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To determine the indications and modalities for resection in the management of primary sublingual and minor salivary gland cancer, and the specific features of each primary location. The French Network of Rare Head and Neck Tumors (REFCOR) formed a steering group who drafted a narrative review of the literature published on Medline and proposed recommendations. The level of adherence to the recommendations was then assessed by a rating group, according to the formal consensus method. Histological evidence (submucosal biopsy) is recommended before surgical treatment of minor salivary gland carcinoma. Surgical treatment is recommended, with optimal oncologic margins, adapted to anatomical factors, histologic type and grade and functional consequences, with reconstruction if necessary. Treatment of primary minor salivary and sublingual gland cancer is surgical, with wide resection margins. The modalities of resection and reconstruction are highly dependent on tumor location, extension and histologic type. [ABSTRACT FROM AUTHOR]

To determine the therapeutic indications for systemic medical treatment in the management of adenoid cystic carcinoma (ACC) according to the clinical situation. The French Network of Rare Head and Neck Tumors (REFCOR) formed a steering group, which drafted a narrative review of the literature published on Medline and proposed recommendations. The level of adherence to the recommendations was then assessed by a rating group, according to the formal consensus method. ACCs are rare tumors and there is currently insufficient evidence to indicate chemotherapy at the localized stage. At the metastatic stage, progression is often slow. In case of oligometastatic ACC, local treatment should be discussed. The most often indolent nature of polymetastatic ACC can allow management by active surveillance. Molecular screening is recommended, for abnormalities potentially accessible to targeted therapy. ACCs are rare tumors for which there are currently few effective medical treatments. It is therefore recommended to include patients in clinical trials. [ABSTRACT FROM AUTHOR]

To determine the therapeutic indications for systemic medical treatment in the management of salivary gland carcinoma (excluding adenoid cystic carcinoma) according to the clinical situation. The French Network of Rare Head and Neck Tumors (REFCOR) formed a steering group who drafted a narrative review of the literature published on Medline and proposed recommendations. The level of adherence to the recommendations was then assessed by a rating group, according to the formal consensus method. Salivary gland carcinoma is rare and there is currently insufficient evidence to indicate chemotherapy at the localized stage. At the metastatic stage, initial management can be based on a phase of monitoring for indolent disease. Some histological subtypes (salivary duct carcinoma and adenocarcinoma) are more aggressive and require systemic treatment from the outset. To guide systemic treatment, it is recommended to perform immunohistochemistry and molecular biology analyses (overexpression of HER2 and androgen receptors, NTRK fusion, next-generation sequencing). Salivary gland carcinoma is a rare tumor for which there are currently few effective medical treatments. It is therefore recommended to include patients in clinical trials. [ABSTRACT FROM AUTHOR]

To determine the indications for radiotherapy in salivary gland cancer and to specify the modalities and target radiation volumes. The French Network of Rare Head and Neck Tumors (REFCOR) formed a steering group which drafted a narrative review of the literature published on Medline and proposed recommendations. The level of adherence to the recommendations was then assessed by a rating group, according to the formal consensus method. Postoperatively, radiotherapy to the primary tumor site ± to the lymph nodes is indicated if one or more of the following adverse histoprognostic factors are present (risk > 10% of locoregional recurrence): T3–T4 category, lymph node invasion, extraglandular invasion, close or positive surgical margins, high tumor grade, perineural invasion, vascular emboli, and/or bone invasion. Intensity-modulated radiation therapy (IMRT) is the gold standard. For unresectable cancers or inoperable patients, carbon ion hadrontherapy may be considered. Radiotherapy in salivary gland cancer is indicated in postoperative situations in case of adverse histoprognostic factors and for inoperable tumors. [ABSTRACT FROM AUTHOR]

To determine the indications for neck dissection in the management of parotid, submandibular or minor salivary gland cancers depending on the clinical situation: i.e., clinical lymph node involvement (cN+) or not (cN0); low or high risk of occult nodal metastasis; diagnosis of malignancy before, during or after surgery. The French Network of Rare Head and Neck Tumors (REFCOR) formed a steering group which drafted a narrative review of the literature published on Medline and proposed recommendations. The level of adherence to the recommendations was then assessed by a rating group according to the formal consensus method. In cN+ salivary gland cancer, ipsilateral neck dissection is recommended. In cN0 salivary gland cancer, ipsilateral neck dissection is recommended, except for tumors at low risk of occult nodal metastasis. If definitive pathology reveals a high risk of occult nodal involvement, additional neck treatment is recommended: ipsilateral neck dissection or elective nodal irradiation. The rate of occult lymph node involvement, and therefore the indication for elective neck dissection, depends primarily on the pathologic grade of the salivary gland cancer. [ABSTRACT FROM AUTHOR]

