rare tumors. Case Report: Patient 1 and patient 2 were 35-year-old and 45-year-old woman, respectively. Patient 1 presented with a history of controlled hypertension and symptoms of anxiety along with palpitations, diaphoresis, and flushing. Patient 2 complained of abdominal pain and underwent abdominal computed tomography (CT) imaging. Patient 1 and patient 2 had metanephrine levels of 76 pg/mL and 61 pg/mL (normal <57 pg/mL), respectively, and normetanephrine levels of 161 pg/mL and 116 pg/mL (normal < 148 pg/mL), respectively. CT scans depicted right adrenal masses in both cases: patient 1 had a 2.3 × 2.6 cm mass measuring 36 Hounsfield units on noncontrast CT imaging and patient 2 had a 4.5 × 3.5 cm right adrenal mass measuring 73 Hounsfield units on contrast CT imaging. Both patients underwent laparoscopic robotic adrenalectomies without complications. The pathologic analyses of both cases revealed composite pheochromocytoma-ganglioneuroma tumors. Surveillance at 1 year in both patients demonstrated no evidence of recurrence. Discussion: The clinical and radiological presentation of composite pheochromocytoma-ganglioneuromas mirrors pheochromocytomas. The diagnosis relies on histopathological analysis. Treatment of pheochromocytoma-ganglioneuromas is complete surgical excision in a high-volume center with adrenal expertise and is associated with an overall excellent prognosis. The probability of recurrence is low, and distant metastases have rarely been reported. Conclusion: Pheochromocytoma-ganglioneuromas may present with plasma metanephrine levels in the subclinical range. As with isolated pheochromocytomas, lifetime surveillance is critical for composite pheochromocytoma-ganglioneuromas.http://www.sciencedirect.com/science/article/pii/S2376060524001123composite tumorpheochromocytoma-ganglioneuromasubclinical pheochromocytomadiagnosistreatment |
| spellingShingle | Mayssam El Najjar, MD Elie Naous, MD Caroline Blake Aysegul Bulut, MD Alexander Perepletchikov, MD Ann T. Sweeney, MD Two Cases of Composite Pheochromocytoma-Ganglioneuromas With Plasma Metanephrine Levels in the Subclinical Range Pheochromocytoma-Ganglioneuroma AACE Clinical Case Reports composite tumor pheochromocytoma-ganglioneuroma subclinical pheochromocytoma diagnosis treatment |
| title | Two Cases of Composite Pheochromocytoma-Ganglioneuromas With Plasma Metanephrine Levels in the Subclinical Range Pheochromocytoma-Ganglioneuroma |
| title_full | Two Cases of Composite Pheochromocytoma-Ganglioneuromas With Plasma Metanephrine Levels in the Subclinical Range Pheochromocytoma-Ganglioneuroma |
| title_fullStr | Two Cases of Composite Pheochromocytoma-Ganglioneuromas With Plasma Metanephrine Levels in the Subclinical Range Pheochromocytoma-Ganglioneuroma |
| title_full_unstemmed | Two Cases of Composite Pheochromocytoma-Ganglioneuromas With Plasma Metanephrine Levels in the Subclinical Range Pheochromocytoma-Ganglioneuroma |
| title_short | Two Cases of Composite Pheochromocytoma-Ganglioneuromas With Plasma Metanephrine Levels in the Subclinical Range Pheochromocytoma-Ganglioneuroma |
| title_sort | two cases of composite pheochromocytoma ganglioneuromas with plasma metanephrine levels in the subclinical range pheochromocytoma ganglioneuroma |
| topic | composite tumor pheochromocytoma-ganglioneuroma subclinical pheochromocytoma diagnosis treatment |
| url | http://www.sciencedirect.com/science/article/pii/S2376060524001123 |
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