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Phaeochromocytoma--views on current management
D T Williams1, S Dann, M H Wheeler
1Department of Endocrine Surgery, University Hospital of Wales, Heath Park, CF14 4XW, Cardiff, UK.
Insights
Surgical excision of phaeochromocytoma offers a high cure rate for hypertension with zero mortality. Adequate preoperative management and a multidisciplinary approach are crucial for optimal outcomes, especially in complex familial or multiple tumor cases.
Area of Science:
- Endocrinology
- Surgical Oncology
- Nephrology
Background:
- Phaeochromocytoma, a rare neuroendocrine tumor, often presents with hypertension.
- Effective management requires accurate diagnosis, localization, and surgical intervention.
Purpose of the Study:
- To evaluate the diagnostic and management strategies for phaeochromocytoma.
- To assess surgical outcomes and identify challenges in patient care.
Main Methods:
- Retrospective analysis of 47 patients undergoing surgical excision of phaeochromocytoma.
- Utilized urinary catecholamines for diagnosis and imaging (CT, MRI, MIBG) for localization.
- Preoperative management included alpha-adrenergic blockade with phenoxybenzamine and beta-blockers.
Main Results:
- Hypertension was the most common presentation (70%).
- CT and MRI were the most sensitive localization tools.
- Zero 30-day postoperative mortality with 21.3% complication rate.
- 80% of patients were cured of hypertension.
- Cortex-sparing procedures were unsuccessful for familial or multiple tumors.
Conclusions:
- Surgical excision of phaeochromocytoma is safe and effective, leading to high hypertension cure rates.
- Preoperative pharmacological control and a multidisciplinary approach are essential.
- Laparoscopic techniques are increasingly utilized; familial and multiple tumors present management challenges.
Aims:
To evaluate the current investigation and management of phaeochromocytoma.
Methods:
Retrospective analysis of patients who underwent surgical excision of phaeochromocytoma in the Department of Endocrine Surgery at the University Hospital of Wales, Cardiff. Forty-seven patients (24 female and 23 males) were studied. Preoperative diagnosis was established by measurement of urinary catecholamines (HMMA, metadrenalines, and fractionated catecholamines). Tumour localisation was achieved by using ultrasound, CT, MRI and MIBG scintigraphy. Preoperative medical preparation and control of hypertension was achieved in the majority of cases by alpha adrenergic blockade with phenoxybenzamine and the beta blocker propranol. Surgery was performed by a variety of approaches which included laparotomy, posterior extraperitoneal and laparoscopic methods. All patients were followed up post-operatively in a surgical endocrine clinic.
Results:
Seventy percent of patients presented with hypertension but only 21.3% gave a history of paroxsmal hypertension. CT scanning and MRI proved to be the most sensitive localisation investigations. Excellent preoperative control of hypertension was achieved with alpha adrenergic blockade but induction of anaesthesia, rather than tumour handling was noted to be associated with most hypertensive surges of blood pressure. There was a zero 30 day post-operative mortality but 10 complications of surgery occurred in 8 patients (21.3%). Cure of hypertension was achieved in 80% of patients. Attempts to perform cortex sparing procedures in patients with familial disease and multiple tumours was not successful in the long term.
Conclusions:
Surgical excision of phaeochromocytoma is a procedure, which can be performed with zero mortality and a low morbidity resulting in a high cure rate for hypertension. Adequate preoperative pharmacological control of hypertension is mandatory. Localisation techniques permit a focussed approach with increasing use of laparoscopy. Those patients with familial disease and those with multiple tumours pose particular management challenges. For an optimum and satisfactory outcome a planned multidisciplinary approach is required.
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