Diagnosis and Treatment > Medication
Search for other papers by Valeria de Miguel in
Google Scholar
PubMed
Search for other papers by Andrea Paissan in
Google Scholar
PubMed
Search for other papers by Patricio García Marchiñena in
Google Scholar
PubMed
Search for other papers by Alberto Jurado in
Google Scholar
PubMed
Search for other papers by Mariana Isola in
Google Scholar
PubMed
Search for other papers by José Alfie in
Google Scholar
PubMed
Search for other papers by Patricia Fainstein-Day in
Google Scholar
PubMed
Summary
We present the case of a 25-year-old male with a history of neurofibromatosis type 1 and bilateral pheochromocytoma 4 years after kidney transplantation that was successfully treated with simultaneous bilateral posterior retroperitoneoscopic adrenalectomy.
Learning points:
-
Hypertensive patients with NF1 should always be screened for pheochromocytoma.
-
Pheochromocytoma is rarely associated with transplantation, but it must be ruled out in patients with genetic susceptibility.
-
Posterior retroperitoneoscopic adrenalectomy (PRA) allows more direct access to the adrenal glands, especially in patients with previous abdominal surgeries.
Search for other papers by Omayma Elshafie in
Google Scholar
PubMed
Search for other papers by Yahya Al Badaai in
Google Scholar
PubMed
Search for other papers by Khalifa Alwahaibi in
Google Scholar
PubMed
Search for other papers by Asim Qureshi in
Google Scholar
PubMed
Search for other papers by Samir Hussein in
Google Scholar
PubMed
Search for other papers by Faisal Al Azzri in
Google Scholar
PubMed
Search for other papers by Ali Almamari in
Google Scholar
PubMed
Search for other papers by Nicholas Woodhouse in
Google Scholar
PubMed
Summary
A 48-year-old hypertensive and diabetic patient presented with a 10-year history of progressive right facial pain, tinnitus, hearing loss, sweating, and palpitations. Investigations revealed a 5.6 cm vascular tumor at the carotid bifurcation. Her blood pressure (BP) was 170/110, on lisinopril 20 mg od and amlodipine 10 mg od and 100 U of insulin daily. A catecholamine-secreting carotid body paraganglioma (CSCBP) was suspected; the diagnosis was confirmed biochemically by determining plasma norepinephrine (NE) level, 89 000 pmol/l, and chromogranin A (CgA) level, 279 μg/l. Meta-iodobenzylguanidine and octreotide scanning confirmed a single tumor in the neck. A week after giving the patient a trial of octreotide 100 μg 8 h, the NE level dropped progressively from 50 000 to 25 000 pmol/l and CgA from 279 to 25 μg/l. Treatment was therefore continued with labetalol 200 mg twice daily (bid) and long-acting octreotide-LA initially using 40 mg/month and later increasing to 80 mg/month. On this dose and with a reduced labetalol intake of 100 mg bid, BP was maintained at 130/70 and her symptoms resolved completely. CgA levels returned to normal in the first week and these were maintained throughout the 3 month treatment period. During tumor resection, there were minimal BP fluctuations during the 10 h procedure. We conclude that short-term high-dose octreotide-LA might prove valuable in the preoperative management of catecholamine-secreting tumors. To the best of our knowledge, this is the first report on the successful use of octreotide in a CSCBP.
Learning points
-
The value of octreotide scanning in the localization of extra-adrenal pheochromocytoma.
-
Control of catecholamine secretion using high-dose octreotide.
-
This is a report of a rare cause of secondary diabetes and hypertension.
