Pediatric sinonasal rhabdomyosarcoma: A case report

Bostancı, Aslı; Aşık, Mehmet; Turhan, Murat

doi:10.3892/etm.2015.2839

Cited by 7 publications

(24 citation statements)

References 17 publications

(19 reference statements)

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“…However, the 87% relates to a patient group with confined, favorable location [ 9 , 12 ]. It is necessary to explore the efficacy of such protocols in groups with unfavorable factors (five-year survival rate of 31%) i.e., parameningeal location, alveolar histology, >10 years of age, incomplete surgical resection prior to adjuvant systemic treatment, and regional lymph node infiltration [ 8 , 18 , 22 , 30 ]. The IRSG/COG clinical grouping system is depicted in Table 3 .…”

Section: Reviewmentioning

confidence: 99%

“…Thus, the surgeon should aim for a good 0.5 cm margin in the first procedure if possible [ 13 ]. Otherwise, second-look explorations may be necessary, especially if a gross residual mass remains unresected [ 14 , 15 , 18 , 20 , 31 ].…”

Section: Reviewmentioning

confidence: 99%

“…RMS lacks a capsule, a membranous envelope [ 8 , 14 - 15 ]. This allows it to expand rapidly and engulf regional structures, so there are early and considerable local invasions and significant metastatic potential [ 2 - 4 , 7 , 9 , 14 , 16 - 18 ]. RMS also has a local relapse rate of 20%-40%, which contributes to the dismal five-year survival rate of 24%-30% [ 1 , 2 , 8 , 10 , 12 - 16 , 19 ].…”

Section: Introductionmentioning

confidence: 99%

“…Relapse precedes rapid development of a metastatic disease [ 14 ]. Thus, diagnosis of RMS needs to be timely, and a targeted therapeutic protocol should be initiated promptly for an optimal outcome [ 18 , 20 - 21 ]. However, this is seldom achieved; 50% of all RMS cases are high-grade, locally advanced, or even metastatic at the time of diagnosis [ 22 ].…”

Section: Introductionmentioning

confidence: 99%

“…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 ].…”

Section: Introductionmentioning

confidence: 99%

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Surgical Interventions for Advanced Parameningeal Rhabdomyosarcoma of Children and Adolescents

Choi¹,

Iwanaga²,

Tubbs³

et al. 2018

Cureus

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Owing to its rarity, rhabdomyosarcoma of the head and neck (HNRMS) has seldom been discussed in the literature. As most of the data is based only on the retrospective experiences of tertiary healthcare centers, there are difficulties in formulating a standard treatment protocol. Moreover, the disease is poorly understood at its pathological, genetic, and molecular levels. For instance, 20% of all histological assessment is inaccurate; even an experienced pathologist can confuse rhabdomyosarcoma (RMS) with neuroblastoma, Ewing’s sarcoma, and lymphoma. RMS can occur sporadically or in association with genetic syndromes associated with predisposition to other cancers such as Li-Fraumeni syndrome and neurofibromatosis type 1 (von Recklinghausen disease). Such associations have a potential role in future gene therapies but are yet to be fully confirmed. Currently, chemotherapies are ineffective in advanced or metastatic disease and there is lack of targeted chemotherapy or biological therapy against RMS. Also, reported uses of chemotherapy for RMS have not produced reasonable responses in all cases. Despite numerous molecular and biological studies during the past three decades, the chemotherapeutic regimen remains unchanged. This vincristine, actinomycin, cyclophosphamide (VAC) regime, described in Kilman, et al. (1973) and Koop, et al. (1963), has achieved limited success in controlling the progression of RMS. Thus, the pathogenesis of RMS remains poorly understood despite extensive modern trials and more than 30 years of studies exploring the chemotherapeutic options. This suggests a need to explore surgical options for managing the disease. Surgery is the single most critical therapy for pediatric HNRMS. However, very few studies have explored the surgical management of pediatric HNRMS and there is no standard surgical protocol. The aim of this review is to explore and address such issues in the hope of maximizing the number of options available for young patients with HNRMS.

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Section: Reviewmentioning

confidence: 99%

Section: Reviewmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Surgical Interventions for Advanced Parameningeal Rhabdomyosarcoma of Children and Adolescents

Choi¹,

Iwanaga²,

Tubbs³

et al. 2018

Cureus

View full text Add to dashboard Cite

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A Young Man With Proptosis Causing Decreased Visual Acuity

Ghulmiyyah

Khademian

Reilly

2017

JAMA Otolaryngol Head Neck Surg

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Role of Surgery in Rhabdomyosarcoma of the Head and Neck in Children

et al. 2020

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Objectives Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. The goal of this research is to analyze the role of surgery in the management of pediatric parameningeal (PM) and non‐PM head and neck RMS (HNRMS). Study Design Retrospective review. Methods Retrospective chart review of patients <20 years of age treated for HNRMS between 1970 and 2015. Clinical presentation, tumor characteristics, treatment, recurrence, follow‐up, and outcome data were collected. Results Of 97 patients with HNRMS, 56% were male. Overall median (IQR: interquartile range) age at diagnosis was 5.8 (3.3–9.8) years. Sixty‐five patients (67%) had PM tumors. Of 75 patients with histologic subtype identified, 51 (53%) had embryonal and 20 (21%) alveolar RMS. Almost all patients received chemotherapy (99%) and radiotherapy (95%). Forty‐four patients (45%) underwent surgery. Surgery was more likely to be conducted in patients with lesions of a non‐PM site. Median follow‐up time was 3.4 years (IQR: 1.1–10.8). In 5 years of follow‐up, 20% (17 of 85) died and 29% (20 of 70) had recurrence. The estimated 5‐year survival rate was 72% (95% CI, 57.8, 81.5%). Surgery was associated with a reduced risk of mortality after accounting for TNM stage 4 and tumor site (adjusted HR 0.24; 95% CI, 0.07, 0.79; P = .02). The association between surgery and risk of mortality was similar in PM and non‐PM tumors. Conclusion A multimodal protocol for treatment including chemotherapy, surgery, and radiotherapy is the mainstay for management of children with HNRMS. While surgery is more commonly used to treat non‐PM HNRMS, patients who are able to undergo surgery have significantly higher 5‐year survival. Level of Evidence 4 Laryngoscope, 131:E984–E992, 2021

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Pediatric sinonasal rhabdomyosarcoma: A case report

Cited by 7 publications

References 17 publications

Surgical Interventions for Advanced Parameningeal Rhabdomyosarcoma of Children and Adolescents

Surgical Interventions for Advanced Parameningeal Rhabdomyosarcoma of Children and Adolescents

A Young Man With Proptosis Causing Decreased Visual Acuity

Role of Surgery in Rhabdomyosarcoma of the Head and Neck in Children

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