Metastatic pheochromocytoma in multiple endocrine neoplasia type 2A
D. V. Rebrova,
V. F. Rusakov,
L. M. Krasnov,
E. A. Fedorov,
I. K. Chinchuk,
N. V. Vorokhobina,
S. S. Shikhmagomedov,
A. A. Semenov,
R. A. Chernikov,
I. V. Sleptsov,
G. I. Gavton,
E. N. Imyanitov https://doi.org/10.14341/probl13332
Abstract
Pheochromocytoma (PHEO) currently is considered to be malignant due to metastatic potential. One of the most common familial forms of PHEO is multiple endocrine neoplasia syndrome (MEN) type 2. The penetrance of PHEO in MEN2 syndrome is up to 50% of cases. It may be one- or two-sided, but metastases occur extremely rare. The fact that in majority of cases of MEN2 syndrome the source of distant metastases is medullary thyroid carcinoma (MTC) complicates differential diagnosis in case of PHEO metastasis.
Isolated cases of PHEO with metastases to the lymph nodes, lungs, liver, bones, brain in MEN2 patients were described. In the available literature, we have found a description of 31 cases of metastatic PHEO in MEN2 syndrome. The available data of those cases is presented as a table in the article.
We present a description of a 40-year-old woman with MEN2A syndrome (mutation of the RET proto-oncogene p.Cys634Tyr), with a history of twice-performed surgical treatment of MTC, with daily crises of arterial hypertension accompanied by vegetative symptoms, with a giant bilateral PHEO (up to 200 m on the right and up to 150 mm on the left) with synchronous large metastasis (up to 50 mm) into the pubic bone with the destruction. The patient underwent several surgeries: bilateral adrenalectomy, then a bilateral revision of the neck, removal of the right upper and right lower parathyroid glands, residual thyroid tissue, then resection of the right pubic bone with a tumor.
About the Authors
D. V. Rebrova
Saint Petersburg State University, Saint Petersburg State University Hospital
Russian Federation
Russian Federation
Dina V. Rebrova, MD, PhD
154 Fontanka river embankment, 190103, Saint Petersburg
V. F. Rusakov
Saint Petersburg State University, Saint Petersburg State University Hospital
Russian Federation
Russian Federation
Vladimir F. Rusakov, MD, PhD
St. Petersburg
L. M. Krasnov
Saint Petersburg State University, Saint Petersburg State University Hospital
Russian Federation
Russian Federation
Leonid M. Krasnov, MD, PhD
St. Petersburg
E. A. Fedorov
Saint Petersburg State University, Saint Petersburg State University Hospital
Russian Federation
Russian Federation
Elisey A.Fedorov, MD, PhD
St. Petersburg
I. K. Chinchuk
Saint Petersburg State University, Saint Petersburg State University Hospital
Russian Federation
Russian Federation
Igor K. Chinchuk, MD, PhD
St. Petersburg
N. V. Vorokhobina
North-Western State Medical University n.a. I.I. Mechnikov
Russian Federation
Russian Federation
Natalya V. Vorokhobina, MD, PhD, Prof.
St. Petersburg
S. S. Shikhmagomedov
Saint Petersburg State University, Saint Petersburg State University Hospital
Russian Federation
Russian Federation
Shamil S. Shikhmagomedov, MD
St. Petersburg
A. A. Semenov
Saint Petersburg State University, Saint Petersburg State University Hospital
Russian Federation
Russian Federation
Arseny A. Semenov, MD, PhD
St. Petersburg
R. A. Chernikov
Saint Petersburg State University, Saint Petersburg State University Hospital
Russian Federation
Russian Federation
Roman A. Chernikov, MD, PhD
St. Petersburg
I. V. Sleptsov
Saint Petersburg State University, Saint Petersburg State University Hospital
Ilya V. Sleptsov, MD, PhD
St. Petersburg
G. I. Gavton
National Medical Research Center of Oncology n.a. N.N. Petrov
Russian Federation
Russian Federation
Georgy I. Gavton, MD, DSc, Prof.
St. Petersburg
E. N. Imyanitov
National Medical Research Center of Oncology n.a. N.N. Petrov
Russian Federation
Russian Federation
Evgeny N. Imyanitov, MD, PhD, Prof., Corresponding Member of RAS
St. Petersburg
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Supplementary files
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1. Figure 1. Computed tomography of the abdominal organs with contrast: a — arterial phase, coronary plane; b — venous phase, axial plane; c — arterial phase, sagittal plane. Giant multiple pheochromocytomas of both adrenal glands are marked with arrows. | |
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2. Figure 2. Computed tomography of the pelvic organs with contrast: a — arterial phase, bone window, axial plane; b — delayed phase, soft tissue window, axial plane. Arrows indicate pheochromocytoma metastasis to the pubic bone. | |
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For citations:
Rebrova D.V., Rusakov V.F., Krasnov L.M., Fedorov E.A., Chinchuk I.K., Vorokhobina N.V., Shikhmagomedov S.S., Semenov A.A., Chernikov R.A., Sleptsov I.V., Gavton G.I., Imyanitov E.N. Metastatic pheochromocytoma in multiple endocrine neoplasia type 2A. Problems of Endocrinology. 2024;70(6):35-44. (In Russ.) https://doi.org/10.14341/probl13332
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