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Purpose
Acinic cell carcinoma (ACCs) is uncommon malignant epithelial neoplasm of the salivary glands; the most common presentation is a well-defined painless solid mass. Diagnosis of ACCs is frequently complicated, due to its similarity with benign tumors.
Methods
A review of the literature available on ACCs was carried out. Studies were sourced f...
Similar publications
Acinic cell carcinoma (ACC) is a malignant salivary gland tumor characterized by tumor cells displaying acinar features. Usually presenting as a slow-growing tumor, ACC, however, may show dedifferentiation to a higher grade including neuroendocrine carcinoma. In addition, ACC may rarely show focal neuroendocrine differentiation without any frank ev...
Citations
... Malignant salivary gland cancers (SGCs) are rare tumors, representing only 3% of all head and neck cancers; in most cases, they are benign (80%) epithelial tumors arising from the parotid gland [1,2]. In the context of parotid tumors, mucoepidermoid carcinoma is the most common, followed by adenoid cystic carcinoma and acinic cell carcinoma [2]. ...
Background: This study aims to retrospectively investigate the prognostic significance of the tumor microenvironment, with a focus on TILs (tumor-infiltrating lymphocytes), in relation to survival in a large cohort of patients with parotid gland cancer, and it uses the method proposed by the International TILs Working Group in breast cancer. Methods: We included a cohort of consecutive patients with biopsy-proven parotid cancer who underwent surgery between January 2010 and September 2023. A retrospective review of medical records, including surgical, pathological and follow-up reports, was performed. The density of TILs was determined according to the recommendations of the International TILs Working Group for breast cancer. Results: A weak negative correlation (p = 0.3) between TILs and time of survival and a weak positive correlation (p = 0.05) between TILs and months of survival (high TILs were correlated with longer survival in months) were identified. High TILs were weakly negatively, but not statistically significantly p (0.7), correlated with the grading of tumor; this means that high TILs were associated with low-grade tumors. Conclusions: Contrary to previous preliminary reports, this retrospective work found no statistically significant prognostic role of TILs in parotid gland malignancies. This case series represents the largest cohort ever reported in the literature and includes all malignant histological types. Future larger molecular studies may be useful in this regard.
... 1 AcCCs have been considered a disease with a relatively good prognosis for malignant tumors; however, recent findings have highlighted a potential for unpredictable recurrence and metastasis and a more malignant aspect, especially in the subgroup with high-grade transformation. 2,3 Radical marginal resection is the standard treatment for AcCC, although it may be too invasive to perform in some cases. Although previous studies have indicated favorable outcomes with postoperative adjuvant radiotherapy, the treatment's curative role in AcCC remains controversial. ...
... Although previous studies have indicated favorable outcomes with postoperative adjuvant radiotherapy, the treatment's curative role in AcCC remains controversial. [2][3][4][5] The benefits of other interventions such as elective neck dissection and chemotherapy have not been conclusively established. 6 Once AcCC metastasizes or invades the skull base, the presence of adjacent cranial nerves and major vasculatures can render curative resection challenging, because it can cause severe neurological impairment or even life-threatening complications. ...
... Few articles have reported curative radiotherapy for primary AcCC, and the treatment's efficacy remains controversial. 2,3,12 For the treatment of skull base AcCC, two studies reported that conformal radiation therapy and intensity-modulated radiation therapy were applied for local tumor control and led to survival for several years; these cases may indicate that some AcCCs exhibit radiosensitivity. 1,13 Nevertheless, given the tenacious tendency to recur, repeat extensive radiotherapy to the skull base might jeopardize adjacent cranial nerves and brain tissues. ...
BACKGROUND
Acinic cell carcinomas (AcCCs), rare malignancies of the salivary glands, often recur and metastasize, particularly in the skull base. Conventional radical resection can be invasive for skull base AcCCs adjacent to cranial nerves and major vasculature, and the effectiveness of stereotactic radiosurgery (SRS) as an alternative is not well established.
OBSERVATIONS
This case report details the application of SRS for recurrent skull base AcCCs. A 71-year-old male with a history of resection for a right mandibular AcCC 23 years earlier experienced tumor recurrence involving the right cavernous sinus and nasal cavity. He underwent endoscopic transnasal surgery followed by SRS targeting different tumor locations—the cavernous sinus to the pterygopalatine fossa, maxillary sinus, and clivus—each with a prescribed dose of 20 Gy to the 40% to 50% isodose line. After the first skull base metastasis, additional sessions of localized SRS after endoscopic surgery led to a 12-year survival without sequela.
