Primary Ewing's Sarcoma of the Ethmoid Sinus with Intracranial and Orbital Extension: Case Report and Literature Review

Li, Mingsi; Hoschar, Aaron P.; Budd, Thomas; Chao, Samuel T.; Scharpf, Joseph

doi:10.1055/s-0032-1312209

Cited by 3 publications

(6 citation statements)

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“…Indeed, complete radiologic resolution of locally advanced sinonasal adamantinoma-like Ewing sarcoma following an Ewing sarcoma-specific chemotherapy regimen has been previously reported. 9 In our case, the tumor mass demonstrates stromal changes consistent with treatment effect, even though no tumor cell necrosis was identified. Even though the final margin was focally positive (< 5 mm linear extent), local control can be achieved with adjuvant chemoradiation.…”

Section: Discussionmentioning

confidence: 45%

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Outcome for Neoadjuvant Treatment of Parotid Gland Adamantinoma-Like Ewing Sarcoma: Case Report and Review of Literatures

et al. 2022

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Background. Adamantinoma-like Ewing sarcoma typically shows t(11;22) EWSR1::FLI1 translocation and complex epithelial differentiation. It poses a diagnostic challenge, especially in the head and neck region, due to its under-recognition and significant histologic overlap with other malignancies. Neoadjuvant and adjuvant treatment information on head and neck Adamantinoma-like Ewing sarcoma is limited. Case Presentation. Herein, we report a case of a 78-year-old female with Adamantinoma-like Ewing sarcoma of the parotid gland, including the imaging findings and clinical response to neoadjuvant therapy followed by surgery. The efficacy of neoadjuvant therapy in the treatment of Adamantinoma-like Ewing sarcoma is discussed in the context of a review of pertinent literature. Conclusion. Adamantinoma-like Ewing sarcoma in the head and neck is frequently misdiagnosed as poorly differentiated squamous cell carcinoma or a basaloid salivary gland carcinoma. Adamantinoma-like Ewing sarcoma is a EWS1::FLI1 translocation driven tumor; frequently misdiagnosed on head and neck biopsies as poorly differentiated carcinoma, or squamous cell carcinoma. Ewing sarcoma-specific chemoregimen appears effective for this entity. If diagnosed early, patient may be amenable to neoadjuvant therapy, which may improve surgical and cosmetic outcomes. This is especially important in head and neck regions.

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Section: Discussionmentioning

confidence: 45%

“…7 Only a limited number of cases in the head and neck have been reported to date. [7][8][9] The majority of head and neck adamantinomalike Ewing sarcoma occur in the salivary glands, followed by thyroid gland and sinonasal tract. 7 There appears to be no sex predilection.…”

Section: Discussionmentioning

confidence: 99%

Outcome for Neoadjuvant Treatment of Parotid Gland Adamantinoma-Like Ewing Sarcoma: Case Report and Review of Literatures

et al. 2022

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“…Combination of systemic chemotherapy and radiotherapy may be an effective alternative to surgical resection in selected cases for local tumor control, where surgical resection results in significant cosmetic mutilation and/or physical handicap. 21,40 However, a combination of surgical resection, chemotherapy, and radiotherapy is associated with better locoregional control and survival rate compared with those without surgical resection. 40 In a study of 1039 localized ESFT patients who underwent treatment, the 5-year overall event-free survival estimate for extraosseous ESFTs was 76% compared with 69% for patients with osseous ESFTs.…”

Section: Discussionmentioning

confidence: 99%

“…21,40 However, a combination of surgical resection, chemotherapy, and radiotherapy is associated with better locoregional control and survival rate compared with those without surgical resection. 40 In a study of 1039 localized ESFT patients who underwent treatment, the 5-year overall event-free survival estimate for extraosseous ESFTs was 76% compared with 69% for patients with osseous ESFTs. 41 However, patients with systemic metastasis at presentation have a poor prognosis with survival rate of only 20%.…”

Section: Discussionmentioning

confidence: 99%

Primary orbital Ewing sarcoma family of tumors: a study of 12 cases

Kaliki

Rathi

Palkonda

2017

Eye

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PurposeThe purpose of this study is to discuss the clinical presentation, management, and outcomes of patients with primary orbital Ewing sarcoma family of tumors (ESFTs).Patients and methodsRetrospective study of 12 patients with biopsy-proven primary orbital ESFT.ResultsThe mean age at presentation of primary orbital ESFT was 12 years (median, 8 years; range, 5 months to 28 years). There were seven (58%) females and five (42%) males. The presenting complaints included proptosis (n=10; 83%) and swelling in the upper eyelid (n=2; 17%). The mean duration of symptoms was 9 weeks (median, 5 weeks; range, 2-24 weeks). Tumor epicenter was located in the superior orbit (n=6; 50%), lateral orbit (n=3; 25%), inferior orbit (n=2; 17%), and medial orbit (n=1; 8%). Computed tomography of the orbits revealed predominant bony lesion (n=10; 83%) or isolated soft tissue/extraosseous lesion (n=2, 17%). At presentation, extraorbital extension was noticed in 10 patients including intra cranial extension (n=7; 58%), extension into temporal fossa (n=4; 33%), nasal cavity (n=2; 17%), maxillary sinus (n=2; 17%), and ethmoid sinus (n=1, 8%). Systemic metastases at presentation was detected in five (42%) patients involving the bone marrow (n=4; 33%), kidney (n=1; 8%), and retroperitoneal lymphnode (n=1; 8%). Multi-modal treatment including a combination of neoadjuvant chemotherapy, excision biopsy/debulking, and/or radiotherapy was given. Over a mean follow-up period of 21 months (median, 7 months; range, 1-152 months), disease-related death occurred in 11 (92%) cases.ConclusionPrimary orbital ESFT is aggressive at presentation and is associated with poor prognosis.

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“…In 2011, Hafezi et al reviewed a total of 63 cases of any age by merging a multicenter series of 14 cases with 49 cases collected by review of the English literature. Subsequently, an additional 11 cases of sinonasal Ewing's sarcoma have been published. Therefore, the overall number is 74, with only 22 patients older than 18 years.…”

Section: Introductionmentioning

confidence: 99%