Abstract
Chordoma is a malignant tumour showing notochordal differentiation with three identified subtypes including chordoma not otherwise specified, chondroid chordoma and dedifferentiated chordoma. Dedifferentiated chordoma is rare and carries the worst prognosis of all subtypes due to rapid progression and potential for metastases. It is characterized by a high-grade sarcomatous component juxtaposed to conventional chordoma. We report a case of a dedifferentiated chordoma in a 71 year old woman who presented with pain in the lower back, numbness of feet and loss of weight of recent onset. Per-rectal examination revealed a soft tissue mass in the presacral region while imaging showed a solid and cystic lesion with calcifications in the presacral region. The radiological impression was of a retro rectal cystic hamartoma, an epidermoid cyst, or a chronic abscess. The resected specimen comprised a lobulated mass of soft, gelatinous tissue with haemorrhage and necrosis. Microscopy showed a biphasic tumour composed of conventional chordoma juxtaposed with high-grade sarcomatous component. Morphology and immunohistochemistry were compatible with a chordoma with a focal high-grade spindle cell sarcomatous component in keeping with a dedifferentiated chordoma.
Highlights
Chordoma is a malignant tumour arising from embryonic notochordal remnants but may arise from a benign notochordal cell tumour [1].Three histological subtypes of chordoma are identified; chordoma not otherwise specified (NOS), chondroid chordoma and dedifferentiated chordoma (DC)
DC is a biphasic tumour with a chordoma NOS component and high grade sarcomatous component
A DC is characterized by rapid progression, potential for distant metastases and a worse prognosis
Summary
Chordoma is a malignant tumour arising from embryonic notochordal remnants but may arise from a benign notochordal cell tumour [1]. DC may involve diverse sites and its etiology is unknown It may arise de novo, following radiotherapy and as recurrences or metastases of chordoma NOS. A previously well 71-year-old woman presented to National Hospital Kandy with pain in the lower back, numbness of feet and loss of weight for 2 weeks She did not have altered bowel habits or urinary symptoms. Pleomorphic nuclei with high mitotic activity (Figure 3D) Foci of necrosis were seen These high-grade areas juxtaposed with lowgrade areas of chordoma NOS. Tumour cells in chordoma NOS were strongly and diffusely positive for pancytokeratin (PCK) AE1/AE3, S100 and EMA (Figure 5 A, B, C) while the tumour cells in DC areas showed weak and focal positivity for these markers. The tumour was diagnosed as a chordoma with focal high-grade spindle cell sarcoma or DC
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