A population study comparing tracheal and lung adenoid cystic carcinoma.

BACKGROUND: Thoracic adenoid cystic carcinoma (ACC) is rare, and the differences between tracheal and lung lesions have not been fully understood. METHODS: Patients were identified from a Chinese cancer center (FUSCC) (2005-2022) and the Surveillance, Epidemiology, and End Results (SEER) database (2000-2019). Incidence was calculated and trends were quantified. Clinicopathological features and overall survival (OS) were analyzed. Nomograms predicting OS were constructed. RESULTS: Totally, 55 tracheal adenoid cystic carcinoma (TACC) and 25 lung and bronchus adenoid cystic carcinoma (LACC) were included in a Chinese cohort, 121 TACC and 162 LACC included in the SEER cohort. There were larger tumor sizes, more lymph nodes and distant metastases for LACC than TACC patients. TACC patients are more likely to get local treatments. Patients with LACC had significantly worse median OS than patients with TACC (SEER cohort: 68.0 months vs. 109.0 months, p = 0.001, Chinese cohort: 62.9 months vs. 124.8 months, p = 0.061). Age, lymph node metastasis, distant metastasis and local treatment were identified as independent prognostic factors for OS of TACC. Distant metastasis and local treatment were identified for LACC. Specifically, surgery alone or in combination with radiotherapy is crucial for improving survival in both TACC and LACC. Only TACC benefits from radiotherapy alone, while chemotherapy does not improve survival for either. The nomograms constructed using these factors revealed good prognostic accuracy. CONCLUSIONS: LACC is more aggressive and has a worse prognosis than TACC. TACC patients have more opportunities for local treatment, which is important for the prognosis of both TACC and LACC. Nomograms were created for TACC and LACC to aid in personalized survival predictions and clinical decisions.

Platinum-based adjuvant chemoradiotherapy versus adjuvant radiotherapy in patients with head and neck adenoid cystic carcinoma.

PURPOSE: The objective of the study was to assess the effectiveness and toxicity of platinum-based adjuvant chemoradiotherapy (POCRT) in comparison to postoperative radiotherapy (PORT) in patients with head and neck adenoid cystic carcinoma (HNACC). MATERIALS AND METHODS: This retrospective study analyzed patients diagnosed with HNACC at our center between January 2010 and April 2020. A 1:1 propensity score matching method was used to create a matched cohort. RESULTS: In this study, 206 patients were analyzed, with 147 patients (71.4%) receiving postoperative radiotherapy (PORT) and 59 patients (28.6%) receiving POCRT. Twenty-one patients experienced local-regional failure. The 3-, 5-, and 10-yr local-regional control (LRC) rate for the cohort were 92.0%, 90.6%, and 86.9%, respectively. In both the entire cohort and the matched cohort, the POCRT group exhibited superior LRC compared to the PORT group (Gray's test, all P < 0.05*). Multivariate analysis identified adjuvant concurrent chemotherapy as an independent prognostic factor for LRC (Competing risks regression, HR = 0.144, 95% CI 0.026-0.802, P = 0.027*). In addition, the POCRT group had higher incidences of upper gastrointestinal toxicity and hematologic toxicities, including leukopenia, neutropenia, and anemia (all P < 0.05*). CONCLUSION: In terms of reducing locoregional failures in HNACC patients, POCRT may potentially offer a more effective therapeutic approach than using PORT alone, although it also entails an augmented burden of treatment-related toxicity.

Adenoid Cystic Carcinoma, Clinical Presentation, Current Treatment and Approaches Towards Novel Therapies.

Adenoid cystic carcinoma (AdCC) is a rare cancer originating from secretory glands with unknown aetiology. It is one of the most dominant malignant salivary tumours (MST). However, it can arise in other areas of the head and neck region and in secretory glands outside this area. It occurs at all ages, but is more frequent between 50-70 years of age and more common in females than in males. The symptoms of AdCC are generally unspecific and the clinical diagnosis of AdCC maybe challenging, partially due to its heterogenous histopathology and indolent growth. Moreover, there is a lack of good prognostic markers, and due to its rarity, it is difficult to predict which therapeutic methods are the most optimal for each patient, especially since very late recurrences occur. This review presents some major characteristics of AdCC and some current treatments for this disease.

[Diagnosis and treatment of subglottic mass（report of 5 cases）].

