Laparoscopic Resection of Aortocaval Paraganglioma Diagnosed by Serial Increase in Urinary Metanephrines After Bilateral Adrenalectomy in a Patient with Multiple Endocrine Neoplasia Type 2A
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Introduction: Although bilateral pheochromocytoma is prevalent in patients with multiple endocrine neoplasia type 2, extra-adrenal tumors rarely occur in the aortocaval area.
Case Presentation: A 35-year-old man with multiple endocrine neoplasia type 2A ( codon Cys634Arg mutation) underwent bilateral adrenalectomy for metachronous pheochromocytoma. After bilateral adrenalectomy, urinary metanephrines decreased below the measurement sensitivity. The levels of urinary metanephrines were serially elevating to a peak of 187 ng/mgCr during the 11-year follow-up period; however, urinary normetanephrine levels remained almost stable. I-metaiodobenzylguanidine single-photon emission computed tomography revealed abnormal accumulation with a mass of 25 × 18 mm in diameter in the aortocaval space cranially to the renal vessels. The extra-adrenal paraganglioma was successfully resected using transperitoneal laparoscopic surgery.
Conclusion: The serial increase in urinary metanephrine levels was useful for the detection of the recurrent tumor in a patient who had undergone bilateral adrenalectomy.
Utility of Retroperitoneal Laparoscopic Surgery for Retroperitoneal Tumors.
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