Adrenal masses in Gardner syndrome: difficulties in diagnosis and treatment (case report and brief review)
Gardner syndrome is an autosomal dominant disorder characterized by colorectal polyposis in combination with extraintestinal manifestations such as desmoid tumors, osteomas, and dental anomalies. Rare extraintestinal manifestations of Gardner syndrome include adrenal masses, which have been reported in 7% of patients.E.S. Drachuk, K.E. Gaidaichuk, K.V. Ivaschenko, N.V. Pachuashvili, L.S. Urusova, M.V. Godzenko, N.V. Tarbaeva, E.A. Pigarova, L.K. Dzeranova, N.M. Platonova
The article presents a case of a 33-year-old woman with Gardner syndrome and a history of left-sided adrenalectomy for an adrenocortical adenoma with a diameter of more than 6 cm. She was referred to the Institute of Clinical Endocrinology of the National Medical Research Center of Endocrinology to clarify the indications for surgical treatment of multiple lesions of the right adrenal gland.
Review of histological preparations and immunohistochemical examination using modern algorithms for assessing oncocytic tumors of the adrenal gland allowed to exclude adrenocortical cancer in the patient. Taking into account the absence of negative dynamics in the size and structure of space-occupying lesions of the right adrenal gland, the absence of signs of their hormonal activity, no absolute indications for surgical treatment of space-occupying lesions of the right adrenal gland were identified.
Despite the prevalence of adrenal tumors in patients with Gardner syndrome, their clinical significance is limited. Management of such patients requires a multidisciplinary approach involving endocrinologists, pathologists and endocrine surgeons to prevent irrational treatment.
Keywords
Gardner’s syndrome
adrenal adenoma
adrenocortical cancer
adenocarcinoma
oncocytic tumor
polyposis
desmoid tumors
About the Authors
Elizaveta S. Drachuk, Resident, National Medical Research Center of Endocrinology, Moscow, Russia; sdr68@mail.ru, ORCID:https://orcid.org/0009-0004-4524-3142
Konstantin E. Gaidaichuk, Resident, National Medical Research Center of Endocrinology, Moscow, Russia; gaidaikon@yandex.ru, ORCID:
https://orcid.org/0009-0006-6107-4494, eLibrary SPIN: 3384-1038
Ksenia V. Ivaschenko, Postgraduate Student, National Medical Research Center of Endocrinology, Moscow, Russia; kseniya223@mail.ru, ORCID: https://orcid.org/0000-0002-0786-7809, eLibrary SPIN: 4526-4222 (corresponding author)
Nano V. Pachuashvili, Cand. Sci. (Med.), National Medical Research Center of Endocrinology, Moscow, Russia; npachuashvili@bk.ru, ORCID: https://orcid.org/0000-0002-8136-0117, eLibrary SPIN: 3477-8994
Liliya S. Urusova, Dr. Sci. (Med.), National Medical Research Center of Endocrinology, Moscow, Russia; Urusova.Liliya@endocrincentr.ru, ORCID: https://orcid.org/0000-0001-6891-0009, eLibrary SPIN: 5151-3675
Maria V. Godzenko, National Medical Research Center of Endocrinology, Moscow, Russia; godzenko.mariya@endocrincentr.ru, ORCID:
https://orcid.org/0000-0001-8783-008X, SPIN-code: 6012-4491
Natalia V. Tarbaeva, Cand. Sci. (Med.), National Medical Research Center of Endocrinology, Moscow, Russia; ntarbaeva@inbox.ru, ORCID:
https://orcid.org/0000-0001-7965-9454, eLibrary SPIN: 5808-8065
Ekaterina A. Pigarova, Dr. Sci. (Med.), National Medical Research Center of Endocrinology, Moscow, Russia; kpigarova@gmail.com, ORCID:
https://orcid.org/0000-0001-6539-466X, eLibrary SPIN: 6912-6331
Larisa K. Dzeranova, Dr. Sci. (Med.), National Medical Research Center of Endocrinology, Moscow, Russia; dzeranovalk@yandex.ru, ORCID:
https://orcid.org/0000-0002-0327-4619, eLibrary SPIN: 2958-5555
Nadezhda M. Platonova, Dr. Sci. (Med.), National Medical Research Center of Endocrinology, Moscow, Russia; doc-platonova@inbox.ru, ORCID: https://orcid.org/0000-0001-6388-1544, eLibrary SPIN: 4053-3033