Laryngeal Synovial Sarcoma: A Systematic Review of the Last 40 Years of Reported Cases

Abstract

Primary laryngeal synovial sarcoma is an extremely rare tumor predominantly affecting young adults. There are currently no well-defined guidelines to direct investigation and management, and treatment is largely based on what is known for synovial sarcoma of the upper and lower limbs. This PROSPERO-registered study aims to review the diagnostic methods, treatment regimens, and survival outcomes for patients with synovial sarcoma of the larynx. A systematic search of databases Medline, Embase, SCOPUS, and Web of Science was undertaken in December 2017. The literature search identified 1031 potentially relevant studies, and after the deletion of duplicates and excluded papers, 98 full-text articles were screened. A total of 39 cases were reviewed from 32 studies in the data extraction. The average age at the time of laryngeal synovial sarcoma diagnosis was 32 years (range, 11-79 years). In all cases (n ¼ 39), patients underwent wide surgical excision, with 20 patients requiring a partial or total laryngectomy. A total of 18 patients received adjuvant and 3 received neoadjuvant radiotherapy. Chemotherapy was used in 10 cases, with ifosfamide the most frequently used agent. There was considerable variability in the order and combinations of the abovementioned treatments. No clinicopathologic factors or treatment regimens were associated with improved overall survival or lower rate of recurrence. There is a paucity of literature and heterogeneity in clinical approaches to this highly aggressive sarcoma. Reporting of cases must be standardized and formal guidelines must be established to guide clinical management.

Sarcoma sinovial da laringe: Relato de caso sob a perspectiva da deglutição, voz e qualidade de vida

“…O trabalho visa reabilitar, habilitar e prevenir disfunções tardias 4 . Em todas as avaliações de qualidade de vida, o paciente, neste estudo, apresentou escores dentro dos valores padrão, o que provavelmente se deve ao constante cuidado multiprofissional e familiar 1,7,8 .…”

“…O sarcoma sinovial é um tumor raro e agressivo dos tecidos moles, com origem habitual no tecido mesenquimal. É responsável por 5% a 10% de todos os sarcomas de tecidos moles e afeta mais comumente os membros superiores e inferiores [1][2][3] . O sarcoma sinovial primário da cabeça e pescoço é raro, representando menos de 5% de todos os sarcomas sinoviais.…”

“…O sarcoma sinovial primário da cabeça e pescoço é raro, representando menos de 5% de todos os sarcomas sinoviais. O local mais comum do sarcoma sinovial de cabeça e pescoço é a hipofaringe, raramente descrito na laringe 1,2 .…”

“…Em uma revisão sistemática de 2019 foram encontrados 28 relatos de casos independentes e 4 séries de casos contendo 11 casos de sarcoma sinovial de laringe no mundo 1 . A literatura recomenda tratamento multimodal com radioquimioterapia e laringectomia total com sobrevida média de dois anos 2 .…”

“…A literatura recomenda tratamento multimodal com radioquimioterapia e laringectomia total com sobrevida média de dois anos 2 . Diversos aspectos do sarcoma sinovial são pouco compreendidos, como a real implicação do tamanho do tumor, o subtipo histológico e o subtipo do gene de fusão SYT-SSX no prognóstico e funcionalidade [1][2][3][4] .…”