Clinicopathologic features and integrated outcome analysis of pediatric and young adult salivary secretory carcinoma
Source: PubMed Central Open Access, NCBI / U.S. National Library of Medicine
Background Salivary secretory carcinoma (SSC) is a rare salivary gland malignancy characterized by recurrent ETV6-related gene fusions and generally classified as low grade. However, age-specific outcome data in pediatric and young adult patients remain limited. The present study aimed to characterize clinicopathologic features and clinical outcomes of SSC in younger patients and to contextualize these findings through an integrated analysis of published cases. Methods We retrospectively analyzed 35 patients with SSC treated at a single institution between 2019 and 2023, including 11 individuals aged ≤29 years. Clinical, histopathologic, immunohistochemical, molecular, and outcome data were evaluated. A focused literature search identified 47 eligible publications comprising 83 reported pediatric and young adult cases, which were incorporated into a descriptive integrated analysis. Results all younger patients presented with parotid tumors and exhibited a higher median Ki-67 labeling index compared with older patients (15.0% vs 7.5%). Among 50 evaluable published cases, recurrence occurred in 9 patients, corresponding to a pooled recurrence rate of 18.0% (95% CI 7.4–28.6%). Lymph node metastasis was documented in 10.0% of evaluable cases. The recurrence rate observed in our young subgroup (18.2%) was consistent with pooled estimates. Conclusions SSC in pediatric and young adult patients may demonstrate a broader biological spectrum than traditionally assumed. Although general
Abstract
Background Salivary secretory carcinoma (SSC) is a rare salivary gland malignancy characterized by recurrent ETV6-related gene fusions and generally classified as low grade. However, age-specific outcome data in pediatric and young adult patients remain limited. The present study aimed to characterize clinicopathologic features and clinical outcomes of SSC in younger patients and to contextualize these findings through an integrated analysis of published cases. Methods We retrospectively analyzed 35 patients with SSC treated at a single institution between 2019 and 2023, including 11 individuals aged ≤29 years. Clinical, histopathologic, immunohistochemical, molecular, and outcome data were evaluated. A focused literature search identified 47 eligible publications comprising 83 reported pediatric and young adult cases, which were incorporated into a descriptive integrated analysis. Results all younger patients presented with parotid tumors and exhibited a higher median Ki-67 labeling index compared with older patients (15.0% vs 7.5%). Among 50 evaluable published cases, recurrence occurred in 9 patients, corresponding to a pooled recurrence rate of 18.0% (95% CI 7.4–28.6%). Lymph node metastasis was documented in 10.0% of evaluable cases. The recurrence rate observed in our young subgroup (18.2%) was consistent with pooled estimates. Conclusions SSC in pediatric and young adult patients may demonstrate a broader biological spectrum than traditionally assumed. Although generally considered low grade, a measurable subset of younger patients exhibits recurrent or metastatic behavior, underscoring the importance of systematic outcome assessment in this population.