Laryngeal Involvement of Rhabdomyosarcoma in an Adult

Abalı, Hüseyin; Aksoy, Sercan; Sungur, Arzu; Yalçın, Şuayib

doi:10.1186/1477-7819-1-17

Cited by 9 publications

(1 citation statement)

References 8 publications

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“…The traditional surgical intervention for RMS has been extensive, wide local resection with concomitant removal of a 0.5 cm thick envelope of normal tissue to achieve a clear disease-negative margin and to preserve the functions of nearby vital structures [ 10 , 15 , 31 ]. The aim of RMS surgery has been to perform a “curative,” complete resection to achieve the 80% overall survival rate [ 2 , 6 , 10 ]. However, it is often not possible to achieve such a margin in a complex anatomy such as that of the parameningeal region.…”

Section: Reviewmentioning

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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