LESSONS
This is a report indicating that SRS for skull base AcCCs can achieve favorable local control, functional preservation, and long-term survival. SRS may be suitable for skull base AcCC given the lesion’s tendency toward multiple local recurrences. Further investigation is needed to validate the treatment’s efficacy.
... Malignant salivary cancers are rare, only representing 3% of all head and neck cancers, and 80% of these cancers are benign epithelial tumors and arise in the parotid gland [1]. ...
Simple Summary
Acinic cell carcinoma (AciCC) is a rare parotid gland tumour that is indolent in the majority of patients; however, its definition is still evolving because in a subgroup of patients it shows aggressive behaviour with lateral neck metastases and massive recurrences. Several authors have attempted to characterise this subgroup of patients, but the main limitation is the rarity of the tumour for which there are no prospective studies. In this study, we defined the epidemiological, clinical and histological features of 77 patients with AciCC of the parotid gland, with the aim of defining the characteristics of the high-risk patients.
Abstract
Background. The acinic cell carcinoma (AciCC) of the parotid gland is a rare tumor with an indolent behavior; however, a subgroup of this tumor presents an aggressive behavior with a tendency to recur. The aim of this multicenter study was to identify and stratify those patients with AciCC at high risk of tumor recurrence. Methods. A retrospective study was carried out involving 77 patients treated with surgery between January 2000 and September 2022, in different Italian referral centers. Data about tumor characteristics and its recurrence were collected. The histological specimens and slides were independently reviewed by a senior pathologist coordinator (L.C.) and the institution’s local head and neck pathologist. Results. The patients’ age average was 53.6 years, with a female prevalence in the group. The mean follow-up was 67.4 months (1-258, SD 59.39). The five-year overall survival (OS) was 83.2%. The 5-year disease-free survival (DFS) was 60% (95% CI 58.2–61.7). A high incidence of necrosis, extraglandular spread, lymphovascular invasion (LVI), atypical mitosis, and cellular pleomorphism was observed in the high-risk tumors compared to the low-risk ones. Conclusion. AciCC generally had an indolent behavior, optimal OS, DFS with few cervical node metastases, and rare distant relapses. This multicenter retrospective case series provides evidence of the need for clinical–epidemiological–histological stratification for patients at risk of poor outcomes. Our results suggest that the correct definition of high-risk AciCC should include tumor size, the presence of necrosis, extraglandular spread, LVI, atypical mitosis, and cellular pleomorphism.
... The most common malignant salivary gland neoplasms include mucoepidermoid carcinoma (MEC), acinic cell carcinoma (ACC), adenoid cystic carcinoma (AdCC), carcinoma ex-pleomorphic adenoma (CExPA), and adenocarcinoma not otherwise specified (NOS) among the 24 different malignant salivary gland cancers recognized by the World Health Organization (WHO) in 2022 [1][2][3][4]. AdCC accounts for 1-2% of all malignant neoplasms of the head and neck [5][6][7] and 10% of salivary gland malignancies with a reported yearly incidence of 3-4.5 cases per million [8][9][10]. Parotid gland is the most frequently involved between the major salivary glands and those of the palate between the minor salivary glands [11,12]. ...
Background
Adenoid cystic carcinoma of the salivary glands is a relatively rare malignancy characterized by slow growth and a poor prognosis, and effective treatments remain challenging to identify. This systematic review, following the PRISMA guidelines, aimed to analyze the potential benefits of post-operative radiotherapy in terms of local control of recurrences and survival advantages when compared with surgery alone in patients with adenoid cystic carcinoma.
Methods
A comprehensive systematic review was conducted by searching the MEDLINE, Cochrane, EMBASE, and OVID databases from January 1999 to July 2022. The goal was to identify articles comparing surgery alone with surgery plus postoperative radiotherapy for adenoid cystic carcinoma of the salivary glands. Downs and Black Checklist was used to assess the methodological quality and risk of bias of each included study. The data analysis was performed using Review Manager version 5.4.1.
Results
This review included 8 studies comprising a total of 3103 patients, divided based on the analyzed outcomes. The pooled odds ratio for overall survival at 5 years was 0.87 (95% confidence interval 0.43–1.76, p = 0.70), and at 10 years was 1.23 (95% confidence interval 0.69–2.16, p = 0.48). In both cases, no statistically significant differences were observed. However, the pooled odds ratio for local control at 5 years was 3.37 (95% confidence interval 1.35–8.42, p = 0.009), providing strong support for the use of post-operative radiation.
