Why the study?
Angiosarcomas rarely involve the oral cavity in young adults, presenting diagnostic challenges due to their unusual presentation and aggressive nature.
This case highlights the diagnostic challenges of primary angiosarcoma presenting in an unusual site (upper alveolus and maxilla) in a young adult.
Highlights diagnostic challenges of angiosarcoma in atypical maxillary sites in young adults; leaves open need for larger series to inform practice.
Angiosarcoma is a rare neoplasm, constituting only 2% of all the soft tissue tumors and most frequently involves the skin of the head and neck region in elderly males. They are extremely aggressive tumors with high rates of metastasis and poor outcomes. We report a unique case of angiosarcoma involving an unusual site - upper alveolus and maxilla in a young patient highlighting the diagnostic challenges in such a scenario. A 29 years old female presented with a non-healing wound of the oral cavity, which had progressed to the current maximum size of 6.4 cm within one month. Magnetic resonance imaging (MRI) scan revealed the involvement of maxilla up to the floor of the orbit and adjacent soft tissue. However, no distant metastasis was detected on Positron Emission Tomography (PET) scan. Biopsy of the lesion showed an irregular, highly pleomorphic, and mitotically active epithelioid soft tissue tumor conclusively diagnosed as angiosarcoma.
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Lali et al. (2020) studied this question.
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