Multimodal treatment of pediatric patients with Askin’s tumors: our experience

Triarico, Silvia; Attinà, Giorgio; Maurizi, Palma; Nanni, Lorenzo; Briganti, Vincenzo; Meacci, Elisa; Margaritora, Stefano; Boccadoro, Mario; Ruggiero, Antonio

doi:10.1186/s12957-018-1434-2

Cited by 7 publications

(4 citation statements)

References 20 publications

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“…Thus, the question arises, which surgical specialists should build one team and join their comprehensive skills when approaching a child with Ewing sarcoma of the chest wall. In the literature, there are some reports about the team approach ( 6 , 15 , 29 ), but the studies report lower survival rates than those achieved in ours. We orchestrated the team according to the surgical complexity and organ involvement, targeting “en-bloc” resection with a preferably macroscopic safety margin of 1 cm.…”

Section: Discussioncontrasting

confidence: 67%

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Interdisciplinary Radical “En-Bloc” Resection of Ewing Sarcoma of the Chest Wall and Simultaneous Chest Wall Repair Achieves Excellent Long-Term Survival in Children and Adolescents

et al. 2021

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Introduction: Ewing sarcomas of the chest wall, historically known as “Askin tumors” represent highly aggressive pediatric malignancies with a reported 5-year survival ranging only between 40 and 60% in most studies. Multimodal oncological treatment according to specific Ewing sarcoma protocols and radical “en-bloc” resection with simultaneous chest wall repair are key factors for long-term survival. However, the surgical complexity depends on tumor location and volume and potential infiltrations into lung, pericardium, diaphragm, esophagus, spine and major vessels. Thus, the question arises, which surgical specialties should join their comprehensive skills when approaching a child with Ewing sarcoma of the chest wall.Patients and Methods: All pediatric patients with Ewing sarcomas of the chest wall treated between 1990 and 2020 were analyzed focusing on complete resection, chest wall reconstruction, surgical complications according to Clavien-Dindo (CD) and survival. Patients received neo-adjuvant chemotherapy according to the respective Ewing sarcoma protocols. Depending on tumor location and organ infiltration, a multi-disciplinary surgical team was orchestrated to perform radical en-bloc resection and simultaneous chest wall repair.Results: Thirteen consecutive patients (seven boys and six girls) were included. Median age at presentation was 10.9 years (range 2.2–21 years). Neo-adjuvant chemotherapy (n = 13) and irradiation (n = 3) achieved significant reduction of the median tumor volume (305.6 vs. 44 ml, p < 0.05). En-bloc resection and simultaneous chest wall reconstruction was achieved without major complications despite multi-organ involvement. Postoperatively, one patient with infiltration of the costovertebral joint and laminectomy required surgical re-intervention (CD IIIb). 11/13 patients were treated with clear resections margins (R1 resection in one patient with infiltration of the costovertebral joint and marginal resection <1 mm in one child with multiple pulmonary metastases). All patients underwent postoperative chemotherapy; irradiation was performed in four children. Two deaths occurred 18 months and 7.5 years after diagnosis, respectively. Median follow-up for the remaining patients was 8.8 years (range: 0.9–30.7 years). The 5-year survival rate was 89% and the overall survival 85%.Conclusion: EWING specific oncological treatment and multi-disciplinary surgery performing radical en-bloc resections and simultaneous chest wall repair contribute to an improved survival of children with Ewing sarcoma of the chest wall.

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Section: Discussioncontrasting

confidence: 67%

“…Ewing sarcoma of the chest wall usually have a poor prognosis. Triarico et al have reported a series of 9 patients with “Askin tumors,” and their 5-year survival was 60% at a median follow-up of 53.1 months ( 15 ). Five-year survival of patients with localized extra-skeletal Ewing sarcomas has been described with 69.7% ( 16 ).…”

Section: Discussionmentioning

confidence: 99%

Interdisciplinary Radical “En-Bloc” Resection of Ewing Sarcoma of the Chest Wall and Simultaneous Chest Wall Repair Achieves Excellent Long-Term Survival in Children and Adolescents

et al. 2021

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“…Neoadjuvant chemotherapy typically consists of a combination regimen (eg, vincristine, ifosfamide, doxorubicin, and actinomycin), and its use enhances the ability to obtain complete tumor resection with negative microscopic margins. It may allow for the avoidance of external beam radiation for patients with ES/PNET 29,30 . Unsurprisingly, among patients with PNET, a diagnosis of cPNET is independently associated with an increased likelihood of receiving radiation therapy compared to pPNET.…”

