131I-MIBG ON DIAGNOSTIC AND THERAPEUTIC FRONTIERS IN EXTRA-ADRENAL ABDOMINAL PARAGANGLIOMAS: A CASE REPORT WITH MINI REVIEW

Maseeh uz Zaman, Nosheen Fatima, Zafar Sajjad, Unaiza Zaman, Khalil Khan, Gufran Khan, Sadaf Saleem, Zohra Pirwani

Abstract


Paraganglioma are tumors originating from paraganglial system and may be associated with parasympathetic (head, neck and anterior mediastinum tumors) or sympathetic (adrenal or extraadrenal or posterior mediastinal tumor) system. Majority of sympathetic paraganglioma and minority of parasympathetic tumor are functional and 131I or 123I labeled MIBG (Meta Iodio Benzyl Guanidine) is a well established tool with good sensitivity and exceedingly high specificity. We are presenting a case report of a large extradrenal paraganglioma with extensive bony metastases and raised serum Chromogranin-A level. In this case 131-MIBG was not only helpful in revealing the true burden of functional disease but also showed an overwhelming therapeutic response proven on post-therapy MIBG scan and normalization of serum Chromogranin-A level. This case report is followed by a mini-review about pathology, genetic mutation, diagnosis and treatment of focused on extraadrenal paragangliomas. Key words: paraganglioma; extraadrenal; MIBG; MIBG therapy; FDG-PET; Somatostatin receptor imaging

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