Role of Surgery in Rhabdomyosarcoma of the Head and Neck in Children

Dombrowski, Natasha D.; Wolter, Nikolaus E.; Robson, Caroline D.; Kawai, Kosuke; Irace, Alexandria L.; Vargas, Sara O.; Marcus, Karen J.; Mack, Jennifer W.; Collins, Natalie B.; Rahbar, Reza

doi:10.1002/lary.28785

Cited by 16 publications

(14 citation statements)

References 35 publications

(61 reference statements)

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“…However, the difference did not reach statistical significance, which may be due to the small patient number of our cohort. Our data are supported by various studies which showed that surgery is an important element for gaining remission status leading to prolonged survival 11 12 20 . In addition, recent reports demonstrated that re-irradiation even in unfavorable sites can achieve remission in a significantly higher percentage of patients compared to those without radiotherapy 10 21 22 .…”

Section: Discussionsupporting

confidence: 87%

“…Whereas large clinical trials assessing treatment strategies for de novo disease included several hundred patients [3,4], therapeutic concepts for relapsed patients are not well established. Intensive chemotherapeutic regimens using topotecan, carboplatin, cyclophosphamide and etoposide (TECC) or regimens including anthracyclines (e. g. ACCTIVE) [5,6] or vincristine, irinotecan and temozolomide such as the new European standard protocol for STS relapse [7] demonstrated some efficacy in limited numbers of patients to date, and local tumor control by surgery, radiation [8][9][10] or the combination of both [11,12] seems to play a role in improving overall survival. In addition, according to preliminary data, it was hoped that the use of novel compounds which inhibit specific molecular tumor targets ("targeted therapy"), such as PARP/ MEK/HDAC/TK inhibitors improves outcome [13,14] and relapse studies on individualized treatment approaches based on genetic tumor alterations might play a more important role in future [20].…”

Section: Introductionmentioning

confidence: 99%

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Progression and Relapse of Pediatric Soft Tissue Sarcoma: Individualized Approach of Treatment – Experience from a Major Pediatric Cancer Center in Europe

Barnbrock,

Luckowitsch,

Schmidt

et al. 2023

Klin Padiatr

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BackgroundThe outcome of children with refractory or relapsed soft tissue sarcoma (STS) is extremely poor. Whereas larger clinical trials evaluated specific treatment modalities, real-life data on individual multimodal therapeutic strategies, given alone or in combination, are scarce. Patients and Methods We retrospectively analyzed the clinical course of 18 pediatric patients with progression of or relapsed STS treated between 2008 and 2018 in our institution. Results A total of 18 patients (median age 12.4 years) suffered from progression or relapse of alveolar (n=7), embryonal (n=5), undifferentiated (n=2) rhabdomyosarcoma or desmoplastic small round cell tumor (n=4). 14 patents had an initial stage IV disease. All but one patient died. Median survival was 12.5 months. Shortest survival was seen in patients with systemic progression of the disease, longest in patients with local relapse. Patients with an Oberlin score<2 at the time of relapse had a significant longer time of survival than those with a score≥2. No significant advantage of a specific therapeutic modality was observed. Discussion We critically analyzed the clinical course in the real-life setting, in which various treatment options were applied to an individual patient according to the best of available data. We observed that some patients died within a short period of time despite multiple treatment modalities, which underlines the need for better prognostic parameters. Conclusion In addition to well characterized clinical factors such as local or systemic relapse, the Oberlin score could be helpful in counselling patients and their families for choosing the best strategy of care.

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Section: Discussionsupporting

confidence: 87%

Section: Introductionmentioning

confidence: 99%

Progression and Relapse of Pediatric Soft Tissue Sarcoma: Individualized Approach of Treatment – Experience from a Major Pediatric Cancer Center in Europe

Barnbrock,

Luckowitsch,

Schmidt

et al. 2023

Klin Padiatr

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“…In H&N RMS, parameningeal locations are associated with poor prognosis due to a variety of reasons, including relatively delayed clinical presentation (e.g., orbital locations become symptomatic earlier due to visual problems or proptosis) and the proximity to the skull base, nerves and vessels, that can preclude radical surgical excision [2,14,15]. Different biological behavior of parameningeal RMS has also been proposed [2].…”

Section: Discussionmentioning

confidence: 99%

Magnetic resonance features and cranial nerve involvement in pediatric head and neck rhabdomyosarcomas

