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Pheochromocytoma — analysis of 15 consecutive cases from one centre
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Abstract
Introduction: Pheochromocytoma is a rare tumour, but one of great clinical importance as a risk factor of malignancy, cardiovascular diseases and sudden death.
Material and methods: 15 consecutive patients (eight women and seven men) were hospitalised and submitted for adrenalectomy with pheochromocytoma confirmed by histopathologic examination. Adrenalectomies were performed laparoscopically in 14 cases (93.3%): in nine by the retroperitoneal posterior mode and in five by the transperitoneal lateral approach.
Results: Molecular-genetic examination of VHL, RET, SDHB, SDHC and SDHD genes revealed inherited predisposition for PHEO in three of 15 patients (20%): RET mutations typical for MEN 2a in two patients and VHL mutation in one patient. Disturbances of the carbohydrate metabolism occurred in nine patients (60%). Ten patients (66%) reported paroxysmal symptoms. In all cases, with the exception of a von Hippel-Lindau patient, density of tumours exceeded 20 HU. In all studied patients, urine concentration of normetanephrines exceeded their normal range and greatly prevailed over metanephrines values, which were increased in six of them (40%).
Conclusions: Urine metoxycatecholamines and increased tissue density are sufficient in pheochromocytoma detection. However, taking into account clinical and supplemental biochemical data may be helpful in the diagnostic process. Laparoscopic adrenalectomy is a fully sufficient and safe method of pheochromocytoma excision. (Endokrynol Pol 2013; 64 (3): 192–196)
Abstract
Introduction: Pheochromocytoma is a rare tumour, but one of great clinical importance as a risk factor of malignancy, cardiovascular diseases and sudden death.
Material and methods: 15 consecutive patients (eight women and seven men) were hospitalised and submitted for adrenalectomy with pheochromocytoma confirmed by histopathologic examination. Adrenalectomies were performed laparoscopically in 14 cases (93.3%): in nine by the retroperitoneal posterior mode and in five by the transperitoneal lateral approach.
Results: Molecular-genetic examination of VHL, RET, SDHB, SDHC and SDHD genes revealed inherited predisposition for PHEO in three of 15 patients (20%): RET mutations typical for MEN 2a in two patients and VHL mutation in one patient. Disturbances of the carbohydrate metabolism occurred in nine patients (60%). Ten patients (66%) reported paroxysmal symptoms. In all cases, with the exception of a von Hippel-Lindau patient, density of tumours exceeded 20 HU. In all studied patients, urine concentration of normetanephrines exceeded their normal range and greatly prevailed over metanephrines values, which were increased in six of them (40%).
Conclusions: Urine metoxycatecholamines and increased tissue density are sufficient in pheochromocytoma detection. However, taking into account clinical and supplemental biochemical data may be helpful in the diagnostic process. Laparoscopic adrenalectomy is a fully sufficient and safe method of pheochromocytoma excision. (Endokrynol Pol 2013; 64 (3): 192–196)
Keywords
metoxycatecholamines; adrenal tumour; tissue density; hypertension
About this articleTitle
Pheochromocytoma — analysis of 15 consecutive cases from one centre
Journal
Issue
Article type
Original paper
Pages
192-196
Published online
2013-07-01
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529
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1678
Bibliographic record
Endokrynol Pol 2013;64(3):192-196.
Keywords
metoxycatecholamines
adrenal tumour
tissue density
hypertension
Authors
Janusz Myśliwiec
Katarzyna Siewko
Łukasz Żukowski
Piotr Myśliwiec
Maria Kościuszk
Anna Popławska
Małgorzata Szelachowska
Jacek Dadan
Maria Górska
