Secretory Carcinoma of the Salivary Gland: A Rarity in Children

Kelly, Gillean A; Venkatramani, Rajkumar; Quintanilla, Norma; Chelius, Daniel C.; Roy, Angshumoy; Mahajan, Priya

doi:10.1097/mph.0000000000002304

Cited by 7 publications

(7 citation statements)

References 39 publications

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“…Out of a total of 226 studies published between 2010 and 2023, identified at initial search and screened for retrieval, 112 1–3,6–114 articles were included in the study, with a total of 674 patients described in these articles. The identification and screening process is described in Figure 1.…”

Section: Resultsmentioning

confidence: 99%

Salivary Gland Secretory Carcinoma; Review of 13 Years World‐Wide Experience and Meta‐Analysis

Yosefof,

Boldes,

Dan

et al. 2023

The Laryngoscope

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ObjectivesSecretory Carcinoma is a malignant salivary gland tumor, initially described in 2010. This rare tumor is associated with the translocation t(12;15) (p13;q25), resulting in the fusion gene ETV6‐NTRK3. Since this tumor is quite rare, most publications describe only small cohorts of patients. We aimed to investigate the clinical, pathological, and prognostic features of this newly defined malignant entity.Data SourcesPubmed, Google Scholar, and Web of Science databases.Review MethodsAll published articles between 2010 and 2023 were reviewed. Search terms included the terms “Mammary Analogue Secretory Carcinoma” and “Secretory Carcinoma”. All articles published in English reporting on Secretory Carcinoma of salivary glands were retrieved.ResultsOne‐hundred and 12 retrospective articles reporting a total of 674 patients were included, with 52% males and a mean age of 44.9 ± 18.9. The event rate for patients with advanced‐stage disease (Stage 3/4) at presentation was 24.1% (95% CI 17.6%–31.9%, I2 = 9.2%), 14.6% for regional metastases (95% CI 10.5%–20%, I2 = 12%), and the event rate of distant metastasis was 8.4% (95% CI 5.5%–12.7%, I2 = 4.2%). Adjuvant radiotherapy was administered for 30.3% of patients (95% CI 24.1%–37.2%, I2 = 21.5%). The recurrence rate was 19% (95% CI 15.1%–23.8%, I2 = 5%). Survival outcomes showed a 17.2% death of disease rate for Secretory Carcinoma patients (95% CI 13.5%–21.8%, I2 = 7.3%).ConclusionsSecretory Carcinoma is a rare and relatively newly defined entity arising in the parotid gland most commonly. Characterized as a low‐grade tumor, the majority of patients are diagnosed at an early stage, without regional or distant disease, and the prognosis is relatively good.Level of EvidenceN/A Laryngoscope, 2023

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Section: Resultsmentioning

confidence: 99%

Salivary Gland Secretory Carcinoma; Review of 13 Years World‐Wide Experience and Meta‐Analysis

Yosefof,

Boldes,

Dan

et al. 2023

The Laryngoscope

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“…Secretory carcinoma is rare and occurs mainly in the parotid gland. 12 , 19 Our study included three cases and one was preceded by bone marrow transplantation, suggesting that bone marrow transplantation might be a risk factor. Myoepithelial carcinoma may be found in the salivary glands and has also been reported in the soft tissues of the trunk and extremities.…”

Section: Discussionmentioning

confidence: 99%

Head and neck carcinoma in children: A clinicopathological study of 42 cases

Cai

Tang

Liu

et al. 2023

Journal of Dental Sciences

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“…The diagnosis was ultimately confirmed by the detection of the ETV6-NTRK3 fusion gene using NGS analysis. To the best of our knowledge, this is the youngest patient reported with a diagnosis of SC and represents the longest follow-up period (30 years) documented in the literature [ 10 , 12 , 13 , 18 , 19 ]. Among children, mucoepidermoid carcinoma is the most commonly diagnosed malignant tumour of the salivary gland [ 20 ], while literature on the occurrence of SC is primarily limited to individual case reports [ 18 ].…”

Section: Discussionmentioning

confidence: 99%

“…To the best of our knowledge, this is the youngest patient reported with a diagnosis of SC and represents the longest follow-up period (30 years) documented in the literature [ 10 , 12 , 13 , 18 , 19 ]. Among children, mucoepidermoid carcinoma is the most commonly diagnosed malignant tumour of the salivary gland [ 20 ], while literature on the occurrence of SC is primarily limited to individual case reports [ 18 ]. In a comprehensive literature review comprising 119 original studies and a total of 642 patients with SC of the salivary gland, the youngest patient at diagnosis was 5 years old [ 10 ].…”

Section: Discussionmentioning

confidence: 99%

Secretory carcinoma of major and minor salivary glands with ETV6-NTRK3 gene fusion: overcoming misdiagnosis in the era of tumour-agnostic therapy with TRK inhibitors

Durzyńska,

Dominiak,

Sosnowska

et al. 2023

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Introduction Secretory carcinoma (SC) of the salivary gland is an extraordinarily rare tumour. Accurate diagnosis of SC is crucial for understanding the clinical course, prognosis, and selection of optimal therapy. The aim of this research was to analyse retrospectively the clinical and pathological characteristics of patients diagnosed with SC of the salivary gland from 2017 onwards, which aligns with its addition to the World Health Organization classification. Material and methods We conducted a retrospective, single-centre, clinicopathological analysis of patients diagnosed with SC of the salivary gland between 2017 and 2022. The analysis included the evaluation of NTRK3 gene rearrangements and immunohistochemical (IHC) profiling. Results The study included 6 patients, comprising 4 women and 2 men. The average age of the patients was 50 years (standard deviation 26). Three cases presented with tumours in the parotid gland, while one case each involved the submandibular gland, sinonasal tract, and buccal mucosa. Interestingly, despite the characteristic IHC profile, each case was initially diagnosed as a different type of salivary gland cancer. Next-generation sequencing analysis was performed in 3 cases, revealing the presence of the ETV6-NTRK3 fusion gene. This cohort notably features an intriguing case: the youngest patient documented in literature, distinguished by extended follow-up and delayed recurrence. Conclusions In summary, emphasizing the risk of misdiagnosis is pivotal in the context of SC of the salivary gland, which can manifest across diverse glandular sites. Accurate diagnosis, underscored by the assessment of NTRK3 gene rearrangements, assumes a critical role in guiding effective management and treatment decisions.

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Secretory Carcinoma of the Salivary Gland: A Rarity in Children

Cited by 7 publications

References 39 publications

Salivary Gland Secretory Carcinoma; Review of 13 Years World‐Wide Experience and Meta‐Analysis

Salivary Gland Secretory Carcinoma; Review of 13 Years World‐Wide Experience and Meta‐Analysis

Head and neck carcinoma in children: A clinicopathological study of 42 cases

Secretory carcinoma of major and minor salivary glands with ETV6-NTRK3 gene fusion: overcoming misdiagnosis in the era of tumour-agnostic therapy with TRK inhibitors

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