Endocrine Abstracts

Background: A 27-years-old women affected by recurrent pheocromocytoma/paragangliomas. We describe the evolution of disease and the surgical approach. The patient underwent a two-steps bilateral adrenalectomy for pheocromocytoma. At 15 years she underwent a left adrenalectomy. At 19 a right adrenalectomy+removal of paracaval paraganglioma were performed. After 8 years of good health she experienced again headache, tachycardia and hypertension not responsive to therapy. A diagnosis of recurrent disease was made.

Methods: Nor-colesterol and MIBG scintigraphy were performed and stated respectively for a hyperfixation in the left adrenal bed and two spot of hyperfixation on para-caval and paraortic area (Default 1). The last two spots were concurdant with a MRI imaging. The patient underwent surgical exploration using an intraoperative radionuclear scanning probe. A target/background ratio (T/B ratio) was calculated. All lesion with a T/B ratio level more than 1.2 (239 mBq) were considered suspected.

Results: Three lesions with a value respectively of 270, 2020 and 2600 mBq were removed. Pathological report classified the latter two lesions as paragangliomas. At 5 years follow-up the patient has no biological evidence of disease and does not need any replacement therapy.

Conclusions: The easier finding of the lesions by radioguidance allowed the surgeon to perform a focused approach, obtaining a radical procedure, to achieve a successful treatment of a recurrent disease. Sparing the remnant of cortical adrneal tissue on the left side also had a great impact in the quality of life preserving the patient from life-long steroid replacement therapy (Figure 1).

Figure 1 MIBG Scan showing a recidive of paraganglioma

Declaration of interest: The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research project.

Funding: This research did not receive any specific grant from any funding agency in the public, commercial or not-for-profit sector.