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The clinical spectrum of phaeochromocytoma: analysis of 115 patients
Author(s) -
Safwat Ahmed S.,
Bissada Nabil K.,
Seyam Raouf M.,
Al Sobhi Saif,
Hanash Kamal A.
Publication year - 2008
Publication title -
bju international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.773
H-Index - 148
eISSN - 1464-410X
pISSN - 1464-4096
DOI - 10.1111/j.1464-410x.2008.07430.x
Subject(s) - medicine , pathological , pheochromocytoma , disease , magnetic resonance imaging , radiology , von hippel–lindau disease , presentation (obstetrics) , multiple endocrine neoplasia , pathology , biochemistry , chemistry , gene
OBJECTIVE To analyse the presentation, manifestations and outcome in consecutive patients with phaeochromocytoma, as this disease has a wide range of pathological and clinical expressions. PATIENTS AND METHODS The records of 115 patients with phaeochromocytoma were analysed retrospectively, recording the patients’ age, sex, presenting symptoms and clinical signs, chemical, radiological and pathological findings and associated conditions. RESULTS Of the 115 patients, 90 had adrenal tumours, 18 extra‐adrenal and seven combined adrenal and extra‐adrenal tumours. Ten patients had malignant and 105 had benign phaeochromocytoma. Eighty‐six patients had sporadic and 29 had familial phaeochromocytoma, comprising eight with von Hippel‐Lindau (VHL) disease, 17 with multiple endocrine neoplasia type II (MEN II) and four with von Recklinghausen disease. Two patients with sporadic phaeochromocytoma had Grave’s disease. Ten patients (8.7%) had malignant phaeochromocytoma, of whom two had MEN II. A pregnant woman required prolonged intensive‐care management before adrenalectomy and lost a fetus. CONCLUSION Phaeochromocytoma is an interesting clinical entity with a wide spectrum of pathological and clinical manifestations. The diagnosis of phaeochromocytoma is confirmed by chemical methods, and located using imaging techniques, with computed tomography, magnetic resonance imaging and 131 I‐meta‐iodobenzyl guanidine radioisotope scanning being the most common. This series reflects the pathological and clinical spectrum of phaeochromocytoma. The presence of other manifestations of familial phaeochromocytoma influenced the presentation and prognosis of these patients.