Subglottic masses is very rare. The clinical data of five cases of subglottic mass in our hospital from 2017 to 2022 were summarized, and their clinical manifestations, auxiliary examination findings, treatment plan and pathological features were analyzed. Among the 5 patients, 1 case was subglottic pleomorphic adenoma, 1 case was subglottic granuloma, 1 case was subglottic breast cancer metastasis, 1 case was subglottic primary adenoid cystic carcinoma, and 1 case was immunoglobulin G4-related disease. No recurrence was observed in the patients so far. Subglottic mass is easy to be missed. Therefore, when the lesion is suspected in this area, the examination of ear, nose and throat should be carried out systematically to detect the lesion early and improve the prognosis.

Primary pulmonary adenoid cystic carcinoma: A clinicopathological study of 64 patients.

BACKGROUND: This study aimed to investigate the clinicopathological features and prognostic indicators of primary pulmonary adenoid cystic carcinoma (PACC). METHODS: Clinical data were collected from 64 primary PACC patients and analyzed retrospectively at the Tianjin Medical University General Hospital, the West China Hospital of Sichuan University, the First Affiliated Hospital of Guangxi Medical University, and the Bishan Hospital of Chongqing Medical University from January 2003 to August 2023. The 64 patients (28 males and 36 females) were aged from 20 to 73 years, with a median age of 49 years and an average age of 49.3 years. RESULTS: Immunohistochemical staining showed that the tumors expressed CK7, S-100 protein, CK5/6, CD117, and p63. Seven patients underwent fluorescence in situ hybridization (FISH) testing and three were found to have myeloblastosis (MYB) gene translocation. In total, 53 patients underwent surgery, among whom 31 received only surgery and 22 received both surgery and postoperative chemoradiotherapy. In addition, 10 patients received chemoradiotherapy only, while one patient underwent treatment with traditional Chinese medicine. The overall survival rates in the first, third, and fifth years were 98.4%, 95.3%, and 87.5%, respectively. CONCLUSION: Prognostic analysis revealed that age, tumor size, lymph node metastasis status, margin status, and choice of treatment modality significantly influenced the patients' prognosis.

The Efficacy of Immunotherapy and Clinical Utility of Comprehensive Genomic Profiling in Adenoid Cystic Carcinoma of Head and Neck.

Background and Objectives: Adenoid cystic carcinoma (ACC) of the head and neck is generally slow-growing but has a high potential for local recurrence and metastasis to distant organs. There is currently no standard pharmacological treatment for recurrent/metastatic (R/M) ACC, and there are cases in which immune checkpoint inhibitors (ICIs) are administered for ACC according to head and neck squamous cell carcinoma (HNSCC). However, the efficacy of ICIs for ACC remains unclear, and the predictive biomarkers need to be elucidated. Materials and Methods: The Center for Cancer Genomics and Advanced Therapeutics (C-CAT) database enabled the retrospective but nationwide analysis of 263 cases of ACC of the head and neck. Then, we examined and reported four cases of ACC that received ICIs and comprehensive genomic profiling (CGP) in our institution. Results: The C-CAT database revealed that 59 cases out of 263 received ICIs, and the best response was 8% of objective response rate (ORR) and 53% of disease control rate (DCR) (complete response, CR 3%, partial response, PR 5%, stable disease, SD 44%, progressive disease, PD 19%, not evaluated, NE 29%). The tumor mutational burden (TMB) in ACC was lower overall compared to HNSCC and could not be useful in predicting the efficacy of ICIs. Some cases with MYB structural variants showed the response to ICIs in the C-CAT database. A patient with MYB fusion/rearrangement variants in our institution showed long-term stable disease. Conclusions: ICI therapy is a potential treatment option, and the MYB structural variant might be a candidate for predictive biomarkers for immunotherapy in patients with R/M ACC.

Prognostic factors for sublingual gland carcinoma: a population-based Surveillance, Epidemiology and End Results database study.

OBJECTIVE: To identify the clinical features and prognostic factors for sublingual gland carcinoma. METHODS: This was a case-control study. Patients with surgically treated sublingual gland carcinoma were retrospectively included in the Surveillance, Epidemiology and End Results database and divided into adenoid cystic carcinoma (ACC) and non-ACC groups. Primary outcome variables were disease-specific survival (DSS) and overall survival (OS). Prognostic factors for each group were analyzed using Cox models. RESULTS: We included 251 patients (115 men and 136 women). Compared with the non-ACC group, the ACC group had a larger average tumor size and received more adjuvant radiotherapy. In patients with ACC, the pathologic grade had an independent impact on DSS and OS, and patients who were undergoing adjuvant chemotherapy had worse DSS than those who were not receiving chemotherapy. In the non-ACC group, pathologic grade IV, lymph node metastasis, and adjuvant chemotherapy were associated with poor DSS and OS, and tumor extension predicted worsening DSS. CONCLUSIONS: In sublingual gland carcinoma, the pathologic grade and adjuvant chemotherapy were the most important prognostic factors, whereas lymph node metastasis had a negative impact in non-ACC patients but not in ACC patients.

