Sarcomatoid carcinoma is also known as spindle cell carcinoma. It is a rare biphasic tumour and an aggressive variant of squamous cell carcinoma. An accurate diagnosis of this rare variant of squamous cell carcinoma is crucial due to its aggressive behaviour and potential for early metastasis. We present a unique case of a female patient in her 60s with advanced sarcomatoid carcinoma of the tongue, characterised by an atypical clinical course and an exceptional response to immunotherapy, aiming to raise awareness of this rare malignancy and its treatment.
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