Paraganglioma from Organ of Zuckerkandl Associated with Succinate Dehydrogenase Complex Mutation: A Rare Case Report

Aim and background: Here, we present a rare case report of abdominal paraganglioma with succinate dehydrogenase complex (SDHC) mutation originating from the Organ of Zuckerkandl (OZ).Case description: A 59-year-old female with a 12-year history of hypertension and four episodes of hypertensive urgency, along with symptoms of sympathetic overactivity like headache, perspiration, palpitations, and vomiting.The biochemical profile showed elevated normetanephrine, metanephrine, and 3MT.CT abdomen and Ga DOTANOC PET showed a functional tumor originating from the OZ.She underwent laparotomy excision of the tumor.Preoperative optimization of blood pressure with alpha blockade was given to avoid inadvertent cardiovascular complications.Intraoperatively, persistent bleeding and hypotension were encountered.A 7 5 cm lobulated tumor was seen arising from the OZ.The tumor was infiltrating the superficial planes of the lower aorta, left iliac artery, and prevertebral fascia.It was highly vascular, and most of the feeders were directly from the aorta.The postoperative period was uneventful.The biopsy was reported to be a moderately differentiated paraganglioma with SDHC mutation.Our case was followed up for 2 years and had no features suggestive of recurrence or metastasis.Conclusion: A rare case of paraganglioma from OZ with SDHC mutation, which was operated on after appropriate perioperative optimization.Clinical significance: Hypotension and hypoglycemia are two of the most anticipated postoperative complications, which should be meticulously monitored.Succinate dehydrogenase complex mutations are rare, as compared to succinate dehydrogenase complex subunit B (SDHB) and succinate dehydrogenase complex subunit D (SDHD) mutations.If at all present, they are often observed in the head and neck region.Familial paraganglioma tends to be metastatic and has an increased risk of recurrence; hence, they need to be closely followed up.Due to the rarity of the case and for its mutation and location, this case capsule will be instrumental in our clinical understanding.