Adrenal masses in Gardner syndrome: difficulties in diagnosis and treatment (case report and brief review)

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Resumo

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.

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.

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Sobre autores

Elizaveta Drachuk

National Medical Research Center of Endocrinology

Email: sdr68@mail.ru
ORCID ID: 0009-0004-4524-3142

Resident

Rússia, Moscow

Konstantin Gaidaichuk

National Medical Research Center of Endocrinology

Email: gaidaikon@yandex.ru
ORCID ID: 0009-0006-6107-4494
Código SPIN: 3384-1038

Resident

Rússia, Moscow

Ksenia Ivaschenko

National Medical Research Center of Endocrinology

Autor responsável pela correspondência
Email: kseniya223@mail.ru
ORCID ID: 0000-0002-0786-7809
Código SPIN: 4526-4222

Postgraduate Student

Rússia, Moscow

Nano Pachuashvili

National Medical Research Center of Endocrinology

Email: npachuashvili@bk.ru
ORCID ID: 0000-0002-8136-0117
Código SPIN: 3477-8994

Cand. Sci. (Med.)

Rússia, Moscow

Liliya Urusova

National Medical Research Center of Endocrinology

Email: Urusova.Liliya@endocrincentr.ru
ORCID ID: 0000-0001-6891-0009
Código SPIN: 5151-3675

Dr. Sci. (Med.)

Rússia, Moscow

Maria Godzenko

National Medical Research Center of Endocrinology

Email: godzenko.mariya@endocrincentr.ru
ORCID ID: 0000-0001-8783-008X
Código SPIN: 6012-4491
Rússia, Moscow

Natalia Tarbaeva

National Medical Research Center of Endocrinology

Email: ntarbaeva@inbox.ru
ORCID ID: 0000-0001-7965-9454
Código SPIN: 5808-8065

Cand. Sci. (Med.)

Rússia, Moscow

Ekaterina Pigarova

National Medical Research Center of Endocrinology

Email: kpigarova@gmail.com
ORCID ID: 0000-0001-6539-466X
Código SPIN: 6912-6331

Dr. Sci. (Med.)

Rússia, Moscow

Larisa Dzeranova

National Medical Research Center of Endocrinology

Email: dzeranovalk@yandex.ru
ORCID ID: 0000-0002-0327-4619
Código SPIN: 2958-5555

Dr. Sci. (Med.)

Rússia, Moscow

Nadezhda Platonova

National Medical Research Center of Endocrinology

Email: doc-platonova@inbox.ru
ORCID ID: 0000-0001-6388-1544
Código SPIN: 4053-3033

Dr. Sci. (Med.)

Rússia, Moscow

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2. Figure 1. Multispiral computed tomography of the retroperitoneal space, formation of the right adrenal gland in the area of the medial peduncle (indicated by the arrow)

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3. Figure2. Multispiral computed tomography of the retroperitoneal space, formation of the right adrenal gland at the border of the medial peduncle and the body of the adrenal gland (indicated by the arrow)

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4. Figure 3. Morphological examination of postoperative adrenal material (staining with hematoxylin and eosin)

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5. Figure 4. Immunohistochemical examination of the left adrenal tumor (x100)

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