Treatment and outcomes of minor salivary gland cancers of the larynx and trachea: a systematic review.

Objectives: Malignant minor salivary glands carcinomas (MiSGC) of the larynx and trachea are rare tumours and published evidence is sparse. We conducted a systematic review to describe shareable treatment strategies and oncological outcomes of these neoplastic entities. Methods: Full text English manuscripts published from January 1st 2000 to December 14th 2022 were included. Data on demographics, treatments and outcomes were collected. A pooled analysis of 5-year overall survival (OS) was performed. Results: Seventeen articles and 365 patients met the inclusion criteria. The most common subsites involved were subglottic and distal trachea. Adenoid cystic carcinoma was, by far, the most frequent histotype. The first-choice treatment strategy was surgery (86.8%), while adjuvant treatments were delivered in 57.4% of patients. Only 12.9% were treated with definitive radiotherapy with/without chemotherapy. The mean follow-up was 68.3 months. One hundred nine (34.9%) deaths were recorded and 62.4% were cancer-related. Five-year OS ranged from 20% to 100% and, at pooled analysis, it was 83% (range, 78-87%). Conclusions: In case of MiSGC of the larynx and trachea, surgery remains the mainstay of treatment. Adjuvant treatments are frequently delivered. Survival estimates are good overall, but highly heterogeneous.

[Misdiagnosis of adenoid cystic carcinoma of oropharynx: a case report].

Adenoid cystic carcinoma usually occurs in the salivary glands of the head and neck. It is a malignant tumor with a high degree of malignancy, resistance to radiotherapy and chemotherapy and poor prognosis. The clinical course of adenoid cystic carcinoma is slow and easy to be misdiagnosed. The main diagnosis and treatment means are individualized and precise treatment under the multi-disciplinary consultation mode, that is, surgical treatment and radiotherapy and chemotherapy. Adenoid cystic carcinoma is prone to relapse and hematologic metastasis, and the traditional radiotherapy and chemotherapy based therapies have not achieved satisfactory efficacy in the past three decades. How to detect, diagnose and treat early is an urgent task faced by clinicians.

Failure Patterns Within Different Histological Types in Sinonasal Malignancies: Making the Complex Simple.

OBJECTIVE: To analyze the failure patterns in patients with different histological subtypes of sinonasal malignancies (SNMs). STUDY DESIGN: Retrospectively gathered data. SETTING: Academic university hospital. METHODS: Patients with SNMs treated at a tertiary referral center between January 1999 and January 2019 were included. We assessed the failure patterns within different histological subtypes. RESULTS: The study included 897 patients. The median follow-up time was 100 months. Adenoid cystic carcinoma (ACC) had a moderate risk of developing local recurrence (LR) and distant metastasis (DM). Compared with ACC, squamous cell carcinoma (SCC), adenocarcinoma (AC), soft tissue sarcoma (STS), and mucosal melanoma (MM) were classified as a high LR risk group. For DM, neuroendocrine carcinoma (NEC), STS, and MM were in the high-risk group. CONCLUSIONS: ACC had intermediate local and distant failure risks, while SCC, AC, STS, and MM were at high LR risks. NEC, STS, and MM were at high DM risk.

A Review of the Molecular Landscape of Adenoid Cystic Carcinoma of the Lacrimal Gland.

Adenoid cystic carcinoma (ACC) has a worldwide incidence of three to four cases per million population. Although more cases occur in the minor and major salivary glands, it is the most common lacrimal gland malignancy. ACC has a low-grade, indolent histological appearance, but is relentlessly progressive over time and has a strong proclivity to recur and/or metastasise. Current treatment options are limited to complete surgical excision and adjuvant radiotherapy. Intra-arterial systemic therapy is a recent innovation. Recurrent/metastatic disease is common due to perineural invasion, and it is largely untreatable as it is refractory to conventional chemotherapeutic agents. Given the rarity of this tumour, the molecular mechanisms that govern disease pathogenesis are poorly understood. There is an unmet, critical need to develop effective, personalised targeted therapies for the treatment of ACC in order to reduce morbidity and mortality associated with the disease. This review details the evidence relating to the molecular underpinnings of ACC of the lacrimal gland, including the MYB-NFIB chromosomal translocations, Notch-signalling pathway aberrations, DNA damage repair gene mutations and epigenetic modifications.

