Rhabdomyosarcoma of nasopharynx

Das, Sudip; Bhowmick, A.; Mukherjee, Subrata; Ghosh, L. M.; Banerjee, Santanu

doi:10.1007/bf02996543

Cited by 3 publications

(1 citation statement)

References 3 publications

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“…Most RMS in the pediatric population (30%-40%) occurs in the head and neck region [ 2 - 3 , 8 - 11 , 16 - 17 , 23 - 24 ]. Between 1975 and 2005, there were significant and unexplained increases in the annual incidences of rhabdomyosarcoma of the head and neck (HNRMS) by 1.16%, and of alveolar RMS, mostly HNRMS, by 4.2% [ 16 ].…”

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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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%

Surgical Interventions for Advanced Parameningeal Rhabdomyosarcoma of Children and Adolescents

Choi¹,

Iwanaga²,

Tubbs³

et al. 2018

Cureus

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Neurofibrosarcoma in the nasal cavity

Goswami

Kundu

Majumdar

et al. 2001

Indian J Otolaryngol Head Neck Surg

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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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Rhabdomyosarcoma of nasopharynx

Cited by 3 publications

References 3 publications

Surgical Interventions for Advanced Parameningeal Rhabdomyosarcoma of Children and Adolescents

Surgical Interventions for Advanced Parameningeal Rhabdomyosarcoma of Children and Adolescents

Neurofibrosarcoma in the nasal cavity

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

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