Section: Discussionmentioning

confidence: 99%

“…It may allow for the avoidance of external beam radiation for patients with ES/PNET. 29,30 included. Next, there were missing data points that limited our capability to understand and report on the surgical approach taken by surgeons caring for these patients.…”

mentioning

confidence: 99%

Primitive neuroectodermal tumor incidence, treatment patterns, and outcome: An analysis of the National Cancer Database

Woeste

Bhutiani

Hong

et al. 2020

Journal of Surgical Oncology

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Background: Primitive neuroectodermal tumors (PNETs) comprise less than 1% of all sarcomas. The rarity of this disease has resulted in a paucity of information about disease process and management. This study sought to evaluate the incidence, treatment patterns, and outcomes among patients with PNET. Methods: The National Cancer Database was queried for diagnoses of PNET between 2004 and 2014. Patients were dichotomized based on tumor type (central [cPNET] vs peripheral [pPNET]). Demographic, tumor, treatment, and outcome variables were analyzed for the entire patient cohort and by type of PNET. Results: White (86.4%) males (56.6%) represented the majority of patients. The incidence of PNET remained stable over the study period (r 2 = 0.0821). A total of 70.7% underwent surgical resection of the primary site, 50.3% received radiation, and 74.7% received systemic chemotherapy. Compared to those with pPNET, patients with cPNET more often received radiation treatment (P < .001), primary tumor resection (P < .001), and experienced increased 90-day mortality (P < .014). Conclusion: cPNET and pPNET are rare and aggressive malignancies that tend to arise in White males. Multimodal treatment including surgery, chemotherapy, and radiation is conventional. Patients with cPNET more often receive radiation and primary tumor resection with increased 90-day mortality.

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Evaluating the Role of Surgical Resection and Reconstruction in the Management of Ewing sarcoma of the Chest Wall in the Paediatric Population: A Systematic Review

Rice,

Barrett,

Lavan

et al. 2024

Journal of Surgical Oncology

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Background and ObjectivesEwing sarcoma is the most common malignant chest wall tumour in the paediatric population. Following neoadjuvant chemotherapy regimens, the role and extent of surgical resection and reconstruction of the chest wall remain unclear.MethodsA systematic review was conducted in accordance with PRISMA guidelines across four major literature databases. Data regarding overall survival, rate of recurrence, role of surgery and adjuvant therapy role was extracted.ResultsThree thousand ninety‐one studies were initially identified via the search, with 25 single‐centre observational studies (458 patients) and 10 retrospective reviews of multicentre trials (961 patients) included in the final analysis. Five‐year overall survival ranged from 35% to 90%.DiscussionAlong with chemotherapy, local control with surgical resection provides the cornerstone of the management of Ewing sarcoma of the chest wall in children. The literature favours delaying surgery until after neoadjuvant chemotherapy, and there appear to be limited benefits to extensive resection according to pre‐chemotherapy margins, advocated for in current guidelines. Radiotherapy is no longer advocated for all patients, but rather should be guided by surgical margins and the histological response to chemotherapy in the resected specimens. Surgical reconstructive techniques need to be tailored for the growth potential of these paediatric patients to avoid long‐term sequalae, including scoliosis and pulmonary restrictive disease.

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Multimodal treatment of pediatric patients with Askin’s tumors: our experience

Cited by 7 publications

References 20 publications

Interdisciplinary Radical “En-Bloc” Resection of Ewing Sarcoma of the Chest Wall and Simultaneous Chest Wall Repair Achieves Excellent Long-Term Survival in Children and Adolescents

Interdisciplinary Radical “En-Bloc” Resection of Ewing Sarcoma of the Chest Wall and Simultaneous Chest Wall Repair Achieves Excellent Long-Term Survival in Children and Adolescents

Primitive neuroectodermal tumor incidence, treatment patterns, and outcome: An analysis of the National Cancer Database

Evaluating the Role of Surgical Resection and Reconstruction in the Management of Ewing sarcoma of the Chest Wall in the Paediatric Population: A Systematic Review

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