Embryonal and Alveolar Rhabdomyosarcoma in Adults: Real-Life Data From a Tertiary Sarcoma Centre

Abstract

Aims: Embryonal and alveolar rhabdomyosarcoma (ERMS, ARMS) are subtypes of RMS that mainly occur in children, with relatively good outcomes. The incidence in adults is extremely low and survival is significantly worse compared with children. Data are scarce and literature generally combines all RMS subtypes, including pleomorphic RMS, which primarily occurs in adults and behaves more like undifferentiated pleomorphic sarcoma. The aim of this study was to evaluate patient and tumour characteristics, outcome and prognostic factors in adult patients with ERMS and ARMS. Materials and methods: All adult (18 years or older) ERMS and ARMS patients (presenting 1990e2016) were identified from a prospectively maintained database and were included in this analysis. Results: Overall, 66 patients were included (42 men, 24 women). The median age at presentation was 28 years (range 18e71). The median overall survival for all ARMS (n ¼ 42) and ERMS (n ¼ 24) patients was 18 months, with a 5-year overall survival rate of 27%. Patients presenting with localised disease (n ¼ 38, 58%) and metastatic disease (n ¼ 25, 42%), had a 5-year overall survival rate of 36% and 11%, respectively. In univariate analysis we found alveolar subtype, fusion gene positivity, infiltrative tumour and metastatic presentation to be negative prognostic factors. Conclusion: Survival in adult ERMS and ARMS patients is poor and the current data may be useful in the design of trials with novel agents. Ideally, paediatric and adult oncologists should set up trials together to get a better understanding of biological, genetic and clinically relevant factors in this disease.

Rare Adult Subtype of Rhabdomyosarcoma, a Common Childhood Soft Tissue Carcinoma

“…The 5-year survival overall is 27%, for localized disease is 36%, and for metastatic disease is 11%. 5 RMS in an adult has a worse prognosis than in a child, with an overall survival rate of 20% to 40%. 3 Increasing age above 35 years old is associated with decreased survival from all subtypes.…”

“… 1 In addition to older age, poor prognostic factors include size >5 cm, pleomorphic and alveolar subtypes, extremity location fusion gene positivity, infiltrative tumor, and metastatic presentation. 5 , 12 Particularly in the alveolar subtype, the expression of AP2i and P-cadherin indicate a poor prognosis. In the embryonal subtype, hyperdiploid tumors and those that express Epidermal growth factor receptors (EGFR) and fibrillin-2 have a favorable prognosis.…”

“… 4 The 5-year survival overall is 27%. 5 It has been shown that when pediatric guidelines are implemented in adult cases, the prognosis is better but it still does not reach the survival rate that is seen in the pediatric population. This could potentially be due to the increased incidence of poor prognostic indicators in adult cases when compared with pediatric cases or the increased risks or chemotherapy adverse events in the adult population.…”

Cuidados De Enfermagem E Paliativo De Um Jovem Com Rabdomiossarcoma

“…São Paulo (SP), Brasil. 1 https://orcid.org/0000-0003-0613-6569 2 https://orcid.org/0000-0001-8865-8031 3 https://orcid.org/0000-0002-3588-653X 4 https://orcid.org/0000-0003-4022-2612 5 https://orcid.org/0000-0003-3387-513X 6 https://orcid.org/0000-0001-7455-1727…”

“…Apresentou-se, nos resultados, grande diferença em pacientes pediátricos com os mesmos subtipos, cuja sobrevida global em cinco anos relatada foi de 70 a 80%. [3][4] Diagnosticam-se aproximadamente 600 novos casos de RMS por ano nos Estados Unidos, 41% dos casos acometem adultos e 59%, crianças, 5 porém, no Brasil, o Instituto Nacional de Câncer (INCA) não dispõe de dados sobre este tipo de câncer.…”