Adenoid Cystic Carcinoma of Salivary Glands: A Ten-Year Review and an Assessment of the Current Management, Surgery, Radiotherapy, and Chemotherapy

Abstract

Adenoid cystic carcinoma (ACC) is a rare cancer that arises from the salivary glands and other sites in the body, such as the lung and breast. Although the tumor accounts for 10% of all salivary gland malignancies, it only accounts for 1% of head and neck malignancies. It can affect both major and minor salivary glands; here, it is called salivary gland adenoid cystic carcinoma or SACC, with a slight predilection to the latter, and commonly manifests between the 6th and 7th decades of life. The disease also shows a slight female predilection, with a reported female to male ratio of 3 : 2. Lesions of SACC are often insidious and slow-growing, and symptoms such as pain and altered sensation are frequently associated with advanced stages of the disease. Salivary adenoid cystic carcinoma is characterized by perineural invasion (PNI), a distinctive feature that potentially plays a significant role in the tumor’s relapse and recurrence, which is approximately 50%. The disease is not prevalent, and its etiopathogenesis is poorly understood, although several genetic patterns and biomarkers have been linked to its initiation and/or progression. The discovery of these mutations and biomarkers has encouraged several clinical studies to use therapeutic agents to target the specific receptors on the cancer cells to potentially prevent further proliferation of the tumor cells and metastasis of the disease. Diagnosis of SACC is often challenging and frequently requires a combination of clinical examination, imaging, and histopathology. Management of SACC is primarily surgical excision, while radiotherapy has shown to be effective in improving local control in cases with microscopic residual disease. However, treatment of recurrent or metastatic tumors by radiotherapy with or without chemotherapy has so far shown limited success. The aim of this thesis is to provide an update of literature on SACC with a particular focus on the latest management approaches and future trends.

Adenoid Cystic Carcinoma of the Minor Salivary Glands: A Systematic Review and Meta-Analysis of Clinical Characteristics and Management Strategies

“…A total of 10 studies that met the inclusion criteria were selected for critical review [ 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 ]. The sample size was 902 cases, which were analyzed using descriptive statistics and evaluated based on variables such as frequency and proportions.…”

“…In terms of disease control and survival among cases with available data, 79.2% demonstrated no evidence of disease. Local recurrence rates varied between 1% [ 23 ] and 28.5% [ 24 ], and distant metastasis occurred in 18.2% [ 30 ] to 33.3% [ 25 ] of cases, predominantly to lymph nodes (14.5%) [ 21 ]. An analysis of overall survival across various stages and patient numbers indicated a 5-year survival rate of 68.0% among 398 patients.…”

Macrocystic and non-necrotic salivary duct carcinoma of the submandibular gland: A case report

“…Nevertheless, the large cystic changes extending near the skin in our case might be atypical for intraductal carcinoma. Typical CT findings for adenoid cystic carcinoma and acinic cell carcinoma are unavailable [ 17 , 18 ], and multilocular cystic lesions detected by CT may be unusual for mucoepidermoid carcinoma and secretory carcinoma [ 19 , 20 ]. Although the CT finding of multilocularity observed in our case might be used to differentiate from other cystic salivary gland tumors, arriving at a radiological diagnosis of SDC was deemed impossible because of the unusual CT findings exhibited by our case.…”

“… Adenoid cystic carcinoma Unilateral. Typical features are unknown [17] . Acinic cell carcinoma Unilateral.…”

Unraveling the Molecular Complexity of Adenoid Cystic Carcinoma (ACC): A Comprehensive Exploration of Hub Genes, Protein-Protein Interaction (PPI) Networks, microRNA (miRNA) Involvement, and Drug-Gene Interactions (DGIs)

“…Adenoid cystic carcinoma (ACC) is an uncommon and enigmatic malignancy with distinct clinical challenges. Originating primarily in secretory glands, notably the salivary glands, ACC accounts for 1% of malignant tumours and 10% of salivary gland neoplasms [ 1 ]. It is distinguished by its distinct histological features, which include solid, tubular, and cribriform development patterns.…”