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Survival outcomes including salvage therapy of adult head and neck para-meningeal rhabdomyosarcoma: a multicenter retrospective study from Japan.
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- Author(s): Tsuchihashi K;Tsuchihashi K; Ito M; Ito M; Arita S; Arita S; Kusaba H; Kusaba H; Kusaba H; Kusano W; Kusano W; Matsumura T; Matsumura T; Kitazono T; Kitazono T; Ueno S; Ueno S; Taguchi R; Taguchi R; Yoshihiro T; Yoshihiro T; Doi Y; Doi Y; Arimizu K; Arimizu K; Ohmura H; Ohmura H; Kajitani T; Kajitani T; Nio K; Nio K; Nio K; Nakano M; Nakano M; Oshima K; Oshima K; Tamura S; Tamura S; Tamura S; Shirakawa T; Shirakawa T; Shimokawa H; Shimokawa H; Shimokawa H; Uchino K; Uchino K; Hanamura F; Hanamura F; Hanamura F; Okumura Y; Okumura Y; Komoda M; Komoda M; Isobe T; Isobe T; Ariyama H; Ariyama H; Esaki T; Esaki T; Hashimoto K; Hashimoto K; Komune N; Komune N; Matsuo M; Matsuo M; Matsumoto K; Matsumoto K; Asai K; Asai K; Asai K; Yoshitake T; Yoshitake T; Yamamoto H; Yamamoto H; Oda Y; Oda Y; Akashi K; Akashi K; Baba E; Baba E
- Source:
BMC cancer [BMC Cancer] 2023 Oct 31; Vol. 23 (1), pp. 1046. Date of Electronic Publication: 2023 Oct 31.- Publication Type:
Multicenter Study; Journal Article- Language:
English - Source:
- Additional Information
- Source: Publisher: BioMed Central Country of Publication: England NLM ID: 100967800 Publication Model: Electronic Cited Medium: Internet ISSN: 1471-2407 (Electronic) Linking ISSN: 14712407 NLM ISO Abbreviation: BMC Cancer Subsets: MEDLINE
- Publication Information: Original Publication: London : BioMed Central, [2001-
- Subject Terms:
- 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.
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Cancer Med. 2018 Aug;7(8):4023-4035. (PMID: 29956493) - Contributed Indexing: Keywords: Adult; Head and neck; Para-meningeal; Rhabdomyosarcoma; Salvage
- Publication Date: Date Created: 20231031 Date Completed: 20240122 Latest Revision: 20240122
- Publication Date: 20240123
- Accession Number: PMC10617040
- Accession Number: 10.1186/s12885-023-11528-4
- Accession Number: 37904096
- Source:
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