{"entity":{"id":"acinic-cell-carcinoma-salivary","kind":"cancer","name":"Acinic cell carcinoma of the salivary glands","aka":["Acinic cell carcinoma","Acinic cell carcinoma (NR4A3)","Acinar cell carcinoma of the parotid","Acinic cell adenocarcinoma"],"tldr":"Acinic cell carcinoma is a salivary gland cancer, almost always of the parotid gland, whose cells resemble the gland's normal enzyme-making cells. It is usually low grade and slow, presenting as a painless lump that is easily mistaken for a benign tumour, and surgery cures most; a high-grade minority behaves aggressively and needs radiotherapy too.","summary":"Acinic cell carcinoma is a malignant epithelial neoplasm of the salivary glands defined in the WHO head and neck classification by serous acinar differentiation, with a recurrent NR4A3 rearrangement noted on the parent page; the most common presentation is a well-defined painless solid mass, diagnosis is complicated by its similarity to benign tumours, imaging has no clear characteristics, radical surgery is the best treatment, the role of radiotherapy remains controversial and literature on chemotherapy for metastatic disease is insufficient (Eur Arch Otorhinolaryngol 2020). In 2,362 National Cancer Database cases, most patients were women (61.3 percent) with a median age of 54; age over 70, high grade, size over 3 cm, four or more positive nodes and T4 stage were significant adverse prognosticators (Oral Oncology 2018).\n\nHow it differs from its parent: the salivary gland page covers all types; acinic cell carcinoma is the low-grade type with the best outlook after adenoid cystic and mucoepidermoid carcinoma are set aside, with a defined high-grade transformed variant that changes the plan.\n\nHow common: the third or fourth most common salivary malignancy; no UK share in the sources read.\n\nTreatment: parotidectomy with facial nerve preservation where possible and neck dissection for node-positive or high-grade disease; postoperative radiotherapy for high grade, positive margins or nodes; systemic therapy for metastatic disease has no evidence base and follows the parent page (Eur Arch Otorhinolaryngol 2020).","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/Acinic_cell_carcinoma","links":[{"label":"NCI PDQ: salivary gland cancer treatment","url":"https://www.cancer.gov/types/head-and-neck/patient/adult/salivary-gland-treatment-pdq"},{"label":"Oral Oncology 2018: parotid acinic cell carcinoma, National Cancer Database study of 2,362 cases","url":"https://doi.org/10.1016/j.oraloncology.2018.05.002"},{"label":"Eur Arch Otorhinolaryngol 2020: acinic cell carcinoma of the parotid gland, from pathogenesis to management (review)","url":"https://doi.org/10.1007/s00405-020-05998-6"}],"tags":["subtype-page","wave4","rare"],"related":["salivary-gland","carcinoma-ex-pleomorphic-adenoma","secretory-carcinoma-breast","adenoid-cystic-carcinoma","salivary-duct-carcinoma"],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"head and neck","burden":"2,362 cases of parotid acinic cell carcinoma in the US National Cancer Database over 2004 to 2012, 61.3 percent women, median age 54 (Oral Oncology 2018). Cancer Research UK counts salivary gland cancers together.","subtypes":["Low-grade acinic cell carcinoma of the parotid (the usual form)","Acinic cell carcinoma with high-grade transformation (aggressive)","Acinic cell carcinoma of the submandibular or minor salivary glands (rare)"],"biomarkers":["Serous acinar differentiation; DOG1 and SOX10 positive","NR4A3 rearrangement","Grade, size over 3 cm, node number and T stage (prognostic)","Distinction from secretory carcinoma (ETV6-NTRK3)"],"standardOfCare":[{"setting":"All stages","approach":"Parotidectomy with nerve preservation; neck dissection and postoperative radiotherapy for high grade, positive margins or nodes; metastatic disease as on the parent page.","refs":["salivary-gland"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"salivary-gland"},"route":"/cancers/acinic-cell-carcinoma-salivary/","neighbours":{"cancer":[{"id":"adenoid-cystic-carcinoma","kind":"cancer","name":"Adenoid cystic carcinoma","route":"/cancers/adenoid-cystic-carcinoma/"},{"id":"carcinoma-ex-pleomorphic-adenoma","kind":"cancer","name":"Carcinoma ex pleomorphic adenoma","route":"/cancers/carcinoma-ex-pleomorphic-adenoma/"},{"id":"salivary-duct-carcinoma","kind":"cancer","name":"Salivary duct carcinoma","route":"/cancers/salivary-duct-carcinoma/"},{"id":"salivary-gland","kind":"cancer","name":"Salivary gland cancers","route":"/cancers/salivary-gland/"},{"id":"secretory-carcinoma-breast","kind":"cancer","name":"Secretory carcinoma of the breast","route":"/cancers/secretory-carcinoma-breast/"}]}}