The prognosis and effects of local treatment strategies for orbital embryonal rhabdomyosarcoma: a population-based study

Abstract

IntroductionOrbital embryonal rhabdomyosarcoma is a rare childhood malignancy with a good prognosis, but the optimal treatment remains unclear. Using a population-based cancer registry, we assessed the prognoses and survival outcomes of patients with orbital embryonal rhabdomyosarcoma according to the local treatment strategy.Patients and methodsPatients diagnosed with orbital embryonal rhabdomyosarcoma between 1988 and 2012 as part of the Surveillance Epidemiology and End Results program were included. Univariate and multivariate Cox regression analyses were performed to determine the prognostic factors associated with cause-specific survival (CSS) and overall survival (OS).ResultsIn total, 102 patients were included; their median age was 6 years, 78.4% were white, and 56.9% were male. The median tumor size was 30 mm. Of 20 patients with an available histologic grade, the tumors of 90% were poorly differentiated/undifferentiated. Of 92 patients with available surgical and radiotherapy (RT) statuses, 50 (54.3%), 36 (39.1%), and 6 (6.5%) received surgery and RT, primary RT, and primary surgery, respectively. Ninety-five patients (93.1%) received chemotherapy. The 5- and 10-year CSSs of the entire cohort were 94.3% and 92.2%, respectively. The 5- and 10-year OSs were 93.3% and 91.3%, respectively. In 95 patients who were followed up for at least 12 months, there were no significant prognostic factors related to CSS and OS. Furthermore, the local treatment strategy did not significantly affect CSS (P=0.29) or OS (P=0.468).ConclusionThere is no local treatment of choice for orbital embryonal rhabdomyosarcoma in terms of survival. However, RT is a reasonable alternative treatment to surgery.

Epidemiology, Incidence, and Survival of Rhabdomyosarcoma Subtypes: SEER and ICES Database Analysis

“…For example, since 2004 the Collaborative Staging Codes were included, which gives clinical and pathologic data that was not previously available. Surgical details also have varied throughout the time period of this database, with recent years including more specifics of the surgeries performed . The lack of these critical details in the earlier years of the database limits the ability to analyze them when the goal is to have a large cohort.…”

“…Prior studies investigating rhabdomyosarcoma demographics and prognostic factors using databases have largely focused on specific anatomic sites or JOURNAL OF ORTHOPAEDIC RESEARCH ® OCTOBER 2019 subtypes. 3,4,6,[14][15][16][17][18][19][20] In a 2017 study by Nasioudis et al 15 rhadomyosarcoma of the lower female genital tract was shown to be most prevalent in prepubertal girls and adolescents, but carry excellent survival rates. Additionally, rare cases affecting adults resulted in poor outcomes.…”

“…Surgical details also have varied JOURNAL OF ORTHOPAEDIC RESEARCH ® OCTOBER 2019 RHABDOMYOSARCOMA EPIDEMIOLOGY, INCIDENCE, AND SURVIVAL throughout the time period of this database, with recent years including more specifics of the surgeries performed. 16 The lack of these critical details in the earlier years of the database limits the ability to analyze them when the goal is to have a large cohort. Another critical limitation of the SEER database is that it is confined to the United States and the region within the United States of the patient's location is not listed.…”

A rare case in an 11-year old child with bilateral primary orbital rhabdomyosarcoma with rapid onset of bilateral proptosis and temporal lobe abscess secondary to chronic suppurative otitis media

“…1,4,5 RMS most commonly involves the retrobulbar region but can involve other areas, including eyelids, conjunctiva, and rarely, uveal tissue, often manifesting as rapidly progressive proptosis or globe displacement. 6,7 We are reporting this case because: rare bilateral orbital RMS with a very aggressive course in an uncommon age group; bilateral proptosis, and simultaneous confusion secondary to temporal lobe abscess in one diagnosis.…”

Adjuvant therapy for orbital non-rhabdomyosarcoma soft tissue sarcoma: comparison of long-term outcome between radiotherapy and chemotherapy

“…Little studies focus on the treatment for orbital sarcoma, except for pediatric rhabdomyosarcoma, which is the most common pediatric orbital malignant tumor [4] . Previous studies convinced rhabdomyosarcoma including orbital lesions was relatively sensitive to chemotherapy and irradiation [5][6][7] . For most types of NRSTSs, surgery with wide margins remains the cornerstone of curative intent treatment, however, wide margins in the orbit are often difficult to achieve due to the proximity of vital structures or the destruction of appearance.…”