Bone and soft tissue sarcomas, tumors of the skinBone and soft tissue sarcomas, tumors of the skinСаркомы костей, мягких тканей и опухоли кожи2219-46142782-3687Publishing House ABV Press225BONE SARCOMASСАРКОМЫ КОСТЕЙNotochordal tumors (chordoma). Differential diagnosis. Immunohistochemical markersОпухоли нотохондры (хор-дома). Дифференциальный диагноз. Иммуногистохимические маркерыBoulytchevaI. V.БулычеваИ. В.
Russian Federation
irena@boulytcheva.comPavlenkoT. V.ПавленкоТ. В.
Russian Federation
MachsonA. N.МахсонА. Н.
Russian Federation
Hospital No. 62, MoscowМосковская городская онкологическая больница № 621101201241222411012022Copyright ©; 2012, Boulytcheva I.V., Pavlenko T.V., Machson A.N.Copyright ©; 2012, Булычева И.В., Павленко Т.В., Махсон А.Н.2012Boulytcheva I.V., Pavlenko T.V., Machson A.N.Булычева И.В., Павленко Т.В., Махсон А.Н.https://creativecommons.org/licenses/by/4.0https://sarbon.abvpress.ru/jour/article/view/225

Chordomas and low-grade chondrosarcomas share many histological features, generating at times, considerable diagnostic difficulty and, not infrequently, requiring immunohistochemical analysis for appropriate classification. In an effort to clarify the diagnosis and differentiation with other cartilaginous tumors, 16 samples, obtained from 16 patients with chordoma were examined by immunohistochemistry with a panel of antibodies. The panel included antibodies to cytokeratin (CK), epithelial membrane antigen (EMA), D2-40, GFAP (glial fibrillary acidic protein), NSE (neuron-specific enolase), chromogranin, TTF-1 (thyroid transcription factor), PSA, CDX-2, mammaglobin. The conventional chordoma stained for CK (16/16) EMA (13/16), NSE (12/16) and was negative for chromogranin, GFAP. Specific markers were helpful in differential with metastatic carcinomas. Chordomas are difficult to excise because of their location and intimate relation to critical structures. Seven of 16 patients had recurrences, three of 16 patients had several local recurrences and progression with local effects.

Хордома представляет собой специфическую мезенхимальную опухоль, развивающуюся из эмбриональных зачатков нотохорды со своеобразными клиническими и морфологическими проявлениями. По гистологическому строению хордома напоминает нотохорду. Изучено 16 случаев хордомы у 10 мужчин и 6 женщин в возрасте от 12 до 65 лет. Важным в диагностике хордомы оказался иммуногистохимический метод, выявляющий экспрессию цитокератинов, EMA, GFAP, NSE. Отрицательная реакция с хромогранином А, D2-40, а также органоспецифическими белками TTF-1, mammaglobin, PSA, CDX-2 позволяла исключить хордому и выявить метастазы рака различной локализации.

EMAGFAPNSEnotochordal tumorscytokeratineschromogranin AEMAGFAPNSEнотохордацитокератиныхромогранин АMitchell A., Scheithauer B.W., Unni K.K. et al. Chordoma and chondroid neoplasms of the spheno-occiput. A immunohistochemical study of 41 cases with prognostic and nosologic implications. Cancer. 1993, v. 72, No. 10, р. 2943-2949.Hoch B.L., Nielsen G.P, Liebsh N.G. et al. Base of skull Chordomas in children and adolescents; a clinicopathologic study of 73 cases. Am. J. Surg. Pathol. 2006, v. 30, No. 70, р. 811-818.Kyriakos M., Totty W.G., Lenke L.G. Giant vertebral notochordal rest: a lesion distinct from chordoma: discussion of an evolving concept. Am. J. Surg. Pathol. 2003, v. 27, No. 3, р. 396-406.Yamaguchi T., Suzuki S., Ishiiwa H. et al. Benign notochordal cell tumors: a comparative histological study of benign notochordal cell tumors, classic chordomas, and notochordal vestides of fetal intervertebral discs. Am. J. Surg. Pathol. 2004, v. 28, р. 756-761.Almefty K., Pravdenkova S., Colli B.O. et al. Chordoma and chondrosarcoma: similar, but quite different, skull base tumors. Cancer. 2007, v. 110, No. 11, р. 2457-2467.Fuchs B., Dickey I.D., Yaszemski M.J. et al. Operative management of sacral chordoma. J. Bone Joint Surg. Am. 2005, v. 87, No. 10, р. 2211-2216.O’Hara B.J., Paetau A., Miettinen M. Keratin subsets and monoclonal antibody HBME-1 in chordoma: immunohistochemical differential diagnosis between tumors simulating chordoma. Hum. Pathol. 1998, v. 29, No. 2, р. 119-126.Cho H.Y, Lee M., Takei H. et al. Immunohistochemical comparison of chordoma with chondrosarcoma, myxopapillary ependymoma, chordoid meningioma. Appl. Immunochistochem. Mol. Morphol. 2009, v. 17, No. 2, p. 131-138.Huse J.T., Pasha T.L., Zhang P.J. D2-40 functions as an effective chondroid marker distinguishing true chondroid tumors from choedoma. Acta Neuropathol. 2007, v. 113, No. 1, р. 87-94.Brooks J.J., Trojanowski J.Q., LiVolsi V.A. Chondroid chordoma: A low grade chondrosarcoma and its differential diagnosis. Curr. Top Pathol. 1989, v. 80, р. 165-181.Vujovic S., Henderson S., Presnau N. et al. Brachyury, a crucial regulator of notochordal development, is a novel biomarker for choredomas. J. Pathol. 2006, v. 209, No. 2, р. 157-165.