Extraosseous Ewing Sarcoma Arising at the Papilla of Vater

Abstract Aim: To analyse the presentation, diagnosis and patterns of care of extraosseous Ewing sarcoma treated at our institution between 2008 and 2018. Methods: Electronic medical records of extraosseous Ewing sarcoma patients treated at our institution between January 2008 and April 2018 were reviewed. Kaplan–Meier curves were plotted to assess the overall and disease-free survival with 95% confidence intervals. A univariate analysis was carried out to assess the impact of variables such as surgical excision, completeness of surgery, completeness of chemotherapy and addition of radiation therapy on the survivorship. Results: The records of 65 patients treated at our institution were available for review. The mean age was 26·4 years. The most frequent sites of extraosseous Ewing tumour were kidney—9/65 (13·8%) and brain—10/65 (15·4%). Sixteen (24·6%) patients presented with inoperable/metastatic disease at diagnosis. The other 49 (75·4%) had localised disease at presentation. The median overall survival of the 49 non-metastatic patients was 46 months, and the disease-free survival was 45 months. Conclusion: Extraosseous Ewing sarcoma is a rare and aggressive tumour diagnosed by molecular techniques. Multi-modality treatment including surgical resection with wide margins, adjuvant radiation when indicated and completion of systemic chemotherapy results in optimum outcomes.

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Primary Intradural Extraosseous Ewing Sarcoma of the Spine: Case Report and Literature Review

Neurosurgery ◽

10.1227/neu.0b013e318223b7c7 ◽

2011 ◽

Vol 69 (4) ◽

pp. E995-E999 ◽

Cited By ~ 13

Author(s):

Isaac O Karikari ◽

Ankit I Mehta ◽

Shahid Nimjee ◽

Tiffany R Hodges ◽

June Tibaleka ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Ewing Sarcoma ◽

Correct Diagnosis ◽

Immunohistochemical Analysis ◽

Leg Pain ◽

Treatment Modalities ◽

Magnetic Resonance Imaging Mri ◽

The Right ◽

Extraosseous Ewing Sarcoma

Abstract BACKGROUND AND IMPORTANCE: To report a rare case of spinal intradural extraosseous Ewing sarcoma in an adult and review current literature. Although Ewing sarcoma belongs to the family, the treatment modalities are different, and thus the correct diagnosis is very important despite its rare occurrence. CLINICAL PRESENTATION: A 56-year-old woman presented with nocturnal bilateral buttock and leg pain. Magnetic resonance imaging (MRI) showed an enhancing intradural extramedullary extraosseous tumor at L1. INTERVENTION: A T12-L2 laminectomy was performed to resect the tumor. Immunohistochemical analysis confirmed the diagnosis of Ewing sarcoma. A thorough diagnostic workup did not reveal any bony origin of the tumor. Primary intradural central nervous system Ewing sarcoma is infrequently encountered and shares imaging and histopathological features with central primitive neuroectodermal tumors. Establishment of the right diagnosis is crucial because it mandates a distinct workup and treatment modality different from that for central primitive neuroectodermal tumor. Although osseous Ewing sarcoma predominantly occurs in children and young adults, extraosseous central nervous system Ewing sarcoma is not uncommon in adults and should therefore be considered in the differential diagnosis of extraosseous small blue cell tumors in adult patients.

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Extraosseous Ewing sarcoma in the mesentery: the first report of cases in children

Pediatric Surgery International ◽

10.1007/s00383-015-3782-0 ◽

2015 ◽

Vol 31 (10) ◽

pp. 995-999 ◽

Cited By ~ 1

Author(s):

Soichi Shibuya ◽

Shigeru Takamizawa ◽

Tomoko Hatata ◽

Kazutoshi Komori ◽

Yoshifumi Ogiso ◽

...

Keyword(s):

Ewing Sarcoma ◽

First Report ◽

Extraosseous Ewing Sarcoma

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Primary pediatric intraspinal sarcomas

Journal of Neurosurgery Pediatrics ◽

10.3171/2009.3.peds08272 ◽

2009 ◽

Vol 4 (3) ◽

pp. 222-229 ◽

Cited By ~ 17

Author(s):

Paul Klimo ◽

Patrick J. Codd ◽

Holcombe Grier ◽

Liliana C. Goumnerova

Keyword(s):

Peripheral Nerve ◽

Ewing Sarcoma ◽

Pediatric Population ◽

Spinal Nerve ◽

Neurological Deficits ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath ◽

Lumbar Spinal ◽

Extraosseous Ewing Sarcoma

Sarcomas that arise from within the spinal canal are rare, particularly within the pediatric population. In general, these primary intraspinal sarcomas are highly aggressive, posing unique treatment challenges with respect to surgery and choice of adjuvant therapy. The goal must be to obtain the most complete resection possible to minimize the risk of recurrence and metastasis, while preventing potential neurological deficits that may result from aggressive surgery. Among these primary intraspinal sarcomas are malignant peripheral nerve sheath tumors and members of the Ewing sarcoma family of tumors. The authors present 3 cases of unique spinal sarcomas in children—2 malignant peripheral nerve sheath tumors in patients without neurofibromatosis and an intradural extraosseous Ewing sarcoma arising from the sensory component of a lumbar spinal nerve—and discuss their management and outcome with a review of the current literature.

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