To determine the frequency and modality of post-treatment monitoring of primary salivary gland cancer. The French Network of Rare Head and Neck Tumors (REFCOR) formed a steering group who drafted a narrative review of the literature published on Medline and proposed recommendations. The level of adherence to the recommendations was then assessed by a rating group, according to the formal consensus method. Clinical monitoring should be adapted to the risk of recurrence: initially every 3 months and progressively spaced out, becoming annual after 5 years. Post-treatment head and neck and chest imaging is recommended at 3 months. Local and regional monitoring can then be carried out yearly or twice yearly with contrast-enhanced head and neck imaging. An annual chest CT scan is recommended for high-grade tumors. For lesions at high risk of late recurrence, very prolonged annual surveillance (up to 15 years) is recommended, including screening for pulmonary metastases. Given the wide range of malignant salivary gland tumors, the modalities and frequency of post-treatment monitoring must be adapted to the expected course of the disease. [ABSTRACT FROM AUTHOR]

To determine the role of surgery of the primary tumor site in the management of primary major salivary gland cancer. The French Network of Rare Head and Neck Tumors (REFCOR) formed a steering group, which drafted a non-systematic narrative review of the literature published on Medline, and proposed recommendations. The level of adherence to the recommendations was then assessed by a rating group, according to the formal consensus method. Treatment of salivary gland tumor is mainly surgical. The gold standard for parotid cancer is a total parotidectomy, to obtain clear margins and remove all intraparotid lymph nodes. For low-grade tumors, partial parotidectomy with wide excision of the tumor is acceptable in the case of postoperative diagnosis on definitive histology. In the event of positive margins on definitive analysis, revision surgery should be assessed for feasibility, and performed if possible. Treatment of primary major salivary gland cancer is based on surgery with clear resection margins, as far away as possible from the tumor. The type of surgery depends on tumor location, pathologic type and extension. [ABSTRACT FROM AUTHOR]

To determine the indications for fine-needle cytology and the modalities of frozen section pathological analysis in the management of salivary gland cancer. The French Network of Rare Head and Neck Tumors (REFCOR) formed a steering group who drafted a narrative review of the literature published on Medline and proposed recommendations. The level of adherence to the recommendations was then assessed by a rating group according to the formal consensus method. Fine-needle cytology is recommended as part of the diagnostic work-up for a major salivary gland tumor suspicious for malignancy. Fine-needle cytology should be performed after MRI to avoid artifacts. Frozen section analysis is recommended to confirm the malignant nature of the tumor, to adapt the extent of resection and to indicate neck dissection. Whenever possible, the entire tumor and adjacent salivary or periglandular tissue should be sent for frozen section analysis. Fine-needle cytology and frozen section analysis play an essential role in the management of salivary gland cancers. [ABSTRACT FROM AUTHOR]

To define the indications for each imaging modality in the screening, characterization, extension and follow-up of salivary gland tumors. The French Network of Rare Head and Neck Tumors (REFCOR) formed a steering group who drafted a narrative review of the literature published on Medline and proposed recommendations. The level of adherence to the recommendations was then assessed by a rating group, according to the formal consensus method. If a swelling of a salivary gland is palpable for 3 weeks, an ultrasound scan is recommended to confirm a tumoral lesion and rule out differential diagnoses. For a salivary gland tumor, MRI is recommended with diffusion-weighted and dynamic contrast-enhanced techniques. In the case of histologically proven malignancy or a highly suspicious lesion, a CT scan of the neck and chest is recommended to assess the tumor, lymph nodes and metastases. FDG-PET is not currently recommended in routine clinical practice for initial diagnosis, assessment of extension, evaluation of response to treatment, staging of recurrence, or follow-up of salivary gland tumors. Assessing salivary tumors is based on MRI. Extension assessment is based on neck and chest CT. [ABSTRACT FROM AUTHOR]

Objective Salivary gland cytology is challenging because it includes a diversity of lesions and a wide spectra of tumours. Recently, it has been reported that many types of salivary gland tumours have specific molecular diagnostic signatures that could be identified by fluorescent in-situ hybridisation (FISH). The aim of the present study was to demonstrate the feasibility and efficiency of FISH on routine cytological salivary gland smears. Methods FISH was conducted on 37 cytological salivary gland smears from 34 patients. According to the cytological diagnosis suspected, MECT1/ MAML2 gene fusion and rearrangements of PLAG1, MYB, or ETV6 were analysed. The presence and percentages of cells that had gene rearrangements were evaluated. Results were compared with the histological surgical samples, available from 26 patients. Results The PLAG 1 rearrangement was observed in 12/20 (60%) cases of pleomorphic adenoma. MECT1/ MAML2 gene fusion was observed in 1:2 mucoepidermoid carcinomas but was not observed in five other tumours (two pleomorphic adenomas, one Warthin's tumour, one mammary analogue secretory carcinoma [ MASC] and one cystic tumour). MYB rearrangement was observed in 4/4 adenoid cystic carcinomas. ETV6-gene splitting identified one MASC. Conclusion Overall, FISH had a specificity of 100% and a sensitivity of 66.7%. When FISH and cytological analyses were combined, the overall sensitivity was increased to 93.3%. It can thus be concluded that when the FISH analysis is positive, the extent of surgery could be determined with confidence pre-operatively without needing a diagnosis from a frozen section. [ABSTRACT FROM AUTHOR]