• facial nerve sacrifice
• low‐grade acinic cell carcinoma
• low‐grade mucoepidermoid carcinoma
• parotid
• parotidectomy

• Facial nerve
• Facial palsy
• Parotid tumours
• post-parotidectomy

• Acinar cell
• Acinic cell carcinoma
• Carcinoma
• Neck dissection
• Prognosis
• Propensity score
• Radiotherapy
• Salivary gland neoplasms

• adenoid cystic carcinoma
• mucoepidermoid carcinoma
• precision therapy
• salivary duct carcinoma
• salivary gland cancer

• recurrent salivary cancer
• recurrent salivary tumors
• salivary cancer failure
• salvage surgery
• salvage surgery outcomes

• Disease-Free Survival
• Humans
• Neoplasm Recurrence, Local/pathology
• Retrospective Studies
• Salivary Gland Neoplasms/pathology
• Salvage Therapy

Acinic cell carcinoma (ACC) is a rare low-grade salivary gland malignancy that accounts for approximately 17% of all salivary gland malignancies. The most common site affected by ACC is the parotid gland followed by the submandibular glands, minor salivary glands, and sublingual glands. Also, it could hardly be observed in unusual sites such as the jaw bones.

This case is an example of a central acinic cell carcinoma in a 73-year-old man who came up with a painless gradual swelling for 15 months. Based on clinico-radio-pathologic findings, the diagnosis of a solid variant Intraosseous Acinic Cell Carcinoma was established. Subsequently, the patient underwent hemimandibulectomy and modified radical neck dissection, followed by postoperative radiotherapy. Within a six-month follow-up period, no evidence of residual tumor was found.

Central salivary gland carcinoma is a rare entity and intraosseous ACC is more scarcely observed. Based on our findings, a total of 17 cases of primary intraosseous ACC have been reported so far. Etiology and clinical presentations of this tumor are still vague due to its rarity.

Dentists and oral surgeons must be aware of such a rare malignant lesion when encountering a radiolucent lesion within the jaws. The early diagnosis and a complete surgical excision to achieve tumor-free surgical margins and a long-term follow-up could result in significantly improved survival rates.

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ACCs are a type of uncommon salivary gland tumors and the intra-osseous counterparts are extremely rare.

• Acinic cell carcinoma
• Head and neck
• Mandible
• Salivary gland tumor

To present a case of adrenocorticotropic hormone (ACTH) hypersecretion caused by a metastatic acinic cell carcinoma (AcCC) of the parotid. Only 6 cases have been reported prior to October 2019. We believe that this condition is under-reported and hope that improved recognition will improve its reporting.

Diagnosis in this case was done using surgical pathology of the primary tumor, involving lymph nodes, and a metastatic lesion. Following an initial misdiagnosis, a final diagnosis of AcCC was made using immunohistochemical staining. ACTH hypersecretion was diagnosed by testing for random ACTH, cortisol, and 24-hour urine aldosterone and cortisol levels.

A 57-year-old man presented with hypokalemia, lower-extremity edema, and left-side rib pain 7 months following excision of a 4-cm left-parotid tumor. Immunostaining positive for DOG-1, CK7, pan-cytokeratin (including CAM5.2), and SOX10 led to the diagnosis of AcCC. ACTH hypersecretion was diagnosed based on a random ACTH level of 307 pg/mL (normal morning value, 7.2-63 pg/mL), a cortisol level of 33 μg/dL (normal morning value, 4.3-19.8 μg/dL; normal PM value, 3.1-15.0 μg/dL), a 24-hour urine aldosterone level of <0.7 U (normal, 2.0-20 U), and a 24-hour urine cortisol level of 4564 U (normal, 3.5-45 U). The patient’s ACTH hypersecretion and hypokalemia were treated with potassium replacement, amiloride, and ketoconazole. His metastatic recurrence was treated with radiotherapy, chemotherapy, and immunotherapy. The patient died after being diagnosed with sepsis secondary to multifocal postobstructive pneumonia 4 months after the diagnosis of his metastatic recurrence.

Ectopic ACTH production caused by metastatic AcCC is a rare phenomenon but has been increasingly described over the last 15 years. We believe that this condition likely has a greater prevalence than what is reported and that improved recognition will lead to improved outcomes.

• ACTH, adrenocorticotropic hormone
• AcCC, acinic cell carcinoma
• CMP, comprehensive metabolic panel
• CT, computed tomography
• Cushing's disease
• FNA, fine-needle aspiration
• PET, positron emission tomography
• acinic cell carcinoma
• ectopic ACTH
• paraneoplastic

• chemotherapy
• metastatic acinic cell carcinoma
• unknown primitive tumor

In subset of patients, acinic cell carcinoma (AcCC) exhibits aggressive features such as recurrence, distant metastasis, and mortality. This study aimed to investigate clinicopathologic factors influencing patients’ prognosis and to identify adverse features predictive of an unfavorable prognosis.

Between January 2000 and December 2016, 59 patients with AcCC were enrolled in this study.

