ECE2024 Eposter Presentations Adrenal and Cardiovascular Endocrinology (155 abstracts)
A thousand faces of pheochromocytoma: Insights from a case series
Romana Marušić 1,2 , Ines Bilić-Ćurčić 2,3 , Ema Schönberger 2,3 , Kristina Steiner 3 , Klara Ormanac 2,3 , Eduard Oštarijaš 4 & Silvija Canecki-Varžić 2,3
1General Hospital Vukovar, Department of Internal Medicine, Vukovar, Croatia; 2Faculty of Medicine Osijek, Osijek, Croatia; 3Clinical Hospital Center Osijek, Department of Endocrinology, Osijek, Croatia; 4General Hospital Virovitica, Department of Internal Medicine, Virovitica, Croatia
Introduction: Pheochromocytomas, rare neuroendocrine tumors originating from adrenal medulla chromaffin cells, excessively secrete catecholamines, manifesting diverse symptoms. With an incidence of 2-9 per million inhabitants, these tumors pose diagnostic challenges due to their varied clinical presentation.
Case Description: We present five patients diagnosed with pheochromocytoma at the Clinical Hospital Center Osijek. The incidentaloma of the adrenal gland was discovered while diagnosing unrelated conditions, and none of the patients displayed typical pheochromocytoma symptoms. While one patient reported abdominal pain, others remained asymptomatic. CT was performed, and all patients exhibited significantly elevated normetanephrine levels in serum and urine, with only one showing marginally elevated metanephrine in urine. Patients’ characteristics are shown in Table 1.
| Patient | 1 | 2 | 3 | 4 | 5 |
| Age (years) | 34 | 76 | 67 | 67 | 57 |
| Sex (male/female) | male | female | male | male | female |
| Body mass index (kg/m2;) | 21.5 | 34.1 | 26.6 | 25.8 | 28.4 |
| Prior cardiovascular disease | - | - | yes | - | - |
| Hypertension | - | yes | yes | - | yes |
| Number of antihypertensives | 0 | 2 | 1 | 0 | 1 |
| Beta - blocker | - | - | yes | - | yes |
| Positive family history | - | - | - | - | - |
| CT* characteristics Side, size (cm), native density (**HU), absolute wash - out/relative wash - out (%)) | left, 11x8.8, 30, 60/40 | left, 3.8x4.6, 32 | left, 3.5x3.1, 35, 71/56 | right, 1.6x1.4, 30, 38/54 | left, 3.8x3.4, 20, 59/69 |
| Plasma metanephrine (pmol/l), reference range | 0.22 (0.01-0.3) | - | 0.34 (0.05-0.36) | 0.27 (0.05-0.36) | 0.29 (0.05-0.38) |
| Plasma normetanephrine (pmol/l), reference range | 0.80 (0.13-0.62) | - | 3.87 (0.14-1.05) | 1.68 (0.14-1.05) | 3.20 (0.14-0.75) |
| Urinary metanephrine (umol/dU), reference range | 0.20 (<0.27) | 0.18 (<0.17) | 0.19 (<0.27) | 0.12 (<0.27) | 0.86 (<1.62) |
| Urinary normetanephrine (umol/dU), reference range | 0.48 (<0.24) | 3.96 (<0.19) | 0.95 (<0.24) | 0.38 (<0.24) | 5.34 (<2.13) |
| Tumor type pheochromocytoma/paraganglioma | pheochromocytoma | pheochromocytoma | pheochromocytoma | pheochromo cytoma | |
| Size of tumor (cm) - PHD | 12x11.5 | 5x3.8 | 4.2x2.7 | 1x1.2 | 3x2.5 |
| AJCC*** staging | pT2 | pT2 | pT1 | pT1 | pT1 |
| PASS score**** | 7/20 | 4/20 | 1/20 | - | 0/20 |
| Ki-67 (%) | 1 - 3 | 1 | <1 | < 1 | 2 |
| Treatment | surgery | surgery | surgery | surgery | surgery |
| Follow - up | complete response | complete response | complete response | complete response | complete response |
| *CT: computed tomography; **HU: Hounsfield Units; ***AJCC: American Joint Committee on Cancer Staging for Pheochromocytoma, ****PASS Score: Pheochromocytoma of the Adrenal Gland Scaled Score | |||||
Conclusion: Pheochromocytoma, lacking specific symptoms, often evades recognition. If there is a clinical suspicion or imaging finding of incidentaloma of the adrenal gland, biochemical testing for pheochromocytoma is crucial.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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