| Abstract: |
Background Based on their distinctive histologic and genetic features, the latest WHO classification of soft tissue tumors includes four pathologic variants of rhabdomyosarcoma (RMS): embryonal (ERMS), alveolar (ARMS), spindle cell-sclerosing (SRMS-ScRMS) and pleomorphic RMS. The aim of this study focused on a detailed clinicopathologic and survival analysis of head and neck RMS (HNRMS) using the latest pathologic and molecular criteria reflecting this new subclassification in a large cohort. Patients and methods Patients managed for HNRMS in our institution (1996–2015) were analyzed. The presence of a FOXO1 fusion was required for the classification of ARMS. MYOD1 mutations in SRMS-ScRMS were tested when material available. Univariate and multivariate analyses were performed to evaluate variables related to overall survival (OS). Results Ninety-nine HNRMS patients (52 males and 47 females, mean of 16 years) were included in the study after pathologic re-review. The most common location was parameningeal (PM) (n = 64), followed by non-orbital/non-PM (n = 25) and orbital (n = 10). There were 53 ERMS, 33 fusion-positive ARMS and 13 SRMS-ScRMS [SRMS (8); ScRMS (5)]. The 5-year OS rate for ERMS patients was significantly higher (82%) compared to ARMS (53%) and SRMS-ScRMS (50%) [SRMS (75%); ScRMS (30%)]. Univariate analysis showed that survival was dependent on histology (P = 0.012), tumor size >5 cm (P < 0.001), regional lymph node involvement (P = 0.002), metastasis at initial presentation (P < 0.001), stage (P < 0.001), and recurrence (P = 0.002). Multivariate analysis confirmed histologic subtype to be significant (P = 0.043). Conclusion Our findings reinforce that HNRMS is a heterogenous disease with ARMS and SRMS-ScRMS having an equally unfavorable outcome. © 2016 Elsevier Ltd |
