Management dilemmas of retroperitoneal paraganglioma: report of a case

A 17-year-old boy presented to emergency department with abdominal pain in the left upper quadrant along with a palpable mass. A CT scan revealed a 10 x 8.5 cm mass in the retroperitoneal area, suggestive of a sarcoma. The patient underwent surgical resection of the lesion, the histological examination of which confirmed a paraganglioma. An 131I-Metaiodobenzylguanidine (MIBG) scan excluded distant metastasis. Paragangliomas are rare tumours of the neuroendocrine system and certain considerations are pivotal in the management of these tumours. Urinary and plasma catecholamines measurement along with CT, MRI, and MIBG scanning facilitate diagnosis, localisation and characterisation of these tumours. Surgical excision is the treatment of choice for both primary and recurrent tumours. Unfortunately, histological characterisation for malignancy is limited and these tumours are considered malignant upon development of metastasis or local recurrence. Inexistence of histological criteria for malignancy means a long-term surveillance is the norm in these patients.