Retroperitoneal synovial sarcoma

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Abstract

Synovial sarcoma of the retroperitoneum is a rare and aggressive tumor. In some cases, differential diagnosis between retroperitoneal sarcoma and adrenocortical cancer is difficult due to the similar clinical and intrascopic manifestations of these neoplasms. The final diagnosis is established based on the results of histological, immunohistochemical and genetic studies. The most important step in the treatment of synovial sarcoma is complete removal of the tumor. However, despite the radical nature of the intervention, the incidence of local relapse and metastasis remains high.

AIM: to conduct a retrospective analysis of the parameters of operated patients with synovial sarcoma of the retroperitoneum.

This study is aimed at studying the results of examination and surgical treatment of patients with synovial sarcoma of the retroperitoneal space who were hospitalized in the E.E. Eichwald Clinic surgical department of the North-Western State Medical University named after. I.I. Mechnikov.

The patients were assessed for hormonal status using an immunoassay to determine the levels of adrenocorticotropic hormone, cortisol, aldosterone, renin, normetanephrine and metanephrine in the blood, and a dexamethasone suppression test has been performed at a dose of 1 mg. High-performance liquid chromatography has determined 6 glucocorticoids in the blood serum: cortisol, cortisone, corticosterone, 11-deoxycorticosterone, 11-dehydrocorticosterone, 11-deoxycortisol. The urine steroid profile was studied in all the patients using gas chromatography-mass spectrometry. All the patients underwent computed tomography of the abdominal organs and retroperitoneal space with contrast, morphological and immunohistochemical examination of the removed material. The material was fixed in 10% neutral buffered formalin. The pieces were then subjected to standard wiring followed by embedding in paraffin. Sections 2–3 microns thick were prepared from the resulting blocks and stained with hematoxylin and eosin. Immunohistochemical studies were performed on paraffin sections. A panel of monoclonal antibodies was used: protein S-100 (poly), pancytokeratin (AE1/AE3), EMA, cytokeratin-8, -18, chromogranin A, synaptophysin, α-inhibin, SOX-10, CD99, TLE-1, bcl- 2, GATA-3. Postoperative outcomes and follow-up were assessed according to the information in the medical records and data obtained from a telephone conversation with each patient. Statistical processing of the results has not been out due to rare cases of the disease.

About the authors

Aleksandr A. Lisitsyn

North-Western State Medical University named after I.I. Mechnikov

Email: aleksandr.lisitsyn@szgmu.ru
ORCID iD: 0000-0003-2045-0044
SPIN-code: 3237-4309

MD, Cand. Sci. (Med.), Assistant Professor

Russian Federation, 41 Kirochnaya St., Saint Petersburg, 191015

Vyacheslav P. Zemlyanoy

North-Western State Medical University named after I.I. Mechnikov

Email: zeml.spb@mail.ru
ORCID iD: 0000-0003-2329-0023

MD, Dr. Sci. (Med.), Professor, Honored Doctor of the Russian Federation

Russian Federation, 41 Kirochnaya St., Saint Petersburg, 191015

Inna Е. Kotkas

North-Western State Medical University named after I.I. Mechnikov

Author for correspondence.
Email: inna.kotkas@yandex.ru
ORCID iD: 0000-0003-4605-9887
SPIN-code: 1853-8825

MD, Dr. Sci. (Med.), Professor

Russian Federation, 41 Kirochnaya St., Saint Petersburg, 191015

Irina A. Bekhtereva

North-Western State Medical University named after I.I. Mechnikov

Email: Irina.Bekhtereva@szgmu.ru
ORCID iD: 0000-0002-5206-3367
SPIN-code: 3954-2873

MD, Dr. Sci. (Med.), Assistant Professor

Russian Federation, 41 Kirochnaya St., Saint Petersburg, 191015

References

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Supplementary files

Supplementary Files
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1. JATS XML
2. Fig. 1. Computed tomography of the abdominal and retroperitoneal area with contrast of a 55-year-old patient with synovial sarcoma of the retroperitoneal area on the right side: a, axial plane, enhanced phase; b, coronal plane, enhanced phase

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3. Fig. 2. Macro specimen of removed retroperitoneal synovial sarcoma (patient V., 55 years old)

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4. Fig. 3. Patient V., 55 years old, diagnosis “synovial sarcoma”. a, tissue of the adrenal cortex (in the center), in the pericapsular adipose tissue there are sites of tumor growth (bottom right); the upper left corner is the focus of synovial sarcoma, staining with hematoxylin and eosin, magnification ×40 (magnifying glass); b, the tumor is formed by spindle-shaped cells, forming short multidirectional bundles; multiple “geographical” types of necrosis are determined, hematoxylin and eosin staining, magnification ×100

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5. Fig. 4. Patient K., 27 years old, diagnosis “synovial sarcoma”. a, the tumor is formed by spindle-shaped cells that form short multidirectional bundles, staining with hematoxylin and eosin, microscope magnification ×100; b, the nucleus of tumor cells are oval; the chromatin is vesicular; the nucleus are poorly defined; the cytoplasm of tumor cells is scanty and has no clear boundaries, hematoxylin and eosin staining, magnification ×400, numerous mitoses

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6. Fig. 5. Patient K., 27 years old, diagnosis “synovial sarcoma”. Staining — immunohistochemical reaction: nuclear expression of TLE-1 (a), diffuse cytoplasmic bcl-2 (b) in most tumor cells (magnification ×200)

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7. Fig. 6. Patient V., 55 years old, diagnosis “synovial sarcoma”. Staining — immunohistochemical reaction: expression of GATA-3 (a) in the most tumor cells (magnification ×200), expression of cytokeratin 8 (b) in a small part of tumor cells (magnification ×200)

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