et al. 2021

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Purpose Rhabdomyosarcoma (RMS) is a malignant tumor frequent in children. The frequency and characteristics of cranial nerve involvement in pediatric head and neck (H&N) RMS have been scarcely reported. The aim of this study is to review a large cohort of pediatric head and neck RMS with an emphasis on cranial nerve involvement. Methods We retrospectively reviewed H&N RMS cases from 3 tertiary hospitals over a 10-year period. Cranial nerve involvement was defined as radiologically apparent tumor extension along a nerve and/or the presence of secondary signs. Scans were reviewed by two pediatric neuroradiologists, blinded to clinical data. Results A total of 52 patients met the inclusion criteria. Histologically, 39/52 were embryonal RMS, while 13/52 were alveolar RMS. Regional lymph nodes metastases were present in 19.2%. Cranial nerve involvement was present in 36.5%. Nerves were mainly involved as a direct extension of the mass through skull base foramina or after invasion of cavernous sinus, Meckel's cave, orbital apex, or stylomastoid foramen. Conclusion Cranial nerve involvement is frequent in pediatric head and neck RMS and occurs secondary to "geographic" invasion due to direct extension through skull base foramina or cavernous sinus. These tumors never showed distant perineural metastatic disease as is seen in cases of adult head and neck carcinomas. This implies a different biological interaction between the nerves and these tumors in comparison to adult H&N tumors.

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“…Postoperative morbidity has also been reduced-thanks to the development of combined minimally invasive endoscopic and transcranial or transfacial approaches and free-flap reconstructive possibilities, which limit functional and cosmetic sequelae (34)(35)(36). In the retrospective study of 92 HN-RMS (both ERMS and ARMS) by Dombrowski et al (16), surgery was associated with a reduced risk of mortality after adjusting for TNM staging and location of the tumor (p = 0.05). Furthermore, in our series, the quality of the resection assessed by the resection margins (R0, R1, or R2) was not correlated with OS and EFS.…”

Section: Survival and Prognostic Factorsmentioning

confidence: 99%

“…About half of the patients treated for localized ARMS undergo a relapse (14). The 5-year overall survival (OS) of patients with head and neck ARMS (HN-ARMS) ranges from 35% (15) to 80% (16). To evaluate the prognosis value of clinical and pathological features and the impact on outcome of LN staging and locoregional therapies, we reviewed all patients <21 years with localized ARMS treated in France in the prospective EpSSG RMS 2005 study.…”

Section: Introductionmentioning

confidence: 99%

Locoregional Control and Survival in Children, Adolescents, and Young Adults With Localized Head and Neck Alveolar Rhabdomyosarcoma—The French Experience

et al. 2022

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IntroductionThe head and neck (HN) are the most frequent sites of pediatric rhabdomyosarcoma (RMS). Alveolar RMS (ARMS) represents ~20% of all RMS cases and frequently spread to lymph nodes (LNs). The aim was to report locoregional control, event-free survival (EFS), and overall survival (OS), according to clinical and pathological features, LN staging, and treatment modalities.MethodsThe study included all patients prospectively enrolled in EpSSG RMS 2005 study under 21 years of age with localized HN ARMS and diagnosed between 2005 and 2016 in France. Medical data including imaging, surgical report, and radiation therapy planes were analyzed.ResultsForty-eight patients (median age 6 years; range 4 months−21 years), corresponding to 30 parameningeal and 18 non-parameningeal ARMS, were included. There were 33 boys (69%). Tumor locations included the following: orbit (n = 7) among which four cases had bone erosion, paranasal sinuses and nasal cavity (n = 16), deep facial spaces (n = 10), nasolabial fold (n = 8), and other non-parameningeal HN sites (n = 7). A fusion transcript of PAX3-FOXO1 or PAX7-FOXO1 was expressed in 33 of the 45 cases (73%) with molecular analysis. At diagnosis, 10 patients had primary resection of the primary tumor (PRPT) (none with microscopic complete resection) and 9 had LN staging. After induction chemotherapy, 26 patients (54%) had secondary resection of the primary tumor (SRPT) and 13 patients (27%) had cervical LN dissection. A total of 43 patients (90%) were treated with radiation therapy.With a median follow-up of 7 years (range 2–13 years), 5-year OS and EFS were 78% (95% CI, 63–88%) and 66% (95% CI, 51–78%), respectively. We observed 16 events (10 deaths): 4 local, 4 regional, 1 local and regional, and 7 metastatic. In univariate analysis, OS was only superior for patients under 10 years of age (p = 0.002), while FOXO1-negative ARMS, SRPT for parameningeal ARMS, and LN surgery were associated with significantly better EFS.ConclusionOur study confirms a better outcome for fusion-negative ARMS and ARMS in children under 10 years. Moreover, LN surgery and SRPT of parameningeal tumor may improve EFS of ARMS. Larger studies are needed to confirm our findings.

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Role of Surgery in Rhabdomyosarcoma of the Head and Neck in Children

Cited by 16 publications

References 35 publications

Progression and Relapse of Pediatric Soft Tissue Sarcoma: Individualized Approach of Treatment – Experience from a Major Pediatric Cancer Center in Europe

Progression and Relapse of Pediatric Soft Tissue Sarcoma: Individualized Approach of Treatment – Experience from a Major Pediatric Cancer Center in Europe

Magnetic resonance features and cranial nerve involvement in pediatric head and neck rhabdomyosarcomas

Locoregional Control and Survival in Children, Adolescents, and Young Adults With Localized Head and Neck Alveolar Rhabdomyosarcoma—The French Experience

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