{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,1,24]],"date-time":"2025-01-24T05:16:52Z","timestamp":1737695812450,"version":"3.33.0"},"reference-count":34,"publisher":"Endocrinology Research Centre","issue":"6","license":[{"start":{"date-parts":[[2024,2,26]],"date-time":"2024-02-26T00:00:00Z","timestamp":1708905600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.probl-endojournals.ru\/jour\/about\/editorialPolicies#openAccessPolicy"}],"content-domain":{"domain":["www.probl-endojournals.ru"],"crossmark-restriction":true},"short-container-title":["Probl Endokrinol (Mosk)"],"abstract":"<jats:p>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\u00a0case of PHEO metastasis.Isolated cases of PHEO with metastases to the lymph nodes, lungs, liver, bones, brain in MEN2 patients were described. In\u00a0the\u00a0available 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.<\/jats:p>","DOI":"10.14341\/probl13332","type":"journal-article","created":{"date-parts":[[2025,1,23]],"date-time":"2025-01-23T10:25:50Z","timestamp":1737627950000},"page":"35-44","update-policy":"https:\/\/doi.org\/10.14341\/crossmarkpolicy2018","source":"Crossref","is-referenced-by-count":0,"title":["Metastatic pheochromocytoma in multiple endocrine neoplasia type 2A"],"prefix":"10.14341","volume":"70","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7840-4174","authenticated-orcid":true,"given":"D. V.","family":"Rebrova","sequence":"first","affiliation":[{"name":"Saint Petersburg State University, Saint Petersburg State University Hospital"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6807-778X","authenticated-orcid":true,"given":"V. F.","family":"Rusakov","sequence":"additional","affiliation":[{"name":"Saint Petersburg State University, Saint Petersburg State University Hospital"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4449-0251","authenticated-orcid":true,"given":"L. M.","family":"Krasnov","sequence":"additional","affiliation":[{"name":"Saint Petersburg State University, Saint Petersburg State University Hospital"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1294-811X","authenticated-orcid":true,"given":"E. A.","family":"Fedorov","sequence":"additional","affiliation":[{"name":"Saint Petersburg State University, Saint Petersburg State University Hospital"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6986-1034","authenticated-orcid":true,"given":"I. K.","family":"Chinchuk","sequence":"additional","affiliation":[{"name":"Saint Petersburg State University, Saint Petersburg State University Hospital"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9574-105X","authenticated-orcid":true,"given":"N. V.","family":"Vorokhobina","sequence":"additional","affiliation":[{"name":"North-Western State Medical University n.a. I.I. Mechnikov"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3876-6306","authenticated-orcid":true,"given":"S. S.","family":"Shikhmagomedov","sequence":"additional","affiliation":[{"name":"Saint Petersburg State University, Saint Petersburg State University Hospital"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6760-0025","authenticated-orcid":true,"given":"A. A.","family":"Semenov","sequence":"additional","affiliation":[{"name":"Saint Petersburg State University, Saint Petersburg State University Hospital"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3001-664X","authenticated-orcid":true,"given":"R. A.","family":"Chernikov","sequence":"additional","affiliation":[{"name":"Saint Petersburg State University, Saint Petersburg State University Hospital"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1903-5081","authenticated-orcid":true,"given":"I. V.","family":"Sleptsov","sequence":"additional","affiliation":[{"name":"Saint Petersburg State University, Saint Petersburg State University Hospital"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3172-2201","authenticated-orcid":true,"given":"G. I.","family":"Gavton","sequence":"additional","affiliation":[{"name":"National Medical Research Center of Oncology n.a. N.N. Petrov"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4529-7891","authenticated-orcid":true,"given":"E. N.","family":"Imyanitov","sequence":"additional","affiliation":[{"name":"National Medical Research Center of Oncology n.a. N.N. Petrov"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"5458","published-online":{"date-parts":[[2024,2,26]]},"reference":[{"key":"ref1","unstructured":"Lloyd RV, Osamura RY, Kloppel G, Rosai J, editors (2017). WHO Classification of Tumours of Endocrine Organs (4th edition). 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