Embryonal rhabdomyosarcoma of nasopharynx

Tuli, B. S.; Parmar, T. L.

doi:10.1007/bf02996847

Cited by 2 publications

(2 citation statements)

References 7 publications

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“…Rhabdomyosarcoma (RMS) is a rare high-grade soft tissue sarcoma of mesenchymal origin, with evidence of striated muscle cell differentiation [ 1 - 7 ]. It represents 5%-7% of all pediatric malignancies and is the most prevalent sarcoma of childhood and adolescence, i.e., 50% of all reported sarcoma cases [ 1 , 2 , 4 , 8 - 11 ].…”

Section: Introductionmentioning

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%

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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: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Surgical Interventions for Advanced Parameningeal Rhabdomyosarcoma of Children and Adolescents

Choi¹,

Iwanaga²,

Tubbs³

et al. 2018

Cureus

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Nasal Embryonal Rhabdomyosarcoma in the Pediatric Population: Literature Review and Report of Midline Presentation

Larson

Rutledge

Hunnell

et al. 2021

Plastic and Reconstructive Surgery - Global Open

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Background: Congenital midline nasal masses are rare anomalies and are typically benign nasal dermoid sinus cysts (NDSCs). Rhabdomyosarcomas (RMSs) are even less common, and only a fraction affect sites like the external nose, nasal cavity, nasopharynx, and paranasal sinuses. We review the clinical presentation and treatment of nasal, nasopharyngeal, and paranasal RMSs and report the first documented midline presentation. Methods: We queried PubMed for articles with titles containing the terms rhabdomyosarcoma or sarcoma botryoides and nose, nasal, paranasal, sinonasal, nasopharynx, or nasopharyngeal. We then searched the references of each included article using the same parameters and continued this process iteratively until no new articles were found. Results: The paranasal sinuses were the most commonly affected site, followed by the nasopharynx, nasal cavity, and external nose. Two patients presented with involvement of the external nose, but each presented with involvement of the right ala rather than a midline mass. The rates of intracranial extension and/or skull base involvement were comparable to those of NDSCs. The alveolar subtype was most common, followed by the embryonal subtype. Conclusions: Most midline nasal masses are benign; however, we report the first documented presentation of an RMS as a midline nasal mass. Accordingly, RMS should be included in the differential diagnosis of midline nasal masses in the pediatric population. Surgery for midline nasal masses is sometimes delayed due to the risks of interfering with developing structures and early anesthesia. However, early surgical treatment should be considered given this new differential and its predilection for early metastasis.

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Embryonal rhabdomyosarcoma of nasopharynx

Abstract: Abstract.,eA,,rare~zase of E~bryonal Rhabdomyosarcolna m a 5 year,male child ~nv0(yttag? N/~loptlary~X,w~th ~ntracranml secondaries ~s reported. The chdd presented w~th dlffi6Ult3' m~bregthm~ and~gwallown~g w~th ,

Cited by 2 publications

References 7 publications

Surgical Interventions for Advanced Parameningeal Rhabdomyosarcoma of Children and Adolescents

Surgical Interventions for Advanced Parameningeal Rhabdomyosarcoma of Children and Adolescents

Nasal Embryonal Rhabdomyosarcoma in the Pediatric Population: Literature Review and Report of Midline Presentation

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