Conclusions
The findings from the meta-analysis suggest that post-operative radiotherapy significantly improves local control in patients with adenoid cystic carcinoma. However, there was no statistically significant increase in survival at 5 and 10 years. It is essential to note that the quality of the studies included in this meta-analysis ranged from fair to poor. To better clarify the indications for post-operative radiotherapy, future high-quality research is needed, particularly with improved stratification of patient groups. Additionally, it is important to recognize that achieving local control in adenoid cystic carcinoma is crucial for enhancing the overall quality of life for patients. We acknowledge that this review was not registered in the PROSPERO database, and the data pooling was conducted using a random effects model.
... Acinic cell carcinoma (AciCC) accounts for 6-7% of all salivary gland malignancies, with >90% of the cases arising in the parotid gland [1]. It is the second most common salivary gland malignancy in children, but adult cases still vastly outnumber pediatric cases [2,3]. ...
... It is the second most common salivary gland malignancy in children, but adult cases still vastly outnumber pediatric cases [2,3]. Morphologically, AciCC resembles the serous acinar cells of the salivary gland [1] and is characterized by cells with abundant cytoplasm with basophilic zymogen granules. Its morphology is highly variable though and may overlap with other salivary gland tumors in limited biopsies. ...
... This discrepancy in nodal metastasis rates between studies using the SEER and the NCDB databases highlights the importance of considering variations in patient populations and data sources when interpreting and comparing findings across different studies. Some studies report that nodal metastasis affects mostly patients who have persistent or recurrent disease [1,2]. Fang et al. reported that positive intra-parotid lymph nodes in patients with negative neck nodes increased the risk for local recurrence and distant metastases; this interesting finding, which includes real angiolymphatic spread and extension by contiguity, has yet to be confirmed by others [15]. ...
Background:
Acinic cell carcinoma (AciCC) comprises 6-7% of all salivary gland neoplasms and is the second most common salivary gland malignancy in children. Like many salivary gland carcinomas, it is considered low grade but occasionally it behaves aggressively. Understanding the risk factors associated with recurrence, metastasis, and death is important to determine the counseling and management of individual patients. Older population-based studies are presumed to have been confounded by the misclassification of other neoplasms as AciCC, in particular secretory carcinoma and cystadenocarcinoma. Since diagnostic tools to reliably separate these entities have been available for over a decade, reevaluation of epidemiologic data limited to the 21st century should allow a better characterization of the clinicopathological characteristics of AciCC.
Methods:
Our study extracted data from the Surveillance, Epidemiology, and End Results (SEER) database for the period 2000 to 2018. Cox regression model analysis was performed to identify risk factors independently affecting survival.
Results:
Data for 2226 patients with AciCC were extracted from the database. Most patients were females: 59%, and white: 80.5%, with a mean age at diagnosis of 51.2 (SD ± 18.7) years. Most cases (81%) were localized at presentation. Tumor size was less than 2 cm in 42%, 2-4 cm in 47%, and >4 cm in 11%. Low-grade tumors had 5-year survival > 90%, whereas high-grade tumors had survival < 50%. Of the patients with known lymph node status only 7.3% had nodal metastases. Distant metastases were documented in 1.1%, involving lungs 44%, bone 40%, liver 12%, and brain 4%. The most common treatment modality was surgery alone: 63.6% followed by surgery and adjuvant radiation: 33%. A few received chemotherapy (1.8%) or multimodality therapy (1.2%). The 5-year overall survival rate was 90.6% (95%CI 89.1-91.9), and disease-specific survival was 94.6% (95%CI 93.3-95.6). Multivariable cox regression analysis showed that undifferentiated (HR = 8.3) and poorly differentiated tumor grade (HR = 6.4), and metastasis (HR = 5.3) were the worst independent prognostic factors. Other poor risk factors included age > 50 (HR = 3.5) and tumor size > 4 cm (HR = 2.5).
Conclusions:
In the US, AciCC is more common in middle age white females, and most tumors are less than 4 cm and localized at diagnosis. The most relevant negative prognostic factor was high tumor grade which was associated with higher hazard ratios for death than all other variables, including regional or distant metastases at presentation.
