Outcomes for patients with perineal and perianal rhabdomyosarcoma: A report from the Children's Oncology Group Soft Tissue Sarcoma Committee Journal Article
| Authors: | Dasgupta, R.; Xue, W.; Dixon, A. H.; Wolden, S.; Yock, T. I.; Venkatramani, R.; Rodeberg, D. A. |
| Article Title: | Outcomes for patients with perineal and perianal rhabdomyosarcoma: A report from the Children's Oncology Group Soft Tissue Sarcoma Committee |
| Abstract: | Purpose: To describe clinical features, risk factors, and outcomes of patients with perineal and perianal rhabdomyosarcoma. Methods: The records of 51 patients (38 perineal and 13 perianal) enrolled on Children's Oncology Group clinical trials between 1997 and 2012 were reviewed. Results: At presentation, 53% were female, 65% were older than 10 years of age, 76% were alveolar histology, 76% were more than 5 cm, 84% were invasive, 65% were regional node positive by imaging, 49% were metastatic, only 16% were grossly resected upfront, and 25% of patients had a delayed excision. At a median follow-up of 6.13 years, estimated 5-year event-free survival (EFS) was 38% [22.17%–53.38%], and overall survival (OS) was 42% [26.66%–58.21%]. The rates of local, regional, and distant failure were 15.6%, 13.7%, 43.1%, respectively; all failures ultimately died. By univariate analysis, only age more than 10 years negatively impacted 5-year EFS (p =.023) and OS (p =.09), and IRS Group also impacted OS (p =.043). In Cox proportional hazards model, neither of these variables were significant after adjusting for other factors. Conclusion: Patients with perineal and perianal rhabdomyosarcoma have a poor overall prognosis, probably related to poor patient and disease characteristics at presentation. © 2024 Wiley Periodicals LLC. |
| Keywords: | adolescent; cancer survival; child; event free survival; preschool child; school child; child, preschool; survival rate; retrospective studies; major clinical study; overall survival; clinical feature; mortality; outcome assessment; follow up; follow-up studies; antineoplastic agent; metastasis; radiation; pathology; retrospective study; risk factor; proportional hazards model; infant; soft tissue sarcoma; surgery; local control; rhabdomyosarcoma; anus cancer; univariate analysis; therapy; african american; embryonal rhabdomyosarcoma; perineum; alveolar rhabdomyosarcoma; demographics; anus neoplasms; anus tumor; perineal; treatment protocol; humans; prognosis; human; male; female; article; prognostic assessment; native hawaiian; perianal rhabdomyosarcoma |
| Journal Title: | Pediatric Blood and Cancer |
| Volume: | 71 |
| Issue: | 11 |
| ISSN: | 1545-5009 |
| Publisher: | Wiley Periodicals, Inc |
| Date Published: | 2024-11-01 |
| Start Page: | e31303 |
| Language: | English |
| DOI: | 10.1002/pbc.31303 |
| PUBMED: | 39228061 |
| PROVIDER: | scopus |
| PMCID: | PMC11583849 |
| DOI/URL: | |
| Notes: | Article -- Source: Scopus |
