Ewing Sarcoma of the Posterior Fossa in an Adolescent Girl

Stark, Andreas M.; Leuschner, Ivo; Mehdorn, H. Maximilian; Claviez, Alexander

doi:10.1155/2014/439830

Cited by 6 publications

(3 citation statements)

References 16 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…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. 8 Clinically, the patient presents with localized swelling, pain, generalized malaise, and fever. 1,2,9 The examination may reveal papilledema, local tenderness, nasal obstruction in skull base lesions, and/or cerebellar signs.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Primary Calvarial Ewing Sarcoma: A Case Series

Mohindra

Tripathi

Batish

et al. 2021

J Neurol Surg B Skull Base

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionmentioning

confidence: 99%

“…All patients had features of bony erosion on the presented computed tomography (CT) scans (►Figs. [1][2][3][4][5][6][7][8].…”

Section: Methodsmentioning

confidence: 99%

Primary Calvarial Ewing Sarcoma: A Case Series

Mohindra

Tripathi

Batish

et al. 2021

J Neurol Surg B Skull Base

View full text Add to dashboard Cite

show abstract

“…The most common symptom at presentation was headache and emesis (10/12, 83.3%). 2,3,6,7,[9][10][11][12][13][14][15] Extracranial spread was uncommon, occurring in only two patients. Management typically requires a multimodal approach to include resection, radiotherapy, and intensive multiagent chemotherapy.…”

Section: Ewing Sarcomamentioning

confidence: 97%

Malignant spindle cell tumors of the posterior fossa in children: case series and review of management

Gigliotti

Mau

Specht

et al. 2021

Journal of Neurosurgery: Pediatrics

View full text Add to dashboard Cite

OBJECTIVE The WHO Classification of Tumours of the Central Nervous System (2016) classifies nonmeningothelial malignant spindle cell tumors involving the extraaxial tissues of the posterior fossa as melanocytic tumors and malignant mesenchymal tumors (sarcomas). The objective of this study was to conduct a review of the literature pertaining to the management strategies of posterior fossa malignant spindle cell tumors in the pediatric population. METHODS The authors performed an institutional search of their pathology database for patients younger than 18 years of age who presented with posterior fossa malignant spindle cell tumors. A literature review was also performed using the PubMed database, with “posterior fossa” or “spindle cell tumors” or “Ewing sarcoma” or “high-grade” or “spindle cell sarcoma” or “leptomeningeal melanocytoma” as keywords. The database search was restricted to pediatric patients (age ≤ 18 years). Parameters reported from the literature review included patient age, tumor location, presenting symptoms, treatment modalities (resection, chemotherapy, and/or radiotherapy), leptomeningeal spread at or after the time of treatment, and follow-up length and resulting outcome. RESULTS The authors report 3 rare cases of posterior fossa malignant spindle cell tumors, including Ewing sarcoma in a 13-year-old male; high-grade spindle cell sarcoma, not otherwise specified in a 10-year-old male; and primary leptomeningeal melanocytoma in a 16-year-old female. All 3 patients underwent resection and radiotherapy and either chemotherapy or targeted immunotherapy. At the last follow-up, all patients were alive with either resolution or stable disease. CONCLUSIONS A review of these 3 cases and the existing literature support managing patients with intracranial malignant spindle cell tumors with multimodal therapy that can include a combination of resection, radiotherapy, and chemotherapy or immunotherapy to prolong progression-free and overall survival.

show abstract