Elsevier

American Journal of Otolaryngology

Volume 34, Issue 6, November–December 2013, Pages 682-689
American Journal of Otolaryngology

Original contribution
Incidence trends and long-term survival analysis of sinonasal rhabdomyosarcoma,☆☆

https://doi.org/10.1016/j.amjoto.2013.04.012Get rights and content

Abstract

Purpose

Sinonasal rhabdomyosarcoma (SNRMS) is a rare malignancy which often presents with nasal obstruction, rhinorrhea and epistaxis. It is the most common sarcoma in children. In this study, we analyze the incidence and long-term survival for SNRMS using a national population-based database.

Methods

The United States National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) registry was utilized to calculate incidence and survival trends for SNRMS between 1973 and 2009. In addition, data were grouped by age, gender, race, and histopathological subtype.

Results

A total of 181 cases of SNRMS were analyzed for incidence trends, showing a 1.23:1 female to male ratio. While the overall incidence of SNRMS increased by 1.02% annually over the last 20 years, this pattern was not equal amongst gender and racial groups. The incidence in males has increased, while in females incidence has decreased. An increase in incidence was noted in white and “others,” but decreased in blacks. Using a total of 314 cases for survival analysis, we found that the rate in the white population has been consistently highest with a 5-year survival of 49.45%, 10- and 20-year survival of 48.81%. Survival rates in cases of embryonal SNRMS were also consistently higher than in cases of alveolar SNRMS.

Conclusion

Overall incidence of SNRMS is increasing. Histologic subtype and race are important considerations in the long-term prognosis of SNRMS. Future studies will further elucidate gender and race related trends.

Introduction

Rhabdomyosarcoma (RMS) is the most common malignant soft-tissue tumor of the nose and paranasal sinuses in children [1], [2], [3], [4]. Prognosis depends on a combination of patient age, histologic character, clinical stage, and tumor location. RMSs are generally divided by meningeal and parameningeal sites [5]. Parameningeal sites include middle ear, nasal cavity, paranasal sinuses, nasopharynx, and infratemporal fossa [2]. Because of the risk of intracranial extension via the parameningeal locations, sinonasal rhabdomyosarcomas (SNRMSs) often have a poor overall prognosis [4]. Tumors may present as painless, asymptomatic masses, or with symptoms of nasal obstruction, epistaxis, and rhinorrhea.

Several different histological types of rhabdomyosarcoma of the head and neck region have been identified, including embryonal, alveolar, pleomorphic, and mixed [1], [2]. Embryonal and alveolar patterns are more prevalent. Histologically, embryonal RMS consists of spindle and primitive round cells with rhabdomyoblasts and alveolar RMS consists of malignant cells bundled by fibrovascular septa to form alveolar-like spaces [6], [7], [8]. In terms of survival, the alveolar pattern is associated with a poorer prognosis than the embryonal pattern [9]. The embryonal form is often found in the head and neck region [10].

While definitive diagnosis requires a biopsy, the diagnosis of SNRMS may be suggested based on a thorough history, as well as radiographic imaging such as CT and MRI [11]. SNRMS is often treated with a combination of chemotherapy, radiotherapy, and surgical resection [1].

A 2011, SEER analysis of all head and neck RMSs by Turner et al. reported an approximate 5-year survival rate of 62.8% [12]. However, that study focused on all head and neck RMSs and did not go in depth into some of the specific details of SNRMS. This current study expands on Turner’s findings in that it reports additional important information in the SEER database specific to SNRMS not previously discussed in the literature. In this study, we expand the knowledge on these rare tumors by exploring the trends in incidence and uncovering prognostic factors influencing the long-term survival of SNRMS. To our knowledge, this is the first study utilizing the Surveillance, Epidemiology, and End Results (SEER) registry to evaluate the incidence of SNRMS based on gender, race, and histopathology, and its long-term survival trends.

Section snippets

Materials and methods

The SEER registry of 1973 to 2009 was accessed using SEER*Stat 8.0.1 (Surveillance Research Program, National Cancer Institute, 2012) to calculate the incidence and survival for the population used in this study. Institutional Review Board approval was not required for this study, as the SEER database is free of any sensitive patient information or identifiers. The SEER database is comprised of registries from numerous geographical areas of the United States. The SEER 18 registry, which is used

Demographic analysis

Of the 181 patients, 58.0% of cases were younger than the age of 20 years and 37.02% were younger than 10 years of age (Fig. 1). In this analysis, 55.25% of cases were seen in females, while 44.75% of cases were males, with a female to male ratio of 1.23:1 (Table 1). In addition, 76.80% of SNRMSs involved whites, 14.92% were black patients, with the remaining 8.29% of patients identifying themselves as “other”. The primary tumor site was most commonly in the paranasal sinuses, seen in 51.93% of

Discussion

RMS, first described by Weber [7] in 1854, is a rare tumor, most commonly found in the head and neck region [13]. It is the most common soft tissue sarcoma of the childhood population [7], [14], [15]. In this study we found that over the last 30 years, SNRMS has a predilection for the young, pediatric population as the highest rates of these lesions are seen in the 0–9 age group (Fig. 1). Furthermore, interesting differences are noted between the histologic sub-types of SNRMS, with the embryonal

Conclusion

RMS is the most common childhood sarcoma, mostly found in the head and neck region. This study focuses on the overall incidence and long-term survival of SNRMS cases, as well as exploring the distribution by gender, race and histological type. The SEER registry used to demonstrate statistical evidence of incidence and survival is a crucial tool for studying a large cohort of patient information. The overall incidence of SNRMS is increasing and the long-term survival stabilizes after 5 years.

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    Financial Disclosures: None.

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    Conflicts of Interest: None.

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