Survival outcomes including salvage therapy of adult head and neck para-meningeal rhabdomyosarcoma: a multicenter retrospective study from Japan.
BMC Cancer
; 23(1): 1046, 2023 Oct 31.
Article
in En
| MEDLINE
| ID: mdl-37904096
ABSTRACT
BACKGROUND:
Rhabdomyosarcoma is the most common soft tissue sarcoma in children, but rare in adults. Para-meningeal rhabdomyosarcoma in head and neck (PM-HNRMS) is less applicable for surgery due to the anatomic reason. PM-HNRMS has a poor prognosis in children. However, its clinical outcomes remain unclear in adults due to the rarity. Further, there is almost no detailed data about salvage therapy.METHODS:
We retrospectively examined the adult patients with PM-HNRMS treated at institutions belonging to the Kyushu Medical Oncology Group from 2009 to 2022. We evaluated the overall survival (OS) and progression-free survival (PFS) of the patients who received a first-line therapy. We also reviewed the clinical outcomes of patients who progressed against a first-line therapy and received salvage therapy.RESULTS:
Total 11 patients of PM-HNRMS received a first-line therapy. The characteristics were as follows median age 38 years (range 25 - 63 years), histology (alveolar/spindle) 10/1, and risk group (intermediate/high) 7/4. As a first-line therapy, VAC and ARST0431-based regimen was performed in 10 and 1 patients, respectively. During a first-line therapy, definitive radiation for all lesions were performed in seven patients. The median PFS was 14.2 months (95%CI 6.0 - 25.8 months) 17.1 months (95%CI 6.0 - not reached (NR)) for patients with stage I-III and 8.5 months (95%CI 5.2 - 25.8 months) for patients with stage IV. The 1-year and 3-year PFS rates were 54.5% and 11.3% for all patients. Median OS in all patients was 40.8 months (95%CI 12.1 months-NR) 40.8 months (95%CI 12.1 - NR) for patients with stage I-III and NR for patients with stage IV. The 5-year OS rate was 48.5% for all patients. Among seven patients who received salvage therapy, three are still alive, two of whom remain disease-free for over 4 years after completion of the last therapy. Those two patients received multi-modal therapy including local therapy for all detected lesions.CONCLUSION:
The cure rate of adult PM-HNRMS is low in spite of a first-line therapy in this study. Salvage therapy might prolong the survival in patients who received the multi-modal therapy including local therapy for all detected lesions.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Rhabdomyosarcoma
/
Head and Neck Neoplasms
Limits:
Adult
/
Humans
/
Middle aged
Country/Region as subject:
Asia
Language:
En
Journal:
BMC Cancer
Journal subject:
NEOPLASIAS
Year:
2023
Document type:
Article
Affiliation country:
Japan