Notochordal tumors: differential diagnosis and prognostic factors

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Abstract

Chordoma is a rare mesenchimal tumor with anusual clinical behavior and morphologic appearance. In the current study we examined 94 cases of chordoma, predominantly localized in sacrum, ages 3-72, 44 men and 50 women. Six cases of dedifferentiated variant of chordoma were revealed. In an effort to clarify the diagnosis and differentiation, the tumor samples of chordoma were examined by immunohistochemistry with a panel of antibodies. The panel included antibodies to cytokeratin, epithelial membrane antigen (ЕМА), D2-40, GFAP (glial fibrillary acidic protein), S-100, brachyury, chromogranin A, TTF-1 (thyroid transcription factor), PSA, CDX-2, mammaglobin. The diagnosis of one case of poorly differentiated chordoma was proved by the lack of SMARCB1/INI-1 reactivity. A negative reaction with chromogranin A, D2-40, as well as a reaction with organ-specific proteins (TTF-1, mammaglobin, PSA, CDX-2) made it possible to exclude chordoma and reveal cancer metastases of various localization. Several examples of benign notochordal rests and differential with classic chordoma are proposed in the paper.

About the authors

Irina Vladislavovna Bulycheva

N.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia

Author for correspondence.
Email: irena@boulytcheva.com

F. Bertoni

Casa di Cura Villa Erbosa University of Bologna

Болонья

P. Baccini

Casa di Cura Villa Erbosa University of Bologna

Болонья

N. S. Babkin

I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia

A. V. Fedorova

N.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia

O. T. Khvan

N.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia

E. R. Musaev

N.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia

N. E. Kushlinskiy

N.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia

References

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