Chordoma : Molecular Targeted Therapies
Image Description
Newer Molecular Targeted Therapies for Chordoma: Since the discovery in chordomas of abnormalities in tyrosine kinase receptors and dysregulation of downstream signaling pathways (PI3K/AKT/mTOR, MAP/ERK, JAK/STAT, and retinoblastoma pathway), various receptor inhibitors have been used in clinical trials with limited success.
Monotherapy with tyrosine kinase inhibitors (TKIs) is recommended as the first-line management. For drug-resistant cases, combination therapy (two TKIs or TKI + mTOR inhibitor such as everolimus) is often used. Some cases have shown partial response, whereas others have remained stable or progressed while on treatment. A recombinant Saccharomyces cerevisiae vaccine encoding Brachyury is also undergoing clinical trials.
About this image: This image of sacrococcygeal chordoma shows epithelioid cells in a myxoid background. Such foci in small biopsies may be mistaken for metastatic carcinoma, especially with cytokeratin positivity. The cells have well-defined borders, abundant eosinophilic cytoplasm, and hyperchromatic nuclei. Many cells have prominent cytoplasmic vacuoles.
Monotherapy with tyrosine kinase inhibitors (TKIs) is recommended as the first-line management. For drug-resistant cases, combination therapy (two TKIs or TKI + mTOR inhibitor such as everolimus) is often used. Some cases have shown partial response, whereas others have remained stable or progressed while on treatment. A recombinant Saccharomyces cerevisiae vaccine encoding Brachyury is also undergoing clinical trials.
About this image: This image of sacrococcygeal chordoma shows epithelioid cells in a myxoid background. Such foci in small biopsies may be mistaken for metastatic carcinoma, especially with cytokeratin positivity. The cells have well-defined borders, abundant eosinophilic cytoplasm, and hyperchromatic nuclei. Many cells have prominent cytoplasmic vacuoles.