Treatment of metastatic pheochromocytoma and paraganglioma

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Pheochromocytoma (PCC) and paraganglioma (PGL) are rare neuroendocrine tumors originating from chromaffin cells of the adrenal medulla or extra-adrenal paraganglia. All PCCs/PGLs have metastatic potential, phenotypically realized in no more than 40% of cases. Due to the lack of validated criteria for the aggressive clinical course and prognostic scales for PCCs/PGLs, they are currently considered «malignant» tumors potentially capable of metastasizing, requiring long-term dynamic observation. The diagnosis of metastatic PCC/PGL can only be made based on the identification of metastatic foci in organs and tissues that normally do not contain chromaffin cells. The treatment of metastatic PCC and PGL is primarily based on radionuclide and drug therapy. This article provides a literature review on the treatment of metastatic PCC and PGL, detailing modern possibilities of radioreceptor and antitumor drug therapy

Keywords:

pheochromocytoma

paraganglioma

chromaffin cells

sunitinib

temozolomide

Authors:

Kolomeytseva A.A.

P.A. Herzen Moscow Oncology Research Institute — Branch of the National Medical Radiology Research Center of the Ministry of Health of Russia

ORCID: 0000-0002-6762-9511

Beltsevich D.G.

National Medical Research Center of Endocrinology

ORCID: 0000-0001-7098-4584

Gurevich L.E.

M.F. Vladimirsky Moscow Regional Research and Clinical Institute (MONIKI)

ORCID: 0000-0002-9731-3649

Roslyakova A.A.

National Medical Research Center of Endocrinology of the Ministry of Health of Russia

ORCID: 0000-0003-1857-5083

Sedova M.V.

P.A. Herzen Moscow Oncology Research Institute — Branch of the National Medical Radiology Research Center of the Ministry of Health of Russia

ORCID: 0000-0001-5999-3164

Batov M.A.

P.A. Herzen Moscow Oncology Research Institute — Branch of the National Medical Radiology Research Center of the Ministry of Health of Russia

ORCID: 0000-0002-3780-4358

Fedenko A.A.

P.A. Herzen Moscow Oncology Research Institute — Branch of the National Medical Radiology Research Center of the Ministry of Health of Russia

ORCID: 0000-0003-4927-5585

Received:

13.04.2023

Accepted:

23.06.2023

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