Molecular Biology and Therapeutic Targets of Primitive Tracheal Tumors: Focus on Tumors Derived by Salivary Glands and Squamous Cell Carcinoma.

Primary tracheal tumors are rare, constituting approximately 0.1-0.4% of malignant diseases. Squamous cell carcinoma (SCC) and adenoid cystic carcinoma (ACC) account for about two-thirds of these tumors. Despite most primary tracheal cancers being eligible for surgery and/or radiotherapy, unresectable, recurrent and metastatic tumors may require systemic treatments. Unfortunately, the poor response to available chemotherapy as well as the lack of other real therapeutic alternatives affects the quality of life and outcome of patients suffering from more advanced disease. In this condition, target therapy against driver mutations could constitute an alternative to chemotherapy, and may help in disease control. The past two decades have seen extraordinary progress in developing novel target treatment options, shifting the treatment paradigm for several cancers such as lung cancer. The improvement of knowledge regarding the genetic and biological alterations, of major primary tracheal tumors, has opened up new treatment perspectives, suggesting the possible role of biological targeted therapies for the treatment of these rare tumors. The purpose of this review is to outline the state of knowledge regarding the molecular biology, and the preliminary data on target treatments of the main primary tracheal tumors, focusing on salivary-gland-derived cancers and squamous cell carcinoma.

Management and outcome of adenoid cystic carcinoma of the major salivary glands: the 22-year experience of a single institution.

BACKGROUND: Prognostic factors and survival rate are difficult to determine for adenoid cystic carcinoma(AdCC) of salivary glands. AIMS/OBJECTIVES: To clarify the clinical characteristics of AdCC and examine factors associated with recurrence and prognosis by histopathological grade classification. MATERIALS AND METHODS: Twenty-five patients with AdCC of the parotid gland and 10 patients with AdCC of the submandibular gland were included. We classified AdCC histopathologically by the proportion of solid components. Clinical features, fine-needle aspiration cytology (FNAC), and patient outcomes were examined according to grade. Factors associated with local recurrence and distant metastases were examined. RESULTS: Age was significantly higher in the grade III group than in the grade I group. The grade III group had significantly higher proportions of patients with cN+, pN+, and perineural invasion. In FNAC, lower-grade groups showed higher rates of correct histopathological type. Five-year disease-specific survival and disease-free survival rates were significantly lower in the grade III than in the grade I. Distant metastases were more common among patients with high-stage and perineural invasion. CONCLUSIONS: Five-year survival is significantly worse in patients with grade III.

Pulmonary adenoid cystic carcinoma: molecular characteristics and literature review.

BACKGROUND: Pulmonary adenoid cystic carcinoma (PACC) is an exceptionally rare salivary gland-type malignant neoplasm. Because of its clinical manifestations, imaging features are not different from other types of non-small cell lung cancer, which is a diagnostic challenge for most doctors. CONCLUSIONS: A review of the literature shows that high amounts of immunohistochemical (IHC) markers, such as CK7, CD117, P63, SMA, CK5/6, and S-100 are helpful for PACC diagnosis. Surgical resection is the main treatment of PACC, but treatment options for advanced PACC patients are limited and the research of molecular targeted drugs is ongoing in advanced cases not eligible for surgery. Currently, research on PACC targeted therapy mainly focuses on the exploration of v-myb avian myeloblastosis virus oncogene homolog (MYB) and its downstream target genes. In addition, median tumor mutation burden and PD-1/PD-L1 were lower in PACC, which may indicate poor efficacy of immunotherapy in PACC patients. This review focuses on the pathologic features, molecular characteristics, diagnosis, treatment and prognosis of PACC to establish a comprehensive understanding of PACC.

A Rare Nasopharyngeal Adenoid Cystic Carcinoma: Case Report and Literature Review.

