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Nine pheochromocytomas in the same patient. Final mapping with ultrasound and angiography
C P Nolsøe1, L T Jensen, S Torp-Pedersen
1Department of Diagnostic Ultrasound, Herlev Hospital, University of Copenhagen, Denmark.
Insights
This case study highlights a patient with multiple pheochromocytomas, emphasizing the importance of comprehensive imaging. Early and complete diagnosis through angiography is crucial for managing this rare adrenal tumor.
Area of Science:
- Endocrinology
- Oncology
- Radiology
Background:
- Pheochromocytoma is a rare neuroendocrine tumor arising from chromaffin cells, often presenting with hypertension due to excess catecholamine secretion.
- Diagnosis typically involves biochemical tests and imaging, but challenges can arise with multifocal or extra-adrenal disease.
Observation:
- A 37-year-old male presented with hypertension and elevated urine catecholamines, initially diagnosed with a right adrenal pheochromocytoma.
- Post-surgical persistence of symptoms indicated additional tumors, confirmed by further imaging revealing multiple retroperitoneal pheochromocytomas.
Findings:
- Histopathological examination confirmed 8 pheochromocytomas without signs of invasive growth.
- Complete surgical extirpation of all identified pheochromocytomas led to full patient recovery and normalization of blood pressure.
Implications:
- Comprehensive abdominal and pelvic imaging, including angiography, should be standard for pheochromocytoma workup to detect multifocal disease.
- Aggressive surgical management of multiple pheochromocytomas can lead to complete cure and long-term remission.
Abstract:
A 37-year-old man presented with hypertension and elevated urine catecholamine. Ultrasound scanning revealed a solid tumour of the right adrenal gland and two solid tumours in the retroperitoneum. The findings were confirmed with computed tomography and abdominal angiography. At surgery only the tumour of the right adrenal gland was removed. The histopathologic diagnosis was pheochromocytoma. Postoperatively the symptoms and biochemistry were unchanged and the patient was referred for further treatment. At ultrasonography and abdominal aortography 6 remaining tumours were demonstrated. Surgery was performed and 8 pheochromocytomas were extirpated (3 were closely spaced small tumours in a conglomerate corresponding to one of the visualized tumour sites). On histopathologic examination no signs of invasive growth were found. The patient recovered completely. The blood pressure was still normal 2 1/2 years later. Angiography and non-invasive examination of the entire abdomen and pelvis should be routine when pheochromocytomas are searched for.