Abstract
This is a review of the clinical and histopathological published data on very rare heterotopic acinic cell carcinomas (AcCCs) with suggested optimal management. Extrasalivary AcCCs originate primarily in parotid lymph nodes.
They present at low clinical stage, show mostly low-grade histopathology and are circumscribed with a complete nodal capsule. Extracapsular dissection was advocated as adequate therapy.
In rare cases with positive surgical margins, a completion parotidectomy or adjuvant radiotherapy should follow. Heterotopic high-grade AcCCs are rare, necessitating radical surgery including neck dissection and adjuvant radiotherapy.
The short term prognosis is excellent, long term outcomes are not known. Longer term follow-up is essential.