Adult rhabdomyosarcoma

Abstract

BACKGROUNDChildhood rhabdomyosarcoma (RMS) has a relatively good prognosis. Outcome for adults with this disease is poorly documented due to its rarity.METHODSThe clinicopathologic features, treatment methods, and disease outcome were reviewed retrospectively for 82 adults with locoregional RMS treated between 1960 and 1998. Patients with distant metastasis at diagnosis were excluded. Actuarial univariate and multivariate statistical methods were used to evaluate outcome.RESULTSPatient ages ranged from 17 to 84 years (median, 27 years). Histologic subtypes were embryonal (34%), pleomorphic (43%), and alveolar (23%). Anatomic sites of origin were head and neck (52%), trunk (26%), and extremity (7%). Tumor size was 5 cm or smaller in 51% of patients. Regional lymph node metastasis was present in 33% of patients at presentation. Treatment consisted of radiation alone in 11%, radiation and surgery in 18%, radiation and chemotherapy in 34%, and all three modalities in 37%. With a median follow‐up of 10.5 years, the 10‐year actuarial disease‐free and overall survival rates were 41% and 40%, respectively. The 10‐year actuarial local, lymph node, and metastatic control rates were 75%, 82%, and 53%, respectively. The major determinant of metastatic control and survival was primary tumor size (≤ 5 vs. > 5 cm). Local control was satisfactory (10‐year rate of 87%) for sites other than parameningeal (50% at 10 years). Patients whose disease responded to chemotherapy had a significantly better metastasis free period (72% at 10 years) than those whose disease failed to respond (19% at 10 years).CONCLUSIONSAdult RMS is a highly malignant tumor with a significant incidence of metastatic recurrence. Continuing investigation of new and potentially more effective chemotherapy is crucial. Local control is satisfactory for sites other than parameningeal where new radiation technologies such as intensity‐modulated therapy may be necessary to safely deliver adequate doses. Cancer 2002;95:377–88. © 2002 American Cancer Society.DOI 10.1002/cncr.10669

Outcome of 449 adult patients with rhabdomyosarcoma: an observational ambispective nationwide study

“…The relapse rate is consistent with that reported in the literature: 33–57% local relapses 11, 12, 13, 14, 19 and up to 48–68% metastatic relapse 14, 16, 19. It is observed often during the first year, leading to death in 6–12 months 11, 12, 14, 15, 16, 17, 19, with median survival of 9, 8, and 7 months for patients with local relapse, metastatic relapse, or both in the present work. This outcome is notably inferior to that achieved in other ASTS of adults in 2016.…”

“…These results are consistent with those described in the literature. RMS occurs frequently in men and young adults: 37 versus 26 years in other studies and increased by twofold in P‐RMS 11, 12, 13, 14, 15, 16, 17. HN and extremities tumors represent the main primary site in this study and previous reports 11, 12, 16, 17.…”

“…RMS occurs frequently in men and young adults: 37 versus 26 years in other studies and increased by twofold in P‐RMS 11, 12, 13, 14, 15, 16, 17. HN and extremities tumors represent the main primary site in this study and previous reports 11, 12, 16, 17. Synchronous metastases were observed in 20% versus 17–44% in previous studies 11, 13, 14, 15, 16, 17.…”

“…LN involvement was uncommon (14%) compared with other series (33–46%) 11, 16, 17. This finding is likely related to the prevalence of P‐RMS: 44% versus 9–20% with the exception of the Little series focused on localized RMS 12 (Table 5). In this present work, the clinical presentation is variable according to the histological subtype and is generally worse than that of the pediatric population with RMS.…”

“…In contrast, the outcome of adult patients remains poor. Furthermore, given the rarity of adult RMS, limited information is available in the literature, and optimal management and identification of prognostic factors (PF) have not been established in large cohorts, in particular in nationwide studies 11, 12, 13, 14.…”