Undifferentiated pleomorphic sarcoma of oral cavity

Case contributed by Dr Ammar Ashraf


Dysphagia, dyspnea, and weight loss. No vomiting, fever, or significant past medical history.

Patient Data

Age: 85 years
Gender: Female

Findings: A heterogeneously enhancing soft tissue mass lesion, containing multiple cystic areas in it, is seen in the left oropharynx/tonsillar region. The lesion is extending across the uvula to the opposite side and invading the base of the tongue. No suspicious bony erosion/destruction is noted. A few enlarged lymph nodes are seen on both sides of the neck, with the largest node noted at right level III showing central necrosis. A heterogeneous nodule containing a few calcifications measuring 16 x 12 mm is seen in the left thyroid lobe. A few sections through the upper lungs demonstrate mild bilateral apical pleural thickening (suggestive of old granulomatous disease) and mild emphysematous changes.  Left otomastoiditis.

Conclusion: Left oropharyngeal mass lesion invading the base of the tongue and extending across the midline to the opposite side along with bilateral upper cervical lymphadenopathy. Possible differential diagnosis includes squamous cell carcinoma, lymphoma, and malignant soft tissue sarcoma.

Case Discussion

Procedure: Debulking oropharyngeal mass and percutaneous tracheostomy.

Histopathology: Sarcomatoid neoplasm with high-grade features including marked nuclear pleomorphism, brisk mitotic activity, and large areas of necrosis. The differential diagnosis includes sarcoma, sarcomatoid carcinoma, and malignant melanoma.

Immunohistochemistry: The tumor stains negatively for CK AE1/AE3, CK5/6, EMA, S100, Desmin, and CD34. CD68 and Vimentin highlight the multinucleated giant cells, as well as individually scattered histiocytic cells. CD34 and Desmin demarcate an increased number of vessels. 

Final diagnosis: High-grade malignant neoplasm consistent with pleomorphic malignant fibrous histiocytoma (MFH), now known as undifferentiated pleomorphic sarcoma (UPS).

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