... Although imaging features are nonspecific, they assist in the evaluation of circumscription, extension, and relationship of lesions with the neighboring structures. 3,6,16 In the current study, imaging exams such as CT, US, and MRI were performed in 12 cases, mainly in those affecting the parotid gland. Most cases presented as a nodular mass without bone involvement in imaging studies. ...
... 15 The use of radiation therapy is still controversial and it is often used in tumors with high-grade t ra n sfor mat ion, recu r rent t umors, posit ive surgical margins, or cases with metastasis. 3,16,19 AciCC is considered a chemoresistant lesion, but chemotherapy may be useful in the treatment of pain. 3 Seven cases in the current study were treated with a combination of surgery, radiation therapy, and chemotherapy. Patients (n = 3) who succumbed to the disease had received adjuvant therapy, in addition to surgical excision. ...
... Distant metastases in this study mostly occurred via hematological spread, predominantly involving the bones and lungs. 16 Recurrence (p = 0.006) and regional metastases (p = 0.04) were significantly associated with patients who succumbed to the disease, confirming previous reports. 5,14 Given the retrospective nature of this study, some data were not available for all cases. ...
Abstract The aim of this study was to describe the prevalence, clinicopathological, and prognostic features of acinic cell carcinoma (AciCC) of the oral and maxillofacial region. AciCC cases were retrospectively retrieved from 11 pathology centers of three different countries. Medical records were examined to extract demographic, clinical, pathologic, and follow-up information. A total of 75 cases were included. Females (65.33%) with a mean age of 45.51 years were mostly affected. The lesions usually presented as an asymptomatic (64.28%) nodule (95.66%) in the parotid gland (70.68%). The association of two histopathological patterns was the most common finding (48.93%) and the tumors presented mainly conventional histopathological grades (86.11%). Surgical treatment was performed in the majority of the cases (59.19%). Local recurrence was observed in 20% of the informed cases, regional metastasis in 30.43%, and distant metastasis in 12.50%. The statistical analysis showed that the cases with a solid histopathological pattern (p=0.01), high-grade transformation (p=0.008), recurrence (p=0.007), and regional metastasis (p=0.03) were associated with poor survival. In conclusion, high histopathological transformation, presence of nodal metastasis, and recurrence were prognostic factors for AciCC of the oral and maxillofacial region.
... In most series, parotid gland ACCs were detected more in women than in men (6). Duzova et al. (7) reported a female dominancy for ACCs histology. ...
Aim: To evaluate the survival outcomes and prognostic factors in acinic cell carcinoma of the parotid gland, a retrospective study was designed. Material and Method: Consecutive patients diagnosed with parotid acinic cell carcinoma and treated with surgery and adjuvant radiotherapy were retrospectively reviewed. Data regarding age, sex, TNM stage, pathologic characteristics, treatment details, and follow-up examinations were collected and analysed. The primary end-point was overall survival; the distant metastasis free survival was calculated from the date of surgery to the date of death or the latest follow-up examination and analysed by the Kaplan-Meier method. Independent prognostic factors were evaluated by the Cox proportional hazards method. Results: Between years of 2010-2020, two radiotherapy centers’ database were reviewed. A total of 32 patients were included. The median age was 55 years (35-80 years). Four-teen (43.75%) were male and 18 (56.25%) were female. Median follow-up was 44 months (8-120). Seven (21.9%) were in T1, 7 (21.9%) in T2, 6 (18.8%) in T3 and 12 (37.5%) in T4 at the time of diagnosis. In all cohort, 6 (18.8%) of them had lymph node metastasis. The 2-year and 5-year OS rates were 92.6% and 78.5%, locoregional recurrence-free survival rates were 100% and 89.1%, distant metastasis free survival rates were 85.9% and 85.9%, respectively. Locoregional recurrence detected in 2 (6.25%), distant metastases detected in 4 (12.5%) patients. All distant metastases detected in the lungs. Univariate analysis showed that age, gender, margin status, T stage, facial nerve involvement, lymphovascular invasion, and perineural invasion were not significantly related to overall survival (all p>0.05). Lymph node involvement (p<0.013) and grade (p<0.006) were the only significant prognostic factors for OS. In multivariate analysis, both lymph node involvement (p<0.050) and grade (p<0.028) remained the significant prognostic factors. Conclusion: In acinic cell carcinoma of the parotid gland, high-grade histology and node positivity are independent variables that affect OS. Since survival is lower in these patient groups, it is imperative to explore other treatment options in addition to adjuvant radiotherapy.