BACKGROUND Adenoid cystic carcinoma (ACC) is a malignant tumor that involves the salivary glands and is rarely seen in the nasopharynx. It is characterized by pain, a high rate of recurrence, and poor survival rate and poor prognosis as it tends to invade the perineurium. Due to the ambiguity of patient presentations, most patients present at a late stage, at which point metastasis has already occurred. CASE REPORT The patient in this case report was an otherwise healthy 56-year-old male smoker who presented to the clinic in March 2022 complaining of nasal obstruction, mouth breathing, recurrent epistaxis, headache, and facial pain. He reported significant weight loss in the past 6 months. The nasal scope showed nasopharyngeal fullness and biopsy-confirmed ACC exhibiting tubular, solid, and focal cribriform growth patterns. Further work-up showed an advanced form of nasopharyngeal ACC with intracranial extension and lung metastasis. The patient received concurrent radiotherapy and chemotherapy. After 6 months, the patient reported an improvement in the pain, and repeated images showed regression of the tumor size. CONCLUSIONS ACC is a malignant disease that is rarely seen in the nasopharynx. Currently, the most commonly agreed upon management for resectable ACCs in the literature is through surgical resection with adjuvant radiotherapy. However, most of the reported cases received radiation and chemotherapy due to the difficulty and risk of radical surgical excision of nasopharyngeal malignant tumors. Patients should be followed up to rule out any local recurrence or distant metastasis.

Pathological nodal status as a main predictive factor of survival and treatment outcomes of submandibular salivary gland cancers: A 25-year single center experience.

INTRODUCTION: Aim of this study was to explore the survival predictive factors and treatment outcomes in a cohort of SGC patients treated at a single center over a period of 25 years. MATERIALS AND METHODS: Patients who had received primary treatment for SGC were enroled. Outcomes evaluated were: overall survival (OS), disease specific survival (DSS), recurrence free survival (RFS), locoregional recurrence free survival (LRFS) and distant metastasis free survival (DFS). RESULTS: A total of 40 patients with SGC were enroled in the study. The most common tumor was the adenoid cystic carcinoma (60% of cases). Cumulative OS for 5-and 10-year follow up period was 81% and 60%, respectively. Thirteen patients (32.5%) developed distant metastases during follow-up. Nodal status, high-grade histology, tumor stage and adjuvant radiation-therapy (RT) were significant variables on multivariate analysis for survival and treatment outcomes. CONCLUSIONS: Submandibular gland carcinomas represent rare and heterogenous tumor group regarding histological appearance and locoregional and distant metastatic potential. Tumor histological grade, AJCC tumor stage and nodal status were the strongest predictive factors for survival and treatment outcomes. RT improved OS and locoregional treatment outcome, but not DFS. Elective neck dissection (END) could be beneficial for selected cases of SGC. Superselective neck dissection of levels I-IIa may be the level of dissection for END. Distant metastases were the main cause of death and treatment failure. Prognostic factors for poor DMFS were AJCC stage III and IV, high tumor grade and nodal status.

Prognostic Model and Nomogram for Early-Onset Adenoid Cystic Carcinoma of Head and Neck: A Retrospective SEER-Based Analysis.

BACKGROUND: Adenoid cystic carcinoma of head and neck (ACCHN) is an uncommon head and neck cancers, whose predilection age is 40 to 60. Some studies have revealed that early-onset cancers, such as colorectal cancers and esophageal adenocarcinoma, might present some unique clinicopathological features and have different prognosis with late-onset ones. However, little is known about the early-onset ACCHN. This study aimed to develop a prognostic nomogram for overall survival (OS) of patients younger than 40 with ACCHN. METHODS: Cases with ACCHN from 1975 to 2016 were retrieved from SEER-18 program. Demographic, clinical, and survival outcomes data of patients were identified for further analysis. The caret package was used to randomly divide early-onset patients into a training cohort and a validation cohort. A prognostic nomogram was constructed based on the univariate and multivariate Cox analysis. The discriminative ability and calibration power of the nomogram was evaluated by the concordance index (C-index), calibration curve, and receiver operating characteristic (ROC) curve. RESULTS: A total of 5858 cases with ACCHN were selectively retrieved from SEER program in this study. The number of patients younger than 40, which was defined as early-onset ACCHN in this study, was 825. Based on the outcomes of multivariate analysis, tumor size, chemotherapy, surgery, and stage were selected for the construction of nomogram to predict 10-year OS. The C-index was 0.792 (95%CI 0.760-0.823) and 0.776 (95%CI 0.720-0.832) in the training and validation set, respectively. The area under the ROC curve values were 0.875 (95%CI 0.810-0.940) and 0.833(95%CI 0.754-0.912). The calibration plot indicated that this nomogram had proper calibration in both the training and validation cohorts. CONCLUSION: A novel prognostic nomogram for early-onset ACCHN was constructed and validated in this study. This nomogram could be applied for assisting clinicians to more accurately assess the prognosis of young patients, which might facilitate clinical decision-making and subsequent follow-up.

