Rhabdomyosarcoma in Adults: New Perspectives on Therapy

Abstract

Rhabdomyosarcoma (RMS) is well known as a pediatric disease. Most of the knowledge, like biology, genetics, and treatments of this disease, comes from studies done in that age group. The two subtypes of RMS, embryonic RMS and alveolar RMS, that affect mainly the pediatric population are well described in the literature and that has had an impact on the improvement in overall survival during the past 20 years. RMS in the adult population has a low incidence, therefor the study of RMS in this group is challenging. Pleomorphic RMS is the subtype that mainly affects adults and its biology and genetics are not yet completely understood and described. The risk factors for this tumor and the differences among adults and children is also poorly understood. The treatments for adults that have RMS are not standardized having an impact on the overall survival. Pleomorphic RMS has, compared to other adult sarcomas, poor overall survival. Adult patients with RMS have poor prognosis. The standardization of treatments for the adult population is necessary as maybe new treatments for this specific group. There are new treatment options that are being studied mostly in pediatrics and young adults. Immunotherapy is currently proposed as an important treatment possibility including different techniques like vaccination, antigen-mediated therapy, and immune checkpoints. Even if we have a better understanding of RMS, there are still unanswered questions. The improvements seen in the pediatric population are encouraging, but there is still the need to enhance better therapies for adults with RMS.

Rabdomiosarcoma embrionario parameningeo en adulto

“…Se estima 350 casos nuevos por año de RMS en los Estados Unidos; la mitad de estos son diagnosticados en pacientes menores de 10 años. El RMS puede surgir en una variedad de sitios anatómicos, incluyendo la región de cabeza y cuello (35-40%), la genitourinaria (25%), tronco y extremidades (20%) (2) . La tasa de supervivencia a 5 años con tratamiento en adultos (27%) continúa muy por debajo respecto a los niños (70%) (1) .…”

Rhabdomyosarcoma of the Paranasal Sinuses Initially Diagnosed as Acute Sinusitis

“…The rhabdomyosarcoma (RMS) subtype is even more rare in adults, representing less than 1% of all malignant solid tumors in adults. When it does occur, one of the most common RMS subsites is the head and neck region [6].…”

Vision loss secondary to orbital extension of rhabdomyosarcoma in an adult

“…2 It originates from immature mesenchymal cells that normally differentiate into skeletal muscle. Computed tomography angiography of her head and neck showed bilateral ophthalmic artery patency.…”

“…RMS is an aggressive malignant tumour that can occur anywhere in the body. 2 It originates from immature mesenchymal cells that normally differentiate into skeletal muscle. 2 RMS is typically a paediatric tumour and rare in adults, accounting for less than 1% of solid malignancies, 2 making this patient's demographic unusual.…”

“…2 It originates from immature mesenchymal cells that normally differentiate into skeletal muscle. 2 RMS is typically a paediatric tumour and rare in adults, accounting for less than 1% of solid malignancies, 2 making this patient's demographic unusual. Case reports of adults with orbital extension of RMS have been reported to cause vision loss as in this case; however, most of these patients are in young adulthood.…”