Malignant parotid tumors in childhood are extremely rarely diagnosed diseases. Due to the rare incidence, there is no consensus on their complex treatment, as well as on the application of postoperative radiation therapy (RT). We present a clinical case of acinic cell parotid carcinoma in a 12-year-old girl, against the background of which we emphasize the optimal complex therapeutic approach. Despite radical facial nerve-sparing parotidectomy in pT2N0 acinic cell parotid carcinoma, due to the two pathohistological negative factors/two foci with vascular tumor invasion and the close proximity of the resection line to the preserved facial nerve, we conducted postoperative adjuvant RT. Due to the risk of late radiation reactions of normal tissues and organs after RT in childhood, intensity modulated radiation therapy (IMRT) with lower radiation doses in the target volumes was performed. Active surveillance and follow-up imaging studies are required for early diagnosis of possible recurrence or distant metastases.
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