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Primary adrenal leiomyosarcoma: clinical case and literature review

https://doi.org/10.14341/serg12711

Abstract

Adrenal leiomyosarcoma — rare mesenchymal tumor, which is diagnosed most often after it reaches a large size. Primary adrenal leiomyosarcoma is extremely rare: in the English-language literature, only 45 cases were previously reported. We report the case of a 46-year-old man with the formation of the right adrenal gland measuring 12x8x7,5 cm. The patient underwent right-sided adrenalectomy, nephrectomy, and plastic of the inferior vena cava. The phenotype was confirmed by light microscopy and immunohistochemistry. Microscopic examination of the tumor is represented by fusiform and polymorphic cells that form multidirectional bundle structures with a high rate of mitosis. Immunohistochemically, tumor cells were positive for smooth muscle actin, CD-34, CD-117.

About the Authors

S. V. Lukyanov
Rostov State Medical University
Russian Federation

Stanislav V. Lukyanov, MD, PhD, assistant professor, elibrary SPIN: 4181-8431



K. M. Blikyan
Rostov State Medical University
Russian Federation

Karina M. Blikyan, MD, PhD, 29 Nahichevansky av., Rostov-on-Don, Russia, elibrary SPIN: 9918-5979



S. S. Todorov
Rostov State Medical University
Russian Federation

Sergey S. Todorov, MD, PhD, assistant professor, Scopus author ID: 7005217473, elibrary SPIN: 4404-3176



V. Y. Deribas
Rostov State Medical University
Russian Federation

Viktoriya Y. Deribas, assistant, Scopus author ID: 57217475386, elibrary SPIN: 8700-5205



N. S. Lukyanov
Rostov State Medical University
Russian Federation

Nikita S. Lukyanov



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Supplementary files

1. Figure 1. Magnetic resonance imaging of leiomyosarcoma of the right adrenal gland, the images show a clearly delineated heterogeneous formation above the right kidney (indicated by arrows): T1-weighted images in the coronal plane (a) and sagittal plane (b, c, d).
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2. Figure 2. Leiomyosarcoma of the right adrenal gland: a - fusiform cells with eosinophilic cytoplasm, forming multidirectional bundle structures (magnification × 200, staining - hematoxylin-eosin); b - figures of mitoses, including atypical ones (magnification × 400, staining - PAS reaction); c - foci of necrosis, large cells with ugly hyperchromic nuclei (magnification × 200, staining - hematoxylin-eosin).
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3. Неозаглавлен
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4. Figure 3. Leiomyosarcoma of the right adrenal gland: a - bright membrane-cytoplasmic expression of specialized smooth muscle actin (magnification × 200, immunohistochemistry (IHC) with an antibody to SMA); b - negative reaction in tumor cells (magnification × 200, IHC with an antibody to protein S-100); c - proliferation index 70% (increase × 200, Ki-67); d - vivid expression in the walls of numerous blood vessels in the tumor tissue, membrane-cytoplasmic expression in a part of tumor cells (magnification × 200, IHC with an antibody to CD-34); e - discrete nuclear expression in tumor cells (magnification × 200, IHC with anti-CD-117 antibody).
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Review

For citations:


Lukyanov S.V., Blikyan K.M., Todorov S.S., Deribas V.Y., Lukyanov N.S. Primary adrenal leiomyosarcoma: clinical case and literature review. Endocrine Surgery. 2021;15(1):36-42. (In Russ.) https://doi.org/10.14341/serg12711

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ISSN 2310-3965 (Online)