Here we present a patient with a parotid secretory carcinoma (SC) with high-grade transformation. A 65-year-old female was referred to our hospital due to a gradually growing right parotid tumor discovered initially about 4 years earlier. MRI imaging detected a right parotid tumor 50 mm in the longer axis. Fine needle aspiration cytology indicated a class III tumor. Nine months after her initial visit, she revisited our department because of pain, trismus and facial paralysis. MRI detected a tumor 69 mm in the longer axis and 64 mm in the shorter axis and a biopsy specimen revealed parotid cancer. Furthermore, positron emission tomography revealed a synchronous small cell lung cancer (SCLC). Chemoradiotherapy for the SCLC was performed followed by an extended total parotidectomy for the parotid SC. Histological findings and ETV6-FISH analysis confirmed a parotid SC with high-grade transformation. Two months after the surgery, CT revealed a loco-regional recurrence and proton beam therapy (70.2 GyE/26 Fr) was performed. Three months after the proton beam therapy, CT indicated pleural effusion and lung metastasis, and fine needle aspiration cytology revealed the metastatic SC. Eight months after the surgery, the patient died due to the lung metastasis of SC.
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