Ewing’s sarcoma of the head and neck in children

Vaccani, Jean‐Philippe; Forte, Vito; Jong, A.L. de; Taylor, Glenn

doi:10.1016/s0165-5876(99)00030-0

Cited by 83 publications

(56 citation statements)

References 11 publications

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“…Furthermore, since many patients with ES are young and are often physically active, the pain is frequently mistaken for bone growth or injury, resulting in a delayed or misdiagnosis (42). ES family tumors often progress rapidly and result in palpable swelling as observed in the majority of case studied (24,25,(43)(44)(45). In the oral cavity, the affected area is seen with tooth mobility (29), corroborating our findings.…”

Section: Histopathological Features Of Ewing's Sarcoma/peripheral Psupporting

confidence: 83%

“…The medical literature contains only single case reports or small case series including six patients (3,24,25,36). To determine the exact number of children/young adults with primary ES or ES/pPNET of the jaws in the medical literature is very difficult, and in fact, the data reported are confusing.…”

Section: Discussionmentioning

confidence: 99%

“…This treatment in ES typically refers to a combination of surgery and radiochemotherapy (15,25,36,(49)(50)(51), with the same treatment being reported for ES/pPNET (20,38,52,53). Most contemporary therapies call for multidrug chemotherapy, followed by local therapies (radiation/surgery) (42).…”

Section: Figure 3 Periodic Acid-schiff (Pas) and Immunostaining In Ementioning

confidence: 99%

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Ewing's Sarcoma Family Tumors in the Jaws: Case Report, Immunohistochemical Analysis and Literature Review

Casaroto

Sampieri

Soares

et al. 2017

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Section: Histopathological Features Of Ewing's Sarcoma/peripheral Psupporting

confidence: 83%

Section: Discussionmentioning

confidence: 99%

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Ewing's Sarcoma Family Tumors in the Jaws: Case Report, Immunohistochemical Analysis and Literature Review

Casaroto

Sampieri

Soares

et al. 2017

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“…There was a slight male predominance [7] and the majority of patients were less than 30 years of age [6] with a median age of 16 years [7]. Although in some series the prognosis has been suggested to be more favorable for the head and neck region [4,8,18], there have been no large studies of EFT in the sinonasal area specifically and most of the previously reported cases have lacked molecular confirmation. Soft tissue EFT has been reported to have a greater chance of survival for those cases that achieve complete tumor removal prior to chemotherapy [5].…”

Section: Discussionmentioning

confidence: 99%

“…Amongst bone EFTs, the head and neck (skull) accounts for 3.8% of cases in one large study [2]. Bone and soft tissue EFTs in the head and neck have been reported to represent anywhere from 1-7% of EFTs in some studies [3,4] and up to 18% of EFTs in childhood in others (5). Primary sinonasal EFT is even rarer as a subgroup and represents only a small subset of these head and neck cancers [6].…”

Section: Introductionmentioning

confidence: 99%

Ewing’s Family of Tumors of the Sinonasal Tract and Maxillary Bone

Hafezi

Seethala

Stelow

et al. 2010

Head and Neck Pathol

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The Ewing's family of tumors (EFT) are malignant neoplasms affecting children and young adults. Most cases arise in the long bones or the pelvis. Primary EFT of head and neck is uncommon and primary sinonasal EFT is even rarer. Previous studies have not focused on the sinonasal region specifically, and the published literature on sinonasal EFT consists of sporadic case reports. Fourteen cases of sinonasal EFT were available and had H&Es for review and immunohistochemical stains for CD99, S100, keratins, synaptophysin and desmin. FISH or RT-PCR was performed for EWSR1 abnormalities on 8 cases. The 14 identified patients included 5 males and 9 females, ranging from 7-70 years of age (mean 32.4 years). Tumors involved nasal cavity (5), sinuses (5) or both (4). Five patients had dural, orbital or brain involvement. The majority involved bone radiologically and/or microscopically. All cases were composed of small cells with variable cytoplasmic clearing. Focal or prominent nesting was noted in most cases. All cases were positive for CD99. Keratins (AE1/3 and/or CAM5.2), S100 and synaptophysin were positive in 4, 3 and 5 cases, respectively. All cases were negative for desmin. The 8 cases tested by FISH or RT-PCR were positive for EWSR1 abnormalities. Follow-up in 8 patients ranged from 1-168 months (average 11.3 m) showing 1 death due to metastatic disease, 1 death due to local disease, 1 patient alive with metastases and 5 patients disease-free at last follow-up. Interestingly, however, an analysis of the literature suggests a better prognosis for sinonasal EFT than EFT overall.

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Adamantinoma‐like Ewing sarcoma of the thyroid

Jones

Maleki

2020

Diagnostic Cytopathology

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Adamantinoma-like Ewing sarcoma (ALES) is a rare variant of Ewing sarcoma family of tumors (ESFTs). ALES is characterized by translocations of the EWSR1 (Ewing sarcoma breakpoint region 1) gene on chromosome 22, morphologic features of small round blue cell tumors with focal squamous differentiation, and a unique immunoprofile. Herein, we report a 21-year-old patient who presented with severe, acute onset back pain. Imaging revealed a large, exophytic, heterogeneously enhancing mass in the left thyroid and numerous lytic bone lesions. Fine-needle aspiration of the thyroid, revealed a cellular smear with "small round blue cell" morphology. The unique immunoprofile of positive ESFT markers (NKX2.2 and CD99), along with positive markers of squamous epithelial differentiation (AE1/AE3 and p40), led to a diagnosis of ALES. This was confirmed by fluorescence in situ hybridization, which demonstrated EWSR1 rearrangement in 74% of nuclei.

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Ewing’s sarcoma of the head and neck in children

Cited by 83 publications

References 11 publications

Ewing's Sarcoma Family Tumors in the Jaws: Case Report, Immunohistochemical Analysis and Literature Review

Ewing's Sarcoma Family Tumors in the Jaws: Case Report, Immunohistochemical Analysis and Literature Review

Ewing’s Family of Tumors of the Sinonasal Tract and Maxillary Bone

Adamantinoma‐like Ewing sarcoma of the thyroid

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