Malignant Paraganglioma; a Story of a Long Time Survival

Abstract

Introduction: Pheochromocytoma and paraganglioma are catecholamine secreting tumours. Malignancy is uncommon (approximately 10% for pheochromocytoma and 20% for paraganglioma) and surgery, when possible, is the first line treatment. However, the prognosis is poor because of frequent local recurrence and/or metastases and the lack of specific chemotherapeutic agents. CASE-REPORT: We present the case of a 60 years-old man who, at the age of 48, was diagnosed with paraganglioma. At that specific time, he presented episodes of paroxystic hypertension with spells, the diagnosis of catecholamine excess being confirmed by high levels of VMA (> 50 mg/24 h at 3 determinations). CT was non-diagnostic, but 131I-MIBG scintigraphy showed the presence of a 1.5 cm nodule in the left abdominal paraaortic region. Patient refused surgery and had a satisfactory evolution with medical antihypertensive therapy. 11 years later, he was admitted in the Vascular Surgery Department for acute ischemia of inferior members and a voluminous tumour (5/6 mm) was excised. Pathology confirmed paraganglioma and described some atypical cells, without being able to discriminate between benign or malignant tumour. One year later he was admitted in the Endocrine Department for the reappearance of the adrenergic syndrome (AVM=30.8 mg/24 h). Thoracic and abdominal scan showed sub diaphragmatic and pulmonary tumours. Subsequently, octreotid scintigraphy confirmed local recurrence and metastatic tumours in the lungs. He is proposed for experimental therapy with sunitinib (multitargeted receptor tyrosine kinase inhibitor), which has been reported as an efficient agent in malignant paraganglioma. Discussion: Malignant paraganglioma with distant metastasis have a poor survival rate (11.8% at five years). Although the initial tumour was not resected for 11 years after the diagnosis, our patient is one of the rare cases with indolent form, which permitted a prolonged survival. The new treatment with sunitinib may contribute to a continuation of this favourable evolution