Multimodality Treatment in Ewing’s Sarcoma Family Tumors of the Maxilla and Maxillary Sinus: Review of the Literature

Thorn, David L.; Mamot, Christoph; Krasniqi, Fatime; Metternich, F. U.; Prestin, Sven

doi:10.1155/2016/3872768

Cited by 13 publications

(8 citation statements)

References 107 publications

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“…To lower the current 55% of combined surgery‐radiotherapy used, we need to obtain more R0 margins accurately classified, to achieve better local control and avoid radiotherapy, thus minimizing long‐term sequelae without jeopardizing the use of systemic chemotherapy. This requires expert surgical teams to ensure quality resections and adequate reconstruction procedures and may require patient referral to competent surgeons in expert centers, with a wider expertise in HNES management. When R1 resections are unavoidable, PORT is required and pediatric radiotherapy expertise is necessary to minimize the risks of sequelae in these children/adolescents whose growth potential might be altered and with the added risk of developing second cancers throughout their lives …”

Section: Discussionmentioning

confidence: 99%

Ewing’s Sarcoma of the Head and Neck: Margins are not just for surgeons

et al. 2018

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Background, MethodsTo describe the characteristics, treatments (systemic/local), and outcome (oncological/functional) of French patients with head and neck Ewing's sarcomas (HNES) registered in the Euro‐Ewing 99 (EE99) database. Specific patient‐level data were reviewed retrospective.ResultsForty‐seven HNES patients in the EE99 database had a median age of 11 years, 89% had bone tumors (skull 55%, mandible 21%, maxilla 11%), 89% had small tumors (<200 mL), and they were rarely metastatic (9%). Local treatment was surgery radiotherapy (55%), exclusively surgery (28%), or radiotherapy (17%). Metastatic relapses occurred in five patients with high relapse risk factors (metastasis at diagnosis, poor histological response, large tumors). Local progression/relapses (LR) after exclusive radiotherapy occurred in three patients with persistent extra‐osseous residue and in four patients considered R0 margins (postchemotherapy surgery, without postoperative radiotherapy [PORT]), reclassified by pathological review as R1a. Pathological review reclassified 72% of R0 margins: 11/18 to R1a and 2/18 to R2. Five patients had confirmed R0 margins after postchemotherapy surgery without PORT and had no LR Eight patients had R2 margins (initial surgery without previous chemotherapy, with PORT) and had no LR With a median follow‐up of 9.3 years, the 3‐year LR rate, EFS, and OS were 84.8%, 78.6%, and 89.3%, respectively. Among the 5‐year survivors, 88% had long‐term sequelae.ConclusionTo optimize HNES management, patients should be treated from diagnosis in expert centers with multidisciplinary committees to discuss treatment strategy (type of surgery, need for PORT) and validate surgical margins.

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Section: Discussionmentioning

confidence: 99%

Ewing’s Sarcoma of the Head and Neck: Margins are not just for surgeons

et al. 2018

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“…Common chemotherapy drugs include cyclophosphamide, ifosfamide, vincristine, actinomycin-D, and doxorubicin. There is no standard chemotherapy regimen for PNET, and some recent case reports have shown longer disease-free intervals after treatment with platinum and etoposide ( Thorn et al, 2016 ). Radiotherapy is usually used for patients with inoperable tumors and/or positive surgical margins, as well as for those with poor histological results ( Wang et al, 2017 ).…”

Section: Discussionmentioning

confidence: 99%

Primary primitive neuroectodermal tumor of the cervix confirmed with molecular analysis in a pregnant woman: A case report and literature review

Ding

Zhao

et al. 2022

Front. Genet.

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Primary primitive neuroectodermal tumor (PNET) in the female tract is rare. Recently, a case of cervical PNET was diagnosed in our hospital. A 29-year-old pregnant woman presented with a cystic-solid cervical mass at the 7th week of gestation. The mass grew rapidly during follow-up and ruptured at the 22nd week. A biopsy was performed on the mass. Pathological examination revealed a malignant neoplasm composed of small cells which exhibited positive immunohistochemical (IHC) staining for CD99, SYN, and FLI1. Fluorescence in situ hybridization (FISH) displayed the presence of EWS-FLI1 fusion gene resulting from the chromosomal translocation t (11;22, q24;q12), which confirmed the diagnosis of cervical PNET. The reverse transcription-polymerase chain reaction (RT-PCR) results showed type 2 EWS-FLI1 fusion occurred in this tumor, suggesting a poor prognosis. The patient underwent surgical resection and was given adjuvant chemotherapy followed by pelvic radiotherapy. PNET arising from the genital tract, especially in the uterine cervix, is very rare and presents a diagnostic challenge. FISH and RT-PCR analysis are helpful for the diagnosis of such a tumor at an unusual site, as in the present case.

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“…Based on retrospective analysis, age, clinical stage, tumor site, and the response to systemic chemotherapy are important prognostic factors for disease-free survival and overall survival in patients with Ewing’s sarcoma of the head and neck ( 3 , 6 ). Like the treatment for skeletal Ewing’s sarcoma, induction chemotherapy and local control (surgical resection ± radiotherapy) are the frequently adopted treatment approaches for Ewing’s sarcoma in the head and neck, followed by adjuvant chemotherapy ( 9 , 10 ).…”

Section: Discussionmentioning

confidence: 99%

Primary Ewing’s sarcoma of sphenoid sinus: A case report and literature review

Zhu

et al. 2022

Front. Oncol.

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BackgroundPrimary Ewing’s sarcoma of sphenoid sinus, observed in children and adolescents, is an extremely rare malignancy. Such rarity makes the imaging features and treatment strategies for Ewing’s sarcoma of sphenoid sinus unclear. This study aimed to offer guidance for treating this very disease by describing a patient with a rare primary Ewing’s sarcoma of sphenoid sinus and reviewing the available data in the literature.Case descriptionA case of Ewing’s sarcoma in sphenoid sinus treated with multidisciplinary treatment approaches, including tumor resection, radiotherapy, chemotherapy, and antiangiogenic therapy, was presented in this study. Moreover, literature for Ewing’s sarcoma in the head was systematically searched, and two cases in the sphenoid sinus and five cases in the sphenoid bone were identified. Furthermore, the clinical features, imaging findings, pathological characteristics, treatment, and prognosis were summarized.ConclusionTumor resection combined with radiotherapy and chemotherapy may provide favorable results for patients with Ewing’s sarcoma of sphenoid sinus and bone. However, more reports are still necessary to further clarify optimal management.

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Multimodality Treatment in Ewing’s Sarcoma Family Tumors of the Maxilla and Maxillary Sinus: Review of the Literature

Cited by 13 publications

References 107 publications

Ewing’s Sarcoma of the Head and Neck: Margins are not just for surgeons

Ewing’s Sarcoma of the Head and Neck: Margins are not just for surgeons

Primary primitive neuroectodermal tumor of the cervix confirmed with molecular analysis in a pregnant woman: A case report and literature review

Primary Ewing’s sarcoma of sphenoid sinus: A case report and literature review

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