Management of pediatric head and neck rhabdomyosarcoma: A case-series of 36 patients

Abstract

Abstract. Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in the pediatric population. In 35% of cases, RMS develops in the head and neck (H&N) region, and only combined therapy is recognized as a curative treatment. However, recent advances in skull base and reconstructive surgery, along with microsurgery and endoscopic surgery, have strengthened the role of surgery as an important part of RMS treatment. In the present study, 36 pediatric RMS cases (24 males and 12 females) were analyzed after surgical treatment. The average age at diagnosis was 7 years. In total, 67% of tumors were localized in the parameningeal region. Alveolar RMS was the most common histopathological type. A total of 16 patients were treated due to disease recurrence or a previous non-radical surgical procedure, while 19 cases had inductive chemotherapy and/or radiotherapy preceding surgical treatment due to locally advanced disease. In 1 case, only diagnostic biopsy was performed. It is recommended that the management of H&N RMS is interdisciplinary from the beginning. Extensive surgical dissection in the H&N region for RMS may result in severe cosmetic defects and functional impairment; thus, these risks should be considered during treatment planning, and the surgical approach should be based on the individual characteristics of each patient.

Congenital Rhabdomyosarcoma Presenting as a Neck Mass at Birth

“…Tissue biopsy is required for definitive diagnosis and histologic classification. Treatment is individualized on a per patient basis and usually includes chemotherapy, surgery, and radiotherapy [6]. …”

Surgical Interventions for Advanced Parameningeal Rhabdomyosarcoma of Children and Adolescents

“…Since the 1970s, using extensive analyses of clinical data, IRSG has developed a multidisciplinary stratification system that categorizes RMS into Group I (a localized disease that is completely resected) to IV (metastatic disease) [ 5 , 8 , 30 , 31 ]. This system has allowed highly individualized multimodal therapeutic protocols, i.e., combinations of surgery, radiotherapy, and chemotherapy [ 5 , 8 , 16 , 20 , 26 ], to be integrated into RMS management and has contributed to the increase in five-year survival rate from 25% in 1970 to 87% [ 2 , 3 , 5 , 8 , 14 , 16 , 17 , 30 ]. However, the 87% relates to a patient group with confined, favorable location [ 9 , 12 ].…”

“…Pediatric HNRMS has an overall survival rate of 28.7% [ 16 ]. 44% percent of all HNRMS occurs in the parameningeal region: paranasal sinuses, nasal cavity, nasopharynx, middle ear, and the skull base [ 8 , 25 - 26 ].…”

“…Symptoms often mimic chronic upper respiratory tract infection (URTI), otitis media (OM), and soft tissue injury (nasal discharge and congestion, otorrhea, and mild swelling) [ 2 ]. Such characteristics of this location increase the risk of misdiagnosis and of diagnosis delayed by up to a month [ 8 - 9 , 18 , 26 - 27 ]. Late diagnosis of HNRMS is directly associated with poor prognosis [ 20 ].…”

“…However, surgical resection is challenging owing to the high likelihood of a disease-positive margin post-resection and risk of injury to the brain parenchyma because of its anatomical proximity and complexity [ 8 , 22 , 25 - 27 ]. Moreover, the role of surgery has been deemed limited in the pediatric population because it is more difficult to achieve suitable surgical access, and functional and cosmetic morbidities often follow [ 25 - 26 ]. However, innovations in craniofacial operation and reconstruction techniques, as an element of the multimodal, multidisciplinary protocol developed by the Intergroup RMS Study Group (IRSG) and Children’s Oncology Group (COG), have yielded satisfactory results in recent years [ 25 ].…”