The patients’ 5-year overall survival rate was 93.3%, and their 5-year recurrence-free survival rate was 80.5%. During the study period, recurrence occurred in 10 patients. The mean time to recurrence after surgery was 26 months (range, 5–60 months). During the study period, three patients died from the disease. Univariate analysis showed that sex, surgical extent, extranodal extension, T classification, and TNM stage were significantly associated with disease recurrence. Multivariate analysis showed that, among the clinicopathologic factors included in the analysis, only TNM stage displayed a statistically significant correlation with disease recurrence.

Surgical treatment alone yielded good results for AcCC, and additional treatment did not affect the recurrence-free survival rate or the overall survival rate, even when the resection margin was less than 1 mm. Other pathologic factors did not show prognostic significance for disease recurrence or death.

• Acinic Cell Carcinoma
• Parotid Gland
• Prognostic Factor

AiCC is a primarily indolent disease process. Our aim with this study is to determine characteristics consistent with rapidly progressive AiCC of the parotid gland.

To report on patients with metastatic lung disease from AiCC and potential correlative factors.

Single-institution retrospective review of patients treated at the University of Michigan between 2000 and 2017. Univariate analyses were performed.

A total of 55 patients were identified. There were 6 patients (10.9%) with primary AiCC of the parotid gland who developed lung metastases. The mean age at diagnosis for patients with lung metastases was 57.8 years of age, in comparison to 40.2 years for those without metastases (P = 0.064). All 6 of the patients with lung metastases demonstrated gross perineural invasion intraoperatively, in comparison to none of those in the non-lung metastases cohort. Worse disease-free and overall survival were significantly associated with gross perineural invasion, high-grade differentiation, and T4 classification (P < 0.001).

AiCC of the parotid gland is viewed as a low-grade neoplasm with good curative outcomes and low likelihood of metastasis. With metastasis, however, it does exhibit a tendency to spread to the lungs. These patients thereby comprise a unique and understudied patient population. In this retrospective study, factors that have been shown to be statistically significant in association with worse disease-free survival and overall survival include presence of gross facial nerve invasion, higher T-classification, and high-grade disease.

• Acinic cell carcinoma
• Parotid gland
• Distant metastases
• Facial nerve
• Perineural invasion

Adrenocorticotropic hormone (ACTH) ectopic production is a rare cause of Cushing syndrome (CS). The most commonly associated tumours are small-cell lung carcinoma along with bronchial and thymic carcinoids. To date, only 5 cases have been published in the literature featuring ectopic ACTH secretion from metastatic acinic cell carcinoma (ACC) of the parotid gland. We hereby describe a very uncommon case of ectopic CS (ECS) unveiling a metastatic parotid ACC.

A 46-year-old man with hypertension and dyslipidemia diagnosed 4-months before, as well as new-onset diabetes mellitus unveiled 1-month earlier, was referred to emergency department for hypokalemia. Hormonal study and dynamic biochemical tests performed indicated ECS. Imaging and cytological findings pointed toward a likely primary right parotid malignancy with liver metastases. Somatostatin receptor scintigraphy has shown an increased uptake in the parotid gland and mild expression in liver metastasis. The patient underwent right parotidectomy, and histopathologic examination confirmed ACC. Meanwhile, hypercortisolism was managed with metyrapone, ketoconazole, and lanreotide. Despite chemotherapy onset, a rapid disease progression and clinical course deterioration was observed.

The present report highlights a rare ECS, exposing a metastatic parotid ACC, with an aggressive and challenging clinical course, representing the first case whose diagnosis of ECS came prior to ACC.

Objectives: This study provides an up-to-date overview of the distribution of salivary gland tumors in relation to sex, land of treatment, localization of the tumor in the mouths, and benign/malignant disease of this type of tumor. We hypothesized that the distribution of patients with salivary gland tumors could vary according to country, gender, age and tumor specificity. In addition there is a comparison of the primary classification of salivary gland tumors from 1981 and the recent classification from 2005.

Materialsand methods: Data from the Medline database PubMed.gov and supplementary sources were used to conduct a systematic literature search. For this purpose, data from different studies were independently collected using a previously designed questionnaire.

Results: The first section analyzes the general features of the relevant salivary gland tumors from 141 studies involving a total of 25,826 patients across 30 different countries in terms of gender and the occurrence of benign/malignant salivary gland tumors. These data were summarized and presented.

Conclusion: This review offers an insight into the dramatic local differences with regard to salivary gland tumor occurrence as a stepping stone to further classify such data in order to derive effective therapy options, prognosis and widen the general understanding of the subject.

• acinic cell carcinoma
• adenoid cystic carcinoma
• mucoepidermoid carcinoma
• pleomorphic adenoma
• salivary gland tumors

• Acinic cell carcinoma
• Mammary analog secretory carcinoma
• Pathology
• Prognosis
• Salivary glands
• Therapy

• acinic cell carcinoma
• grade
• surgery
• survival
• treatment