Phaeochromocytoma--views on current management. 2003

D T Williams, and S Dann, and M H Wheeler
Department of Endocrine Surgery, University Hospital of Wales, Heath Park, CF14 4XW, Cardiff, UK.

OBJECTIVE 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.

UI MeSH Term Description Entries
D006973 Hypertension Persistently high systemic arterial BLOOD PRESSURE. Based on multiple readings (BLOOD PRESSURE DETERMINATION), hypertension is currently defined as when SYSTOLIC PRESSURE is consistently greater than 140 mm Hg or when DIASTOLIC PRESSURE is consistently 90 mm Hg or more. Blood Pressure, High,Blood Pressures, High,High Blood Pressure,High Blood Pressures
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009364 Neoplasm Recurrence, Local The local recurrence of a neoplasm following treatment. It arises from microscopic cells of the original neoplasm that have escaped therapeutic intervention and later become clinically visible at the original site. Local Neoplasm Recurrence,Local Neoplasm Recurrences,Locoregional Neoplasm Recurrence,Neoplasm Recurrence, Locoregional,Neoplasm Recurrences, Local,Recurrence, Local Neoplasm,Recurrence, Locoregional Neoplasm,Recurrences, Local Neoplasm,Locoregional Neoplasm Recurrences,Neoplasm Recurrences, Locoregional,Recurrences, Locoregional Neoplasm
D010673 Pheochromocytoma A usually benign, well-encapsulated, lobular, vascular tumor of chromaffin tissue of the ADRENAL MEDULLA or sympathetic paraganglia. The cardinal symptom, reflecting the increased secretion of EPINEPHRINE and NOREPINEPHRINE, is HYPERTENSION, which may be persistent or intermittent. During severe attacks, there may be HEADACHE; SWEATING, palpitation, apprehension, TREMOR; PALLOR or FLUSHING of the face, NAUSEA and VOMITING, pain in the CHEST and ABDOMEN, and paresthesias of the extremities. The incidence of malignancy is as low as 5% but the pathologic distinction between benign and malignant pheochromocytomas is not clear. (Dorland, 27th ed; DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1298) Pheochromocytoma, Extra-Adrenal,Extra-Adrenal Pheochromocytoma,Extra-Adrenal Pheochromocytomas,Pheochromocytoma, Extra Adrenal,Pheochromocytomas,Pheochromocytomas, Extra-Adrenal
D011247 Pregnancy The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH. Gestation,Pregnancies
D011252 Pregnancy Complications, Neoplastic The co-occurrence of pregnancy and NEOPLASMS. The neoplastic disease may precede or follow FERTILIZATION. Complications, Neoplastic Pregnancy,Neoplastic Pregnancy Complications,Pregnancy, Neoplastic Complications,Complication, Neoplastic Pregnancy,Neoplastic Pregnancy Complication,Pregnancies, Neoplastic Complications,Pregnancy Complication, Neoplastic
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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