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Botiralieva G.K.

Ipsum Pathology LLC

Sharlai A.S.

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

Roshchin V.Yu.

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology

Sidorov I.V.

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

Konovalov D.M.

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology;
Russian Medical Academy of Continuous Professional Education

Rhabdomyosarcomas: structural distribution and analysis of an immunohistochemical profile

Authors:

Botiralieva G.K., Sharlai A.S., Roshchin V.Yu., Sidorov I.V., Konovalov D.M.

More about the authors

Journal: Russian Journal of Archive of Pathology. 2020;82(5): 33‑41

DOI: 10.17116/patol20208205133

Views: 4823

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To cite this article:

Botiralieva GK, Sharlai AS, Roshchin VYu, Sidorov IV, Konovalov DM. Rhabdomyosarcomas: structural distribution and analysis of an immunohistochemical profile. Russian Journal of Archive of Pathology. 2020;82(5):33‑41. (In Russ.)
https://doi.org/10.17116/patol20208205133

Подписка 2025 (Russian Journal of Archive of Pathology)
МСФ на ЯМ
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Rhabdomyosarcoma (RMS) is a malignant soft tissue tumor originating from primitive mesenchymal cells, which is most common in children.

OBJECTIVE

To qualitatively and quantitatively assess the expression of myogenic transcription factors on a large sample, to identify potential phenotypic differences, and to estimate the distribution and frequency of aberrant markers, such as ALK, PAX5, WT1, PCK, CAM5.2, SIX1, and Synaptophysin.

MATERIAL AND METHODS

The investigation included 202 tumor tissue samples. Five tissue microarrays were assembled from the obtained material for subsequent histological and immunohistochemical studies.

RESULTS

Embryonal RMS (ERMS) was diagnosed in 103 cases; alveolar RMS (ARMS) was detected in 80; spindle-cell/sclerosing RMS (SRMS) was found in 16 cases; epithelioid RMS (EpiRMS) was diagnosed in 2 patients. The expression of Myogenin and MyoD1 was detected in all the examined RMS tissue samples. ARMS was more characterized by staining at 1+ and 2+ intensities; at the same time, more than 50% of ERMS, SRMS, and EpiRMS cases showed staining at 1+ intensity. ALK expression was investigated using the D5F3 and p80 clones. The D5F3 clone displayed a higher staining intensity than the p80 clone (p<0.05). The expression of PAX5 was observed in 13 of 75 ARMS cases. That of WT1 and SIX1 was found in all RMS groups.

CONCLUSION

The morphological diagnosis of RMS requires a careful assessment of all of the above factors, especially taking into account the variability in the expression of myogenic transcription factors and the high level of phenotypic aberration.

Keywords:

rhabdomyosarcoma
immunohistochemical profile
ALK
myogenin
MyoD1

Authors:

Botiralieva G.K.

Ipsum Pathology LLC

Sharlai A.S.

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

Roshchin V.Yu.

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology

Sidorov I.V.

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

Konovalov D.M.

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology;
Russian Medical Academy of Continuous Professional Education

Received:

06.03.2020

Accepted:

16.06.2020

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