Rare case of pheochromocytoma with calcitonin hypersecretion


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Abstract

Background. Pheochromocytoma, or adrenal paraganglioma, is a neuroendocrine tumor from the adrenal medulla cells. In the vast majority of cases, pheochromocytoma is a hormonally active tumor with hypersecretion of catecholamines: adrenaline, norepinephrine, dopamine. However, due to the neuroendocrine origin of the tumor, extremely rare cases of ectopic hypersecretion of other peptide hormones, such as adrenocorticotropic hormone, somatostatin, neuropeptide Y., methenkephalin, and vasoactive interstitial peptide, have also been described. In the available literature in Russian and English, we found only 4 cases describing the secretion of calcitonin by pheochromocytoma; the earliest of them was published in 1977. Description of the clinical case. In a 38-year-old patient with a mixed course of arterial hypertension, with sympathoadrenal crises with autonomic symptoms occuring against a background of constantly elevated blood pressure, a large tumor of the right adrenal gland with diameter up to 78 mm was revealed during examination. Detailed radiation characteristics of the tumor according to the data of ultrasound, computed tomography (CT), magnetic resonance imaging, positron emission tomography/CT with 18-fluorodeoxyglucose are presented. According to the results of laboratory tests, catecholamine overproduction by the tumor was detected. In addition, a repeated increase in the blood calcitonin level was revealed, which, in the absence of a genetic study of the RET-protooncogene, did not allow to exclude multiple endocrine neoplasia type 2 (MEN2). After surgical treatment, the blood calcitonin level decreased to undetectable value, which suggested calcitonin overproduction by pheochromocytoma. This hypothesis was confirmed by the results of histological and immunohistochemical studies. Conclusion. Ectopic production of calcitonin by pheochromocytoma is not typical and is extremely rare, however, its development is possible, which should be taken into account in case of its increased level in patients with pheochromocytoma. Preoperative genetic study of patients with pheochromocytoma in such cases greatly facilitates the diagnostic search, significantly reducing the risk of developing medullary thyroid carcinoma in patients without hereditary MEN2.

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About the authors

Dina V. Rebrova

Saint Petersburg State University; Saint Petersburg State University Clinic

Email: endocrinology@list.ru
Cand. Sci. (Med.) 154 Fontanka river embankment, St. Petersburg 198103, Russian Federation

V. F Rusakov

Saint Petersburg State University; Saint Petersburg State University Clinic

St. Petersburg, Russia

E. A Fedorov

Saint Petersburg State University; Saint Petersburg State University Clinic

St. Petersburg, Russia

L. M Krasnov

Saint Petersburg State University; Saint Petersburg State University Clinic

St. Petersburg, Russia

N. V Vorokhobina

North-Western State Medical University n.a. I.I. Mechnikov

St. Petersburg, Russia

Sh. Sh Shikhmagomedov

Saint Petersburg State University; Saint Petersburg State University Clinic

St. Petersburg, Russia

S. L Vorobyev

National Center for Clinical Morphological Diagnostics

St. Petersburg, Russia

I. V Sleptsov

Saint Petersburg State University; Saint Petersburg State University Clinic

St. Petersburg, Russia

R. A Chernikov

Saint Petersburg State University; Saint Petersburg State University Clinic

St. Petersburg, Russia

T. V Savelyeva

Saint Petersburg State University; Saint Petersburg State University Clinic

St. Petersburg, Russia

A. A Semenov

Saint Petersburg State University; Saint Petersburg State University Clinic

St. Petersburg, Russia

I. K Chinchuk

Saint Petersburg State University; Saint Petersburg State University Clinic

St. Petersburg, Russia

I. V Sablin

Saint Petersburg State University; Saint Petersburg State University Clinic

St. Petersburg, Russia

T. S Pridvizhkina

Saint Petersburg State University; Saint Petersburg State University Clinic

St. Petersburg, Russia

Yu. N Fedotov

Saint Petersburg State University; Saint Petersburg State University Clinic

St. Petersburg, Russia

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