Open AccessLaparoscopic resection of aortocaval paraganglioma diagnosed by serial increase in urinary metanephrines after bilateral adrenalectomy in a patient with multiple endocrine neoplasia type 2A
Author(s) -
Miyata Yushi,
Hatano Koji,
Okuno Yosuke,
Ujike Takeshi,
Fukuhara Shinichiro,
Uemura Motohide,
Kiuchi Hiroshi,
Imamura Ryoichi,
Otsuki Michio,
omura Norio
Publication year - 2021
Publication title -
iju case reports
Language(s) - English
Resource type - Journals
ISSN - 2577-171X
DOI - 10.1002/iju5.12345
Subject(s) - metanephrines , metanephrine , medicine , multiple endocrine neoplasia , pheochromocytoma , normetanephrine , paraganglioma , adrenalectomy , urology , urinary system , radiology , biochemistry , chemistry , gene
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 ( RET 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. 123 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.