Malignant salivary gland tumours: treatment outcomes from a tertiary referral centre in the UK.

Salivary gland malignant tumours are a complex and highly variable pathological group. Their diagnosis can be challenging, and management is guided by multidisciplinary teams. This project aimed to establish clinicopathological and sociodemographic features that significantly impacted overall disease-free or progression-free survival in patients diagnosed with malignant salivary gland disease between 2010 and 2019 in a tertiary referral centre. In total, 86 patients were included for analysis, with a female:male gender ratio of 1.3:1. Mean age at diagnosis was 57.7 years. Mucoepidermoid carcinomas constituted almost 25% (n = 20) of all cases, with adenoid cystic carcinomas (20%, n = 17) and acinic cell carcinomas (17.5%, n = 15) being the next most frequently diagnosed. The parotid gland was the most frequently affected site (80.2%, n = 69). Perineural and lymphovascular invasion, and a maximum tumour dimension of ≥4 cm were highly associated with the decision to provide a neck dissection as part of treatment. Involved margins, extracapsular spread, and lymphovascular and perineural invasion were associated with the need for adjuvant treatment. However, no factors remained statistically significant on multivariate analysis. This retrospective service evaluation demonstrates the difficulty of predicting treatment outcomes for patients diagnosed with malignant salivary gland disease.

Epidemiology, outcomes, and prognostic factors in submandibular gland carcinomas: a national DAHANCA study.

PURPOSE: The aim of this study is to present incidence, histological subtypes, survival rates, and prognostic factors based on a national cohort of patients with salivary gland carcinoma. METHODS: All Danish patients with submandibular gland carcinoma diagnosed from 1990 to 2015 (n = 206) were included and analyzed following histological re-evaluation. Data were collected by the Danish Head and Neck Cancer Group (DAHANCA). Overall, disease-specific and recurrence-free survival were evaluated. Prognostic factors were analyzed with multivariate Cox Hazard Regression. RESULTS: The study population consisted of 109 (53%) men and 97 (47%) women, median age 62 years (range 11-102). Adenoid cystic carcinoma was the most frequent subtype (50%). Tumour classification T1/T2 (75%) and N0 (78%) was most frequent. The mean crude incidence was 0.17/100,000/year. Most patients (n = 194, 94%) were treated with primary surgery, and 130 (67%) received postoperative radiotherapy. The 5- and 10-year survival rates were for overall survival 64% and 41%, disease-specific survival 74% and 61%, and recurrence-free survival 70% and 56%, respectively. Survival rates were higher for adenoid cystic carcinoma compared to other subtypes, but the difference was not significant in multivariate analysis. Recurrence occurred in 69 patients, and 37 (53.6%) of them had recurrence in a distant site. Advanced T-classification and regional lymph-node metastases had significant negative impact on survival rates. CONCLUSION: The incidence of submandibular gland carcinoma in Denmark was 0.17/100,000/year and stable during the time period. The most frequent subtype was adenoid cystic carcinoma. Half of the recurrences presented in a distant site, and multivariate analysis confirmed that advanced stage was independent negative prognostic factor for recurrence and survival.

[Primary salivary gland tumors from a pathology perspective : Morphomolecular peculiarities and diagnostic and therapeutic challenges]. / Primäre Speicheldrüsentumoren aus Sicht der Pathologie : Morphomolekulare Besonderheiten und diagnostisch-therapeutische Herausforderungen.

Similar to tumors of other organs, salivary gland neoplasms were historically viewed as a single neoplastic entity and mostly treated as such. Accordingly, only the clinical tumor stage, and not the histological subtype, was considered to be of significant prognostic impact. However, over the years, several distinct sub-entities have been characterized based on morphological features, such as adenoid cystic carcinoma, mucoepidermoid carcinoma, acinic cell carcinoma, and salivary duct carcinoma. Most importantly, the nosology of salivary gland carcinomas has undergone a dynamic "splitting" on the basis of morphological, immunophenotypic, and molecular characteristics, so that 21 independent carcinomas are now listed in the current World Health Organization (WHO) classification. Moreover, it has become evident that splitting of these carcinoma subtypes no longer represents a "pathologist's hobby," but carries significant prognostic and therapeutic relevance for optimized cancer surgery and potentially systemic therapy. The current review summarizes the major features of salivary gland tumors, both benign and malignant, and gives an account of their classification systems and genetic profiles.