Ewing′s Sarcoma of the Head and Neck: A Retrospective Analysis of 24 Cases

Allam, Ayman; El-Husseiny, Gamal; Khafaga, Yasser; Kandil, Alaa; Gray, Alan; Ezzat, Adnan; Schultz, Henrik

doi:10.1080/13577149977811

Cited by 79 publications

(97 citation statements)

References 12 publications

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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%

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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Section: Discussionmentioning

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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“…Multimodal treatment yield five year survival rates of about 60% (52-88%) as reviewed in series including atleast one case of Ewings sarcoma / PNET of the head and neck. 6,7 …”

Section: Discussionmentioning

confidence: 99%

Primitive neuroectodermal tumor of thyroid: a rare presentation

Shetty

Savant

Lokhandwala

et al. 2017

Int J Otorhinolaryngol Head Neck Surg

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<p>Extraskeletal Ewing’s sarcoma (EES) commonly arises in the soft tissues of trunk or extremities. EES is rare in the head and neck region; most having been documented in nasal cavity, paranasal sinuses and neck. Head and neck PNETs have an intermediate prognosis. We report the case of a 12 year old boy who presented with primitive neuroectodermal tumor of the thyroid and was treated with multimodal treatment including surgery, chemotherapy and radiotherapy. The patient is alive and fit with a functional larynx. Major drug regimens use vincristine, doxorubicin, ifosfamide or cyclophosphamide, dactinomycin and/or etoposide. Complete surgical excision is undertaken whenever possible to improve long-term survival. However, the relative radiosensitivity of tumors of the Ewing family, suggest multimodal treatment including adjuvant radiotherapy in case of positive margins or poor response to chemotherapy rather than resection with 2-3 cm margins, which would imply laryngeal sacrifice for thyroid tumors.</p>

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“…When long bones are affected, Ewing's sarcoma is typically metadiaphyseal in location [17,18]. Primary Ewing's sarcoma arising from the bones of the head and neck region is extremely rare representing only 1-4% of all Ewing's sarcoma cases [19]. It is the most common malignant bone tumor in children with the peak age being 15 years old.…”

Section: Discussionmentioning

confidence: 99%

Primary extraskeletal Ewing’s sarcoma of the maxilla with intraorbital extension

Varshney

Bist

Gupta

et al. 2007

Indian J Otolaryngol Head Neck S

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Extraskeletal Ewing's sarcoma is often described as a tumour involving the soft tissues of the lower extremities and the paravertebral region. Primary Ewing's sarcoma of the cranium is extremely rare, with only 17 cases reported so far [24]. Involvement of the paranasal sinus is a very rare entity. Involvement of facial bones is characterized by clinical and radiological features distinct from those commonly observed in other sites. Because of the above peculiarities a delay in diagnosis and thus in starting treatment is very probable in such cases. Primary Ewings sarcoma rarely arises in the facial skeleton and only occasionally in the maxilla. To date, there have been 22 cases of maxillary Ewing's sarcoma reported in the English-language literature [25]. We report here a new case of Ewing's sarcoma localized to the maxillary sinus, nose and the orbit, successfully treated by surgery, local high dosage radiotherapy and systemic chemotherapy. Keywords Ewing's sarcoma Paranasal sinusEwing's sarcoma is a malignant neoplasm of uncertain histogenesis, most commonly arising in the skeleton. The head and neck are unusual sites for primary Ewing's sarcoma. Primary Ewing's sarcoma of the paranasal sinus (PNS) is exceptionally rare and most cases are described in the maxillary sinus [16]. Of the 14 cases that we are aware of reported in the literature, the maxillary sinus (7 cases) is the most common site followed by the ethmoid sinus (2 cases ) and the nasal fossa (5 cases ) [1][2][3][4][5][6][7][8][9][10].

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Ewing′s Sarcoma of the Head and Neck: A Retrospective Analysis of 24 Cases

Cited by 79 publications

References 12 publications

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

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

Primitive neuroectodermal tumor of thyroid: a rare presentation

Primary extraskeletal Ewing’s sarcoma of the maxilla with intraorbital extension

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