Dedifferentiated chordoma: A rare tumor with diagnostic challenge

Chordomas are rare locally invasive malignant bone tumors arising from remnants of embryonic notochord. Dedifferentiated chordoma (DC), a subtype, is characterized by the presence sarcomatous component in conventional (CC) which may arise de novo or as transformation previously treated chordoma. The dedifferentiation warrants poor prognosis due to distant metastasis and recurrences. De DCs pose diagnostic challenge especially small biopsies at metastatic sites. Here, we report case 45-year-old female presenting with long history backache constipation, finally diagnosed DC. Radiological well histomorphological pictures tumor posed challenges because they can mimic other occurring similar location. We found this worth reporting DC rarely reported literature it has potential therapeutic challenges. Here dedifferentiated both radiologically histomorphologically discuss role immunohistochemistry.