Endocrine Abstracts

Introduction: Malignant phaeochromocytomas are rare tumours, developed at the medulla and paraganglia. Their diagnosis is established by the presence of metastases of organs devoid of chromaffin tissue or by the appearance of neoplastic recurrence. They are characterized by a morbidity and mortality due to the effects of uncontrolled and important hypersecretion cathécolaminergique and resistance to conventional cancer treatments.

Aim: Report the observations of seven cases observed in 26 years.

Materials and methods: Seven patients with malignant phaeochromocytomas were treated in our department. Three were males and four females and their ages ranged between 17 and 45 years old. The circumstances discoveries were adrenergic signs and severe hypertension in all cases. Hormonal balance showed very high levels of metanephrine: 20±1.4 (16–22) and CT+MRI showed a large and characteristic adrenal mass: 12.6±1.2 cm (14–9.6). Malignancy was confirmed by the presence of metastasis at diagnosis in five cases and in the developments in the rest of the cases. Evolution was marked a year and a half after on average (after surgery and chemotherapy) by a normalisation of blood pressure, methoxylated derivatives in five patients. Two deaths occurred after the first course of chemotherapy.

Discussion and conclusion: Surgery of malignant phaeochromocytoma, even at metastatic stage increases survival. Chemo radiotherapy and MIBG are part of the therapeutic arsenal. The management of these patients requires a multidisciplinary follow up (surgeon, oncologist, endocrinologist, histopathologist, and geneticist).