Primary Ewing’s Sarcoma of the Skull in Children

Carlotti, Carlos Gilberto; Drake, James M.; Hladky, Jean-Pierre; Teshima, Ikuko; Becker, Laurence E.; Rutka, James T.

doi:10.1159/000028881

Cited by 40 publications

(7 citation statements)

References 39 publications

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“…Furthermore, immunohistochemistry and molecular biology were highly indicative for this diagnosis. 19 The prognosis of Ewing's sarcoma has improved significantly. Patients with primary localized Ewing's sarcoma treated at the Mayo Clinic between 1950 and 1968 had only a 21.8% probability of surviving for longer than 5 years, while currently the 5-year survival rate for patients treated with complete surgical excision, chemotherapy, and irradiation may be as high as 74%.…”

Section: Discussionmentioning

confidence: 99%

Long-Term Survival after Primary Ewing's Sarcoma of the Skull with Intracranial Extension

Rainov,

Haritonov,

Heidecke

2023

J Neurol Surg A Cent Eur Neurosurg

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Background and Objective Primary Ewing's sarcoma of the skull is a very rare malignant neoplasm, predominantly occurring in children and adolescents. We describe here the clinical, neuroradiologic, and histopathologic features of a patient with primary Ewing's sarcoma of the skull and discuss the standards of therapy for this type of tumor. Clinical Presentation This 18-year-old male patient presented with a primary Ewing's sarcoma of the skull, involving the dura of the frontal and parietal lobes of the left cerebral hemisphere. He was treated with gross total surgical excision of tumor, skull reconstruction, chemotherapy, and irradiation. Twelve years after the surgery, the patient has no evidence of local recurrence or distant metastases. Radical surgical excision of the primary tumor with safety margins is thought to play a role in the favorable clinical course. Conclusion The presented case is the longest surviving patient after treatment of primary Ewing's sarcoma of the skull bone. This rare type of tumor may allow better survival rates under adequate management than sarcoma elsewhere in the body.

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Section: Discussionmentioning

confidence: 99%

Long-Term Survival after Primary Ewing's Sarcoma of the Skull with Intracranial Extension

Rainov,

Haritonov,

Heidecke

2023

J Neurol Surg A Cent Eur Neurosurg

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“…EWS is known for its rapid extradural spread, though there are isolated reports of intradural involvement. 1 2 5 6 Predominantly, the tumor grows extradural, and a well-defined plane exists to leave intact dura while lifting the tumor. 7 Usually, dura acts as a formidable barrier for the extradural tumor to invade intradural structures, but dural sacrifice and tumor excision are required in such cases.…”

Section: Discussionmentioning

confidence: 99%

Primary Calvarial Ewing Sarcoma: A Case Series

Mohindra

Tripathi

Batish

et al. 2021

J Neurol Surg B Skull Base

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Background Calvarial Ewing tumor is a relatively rare differential among bony neoplasms. We present our experience of managing primary calvarial Ewing sarcoma (EWS), highlighting their clinical and radiological findings. Method In a retrospective analysis, we evaluated our 12-year database for pathologically proven EWS. A literature search was conducted for the comparative presentation and update on the management and outcome. Result From January 2008 to December 2020, we managed eight patients (male:female = 5:3; age range 6 months to 19 years, mean 11.5 years) harboring primary calvarial EWS. All cases underwent wide local excision; two patients required intradural tumor resection, while one required rotation flap for scalp reconstruction. Mean hospital stay was 8 days. All patients received adjuvant chemo- and radiotherapy. Three patients remained asymptomatic at 5 years of follow-up, while two patients died. Conclusion Primary calvarial EWS is a rare entity. It usually affects patients in the first two decades of life. These tumors can be purely intracranial, causing raised intracranial pressure symptoms, which may exhibit rapidly enlarging subgaleal tumors with only cosmetic deformities or symptoms of both. Radical excision followed by adjuvant therapy may offer a favorable long-term outcome.

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“…There is a slightly male predominance with a male-to-female ratio of 1.5: 1 [ 7 , 10 , 11 ]. Primary EWS of the head and neck region is extremely rare and accounts for only 4–9% of all EWSs [ 3 , 4 , 5 , 6 ]. In general, approximately 20–30％ of EWS patients present with metastases at the time of their diagnosis [ 12 , 13 , 14 , 15 , 16 ].…”

Section: Discussionmentioning

confidence: 99%

“…EWS of the bone, which most commonly occurs in children and young adults, accounts for approximately 3% of all pediatric cancers and most cases arise in the long bones or the pelvis [ 2 ]. Primary EWS of the head and neck region is extremely rare, accounting for only 4–9% of all EWSs [ 3 , 4 , 5 , 6 ]. Furthermore, sinonasal lesions are uncommon.…”

Section: Introductionmentioning

confidence: 99%

Primary Ewing’s Sarcoma of the Sinonasal Tract: A Case Report

Suzuki¹,

Yasumatsu²,

Nakashima³

et al. 2017

Case Rep Oncol

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A 23-year-old male presented with a 3-month history of left purulent rhinorrhea, progressive nasal obstruction, and intermittent epistaxis. A fiberoptic examination revealed a large vascular polypoid mass completely filling the left nasal cavity. CT and MRI scans showed a large hypervascular mass involving the left nasal airway, maxillary antrum, and the anterior ethmoid cells. There was no bony erosion or contiguous spread, and the remaining sinuses, orbit, and cranial fossa were uninvolved. The patient underwent complete removal of the mass via an external lateral rhinotomy approach. The soft mass was large and vascular. A microscopic analysis revealed an undifferentiated tumor consisting of a solid sheet of small, round blue cells. Mitotic figures were also present. Immunohistochemically, the tumor cells were strongly positive for CD99. Molecular studies using a PCR confirmed the chromosomal translocation of FLI1 (exon 6). These findings were considered diagnostic for Ewing’s sarcoma. Postoperatively, the patient was treated with combined chemotherapy and radiotherapy. Adjuvant chemotherapy consisting of vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide (total: 7 cycles) was commenced. He also received radiation therapy for local control (total dose: 50.4 Gy). The patient is currently alive without any evidence of recurrence or metastasis.

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Primary Ewing’s Sarcoma of the Skull in Children

Cited by 40 publications

References 39 publications

Long-Term Survival after Primary Ewing's Sarcoma of the Skull with Intracranial Extension

Long-Term Survival after Primary Ewing's Sarcoma of the Skull with Intracranial Extension

Primary Calvarial Ewing Sarcoma: A Case Series

Primary Ewing’s Sarcoma of the Sinonasal Tract: A Case Report

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