Angiosarcoma initially presented as repeated shedding tonsil neoplasm: a case report and literature review

Background: Angiosarcomas are rare malignant vascular tumors composed of several clinical subtypes and collectively have a poor prognosis especially with metastases. They can involve any soft-tissue structures or viscera and commonly present in the head and neck anatomical region. However, both primary and metastatic angiosarcomas in tonsil are extremely rare. Herein we reported a peculiar case of diffused metastatic angiosarcoma initially presented as a repeated shedding tonsil neoplasm. Case presentation: A 45-year-old Chinese man without history of cancers or radiation in the head and neck region presented with a left tonsil neoplasm which enlarged progressively. And shortly pieces of necrotic tissues began repeatedly shedding from the tonsil neoplasm. About 2 months later he gradually presented with systematic symptoms including fever and dyspnea. Twice tissue biopsy for the tonsil neoplasms in local hospital failed to prove malignant disease with preliminary pathological diagnosis of necrosis and chronic inflammation. In our hospital, functional imaging with PEC-CT supported a diffused metastatic disease status involving left tonsil, right atrium, mediastinum, and multiple bones. Further deep tissue biopsy and histopathology analysis revealed tonsil angiosarcoma with strong positive immunohistochemical staining for CD31 and CD34. Then the patient received two cycles of chemotherapy with paclitaxel plus cisplatin regimen and following efficacy evaluation revealed stable disease (SD). However, the patient gave up subsequent anti-cancer treatment due to personal choice and died 6 months after diagnosis due to progression of metastatic angiosarcoma. Conclusion: We described a unique case of diffused metastatic angiosarcoma involving tonsil in which diagnosis was not achieved until the third biopsy. Although we can't fully exclude the possibility that the tonsil angiosarcoma could be metastasized from a potential heart or bone primary site, this case was still extraordinarily peculiar because of both its rarity and unique initial presentation as a repeated shedding tonsil neoplasm. We have also presented a review of published reports concerning its rare incidence.