... Additionally, a two-timed approach contains the difficulty of finding exactly those spots where the tumor was incompletely resected during the first session and increased the risk of facial nerve palsy due to the scarred tissue. Furthermore, the probability of a malignant tumor of the parotid gland is extremely low, as, e.g., AdCC represents only 2% and ACC about 1% of all neoplasms of the parotid gland [12,13], which is why a cost-benefit assessment based on the morbidity of the surgical approach (e.g., the potential of facial nerve palsy) should be considered carefully. The association of malignant tumors with incidental concurrent true calculi or salivary malignancies with large calcifications within the tumor mimicking salivary calculi have-to the best of our knowledge-only been described in two cases so far [7,14]. ...
Objectives: Sialolithiasis is the most common cause of calcifications detected with ultrasound in patients with chronic inflammatory symptoms and swellings of the salivary glands. Other differential diagnoses of calcifications are extremely rare and mostly benign. Methods: Case report and literature review. Results: Two rare cases of malignant parotid gland tumors with calcifications in a localization typical for sialolithiasis, which were mistaken for salivary calculi based on image findings, are presented. Conclusions: This report intends to highlight the pitfalls in the imaging of parotid gland diseases. Even if malignant tumors of the parotid gland with calcifications are extremely rare, in ambiguous cases, differential diagnoses should be considered carefully. A high suspicion index of the need for further diagnostics in cases with calcifications is practical and could include missing periprandial symptoms, no obstruction signs in the proximal duct, and missing evidence of sialolithiasis in sialendoscopy.
... Acinic cell carcinoma (AciCC) is the fourth most common epithelial tumour of salivary glands after mucoepidermoid carcinoma, adenocarcinoma (not otherwise specified, NOS) and adenoid cystic carcinoma [1]; it can be considered an extremely rare tumour (it comprises 9-11% of all adult parotid malignancies) [2] and its incidence has been previously described by the Surveillance of Rare Cancers in Europe Working Group (RARECARE) as 1.20-1.63 cases per 1,000,000 patients/years [3]. ...
... According to the latest histological classification of the World Health Organization (WHO), AciCC is defined as "a malignant epithelial neoplasm of salivary glands in which at least some of the neoplastic cells demonstrate serous acinar cell differentiation, which is characterized by cytoplasmic zymogen secretory granules. Salivary ductal cells are also a component of this neoplasm" [2]. Despite it having been considered for decades a low-grade indolent tumour with a good prognosis, its propensity to recur and metastasize, especially for a subset of lesions with Pietro De Luca and Luca de Campora share first authorship. ...
Objectives To analyze the demographic data, surgical and adjuvant treatment data, and the survival outcomes in adult patients affected by acinic cell carcinoma of the parotid gland (AciCC).
Methods A retrospective multicenter analysis of patients treated for AciCC of the parotid gland from 2000 to 2021 was performed. Exclusion criteria were pediatric (0-18 yr) patients, the absence of follow-up, and patients with secondary metastatic disease to the parotid gland. Multivariable logistic regression was used to determine factors associated with survival.
Results The study included 81 adult patients with AciCC of the parotid gland. The median age was 46.3 yr (SD 15.81, range: 19-84 yr), with a gender female prevalence (F= 48, M= 33). Mean follow-up was 77.7 months (min 4- max 361, SD 72.46). The 5 years overall survival (OS) was 97.5 % . The 5 years disease free survival (DFS) was 60%. No statistical differences have been found in prognosis for age (<65 or ≥65 years), sex, surgery type (superficial vs profound parotid surgery), radicality (R0 vs R1+Rclose), neck dissection, early pathologic T and N stages, and adjuvant therapy (p>0,05).
Conclusion This study didn’t find prognostic factor for poorest outcome. In contrast with the existing literature, our results showed how also high-grade tumors can’t be considered predictive of recurrence or aggressive behavior.
... Salivary gland cancers account approximately for 7% of all head and neck tumors [1]; these neoplasms can arise from major salivary glands (the parotid gland, submandibular gland, and sublingual gland) or from minor salivary glands in the upper aerodigestive tract [2]. Despite showing a low incidence as compared to other head and neck cancers, data suggest that their incidences have increased during the last twenty years. ...
Salivary gland cancers account approximately for 7% of all head and neck tumors [...]
