Primary Ewing's sarcoma involving the bones of the head and neck

Siegal, Gene P.; Oliver, William R.; Reinus, William R.; Gilula, Louis A.; Foulkes, Mary A.; Kissane, John M.; Askin, Frederic B.

doi:10.1002/1097-0142(19871201)60:11<2829::aid-cncr2820601139>3.0.co;2-s

Cited by 121 publications

(114 citation statements)

References 48 publications

(3 reference statements)

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“…The skull and mandible are the most frequent sites. 1,2 It is rare after the third decade and occurs most often in the second decade. It is more common in males than females.…”

Section: Discussionmentioning

confidence: 99%

“…3 Generally it presents with pain and local swelling, dilated veins, hyperthermia, anemia, increased erythrocyte sedimentation rate and leukocytosis. 2,3,4 A history of previous trauma is present in many reported cases. The initial evaluation includes radiographic study of the suspected area, which shows areas of bone rarefaction, frequently associated with increased density, periosteal reaction, and bone neoformation resulting in an "onion layers" appearance.…”

Section: Discussionmentioning

confidence: 99%

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Ewing's sarcoma of the head and neck

et al. 2000

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a b s t r a c tCONTEXT: Ewing's sarcoma is a rare neoplasm, which usually arises in long bones of the limbs and in flat bones of the pelvis, with the involvement of head and neck bones being very unusual.CASE REPORT: a case of Ewing's sarcoma occurring in the mandible of a 35-year-old female. Pain and swelling of the tumor were the main complaints. The early hypothesis was an undifferentiated malignant neoplasm, possibly a sarcoma. The CT scan depicted an expansive lesion, encapsulated, with septa and characteristics of soft tissue, involving the left side of the mandible and extending to the surrounding tissues. The patient underwent surgical excision of the lesion, the definitive diagnosis of Ewing's sarcoma was established, and the patient commenced on radiotherapy.

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“…The skull and mandible are the most frequent sites. 1,2 It is rare after the third decade and occurs most often in the second decade. It is more common in males than females.…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Ewing's sarcoma of the head and neck

et al. 2000

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“…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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“…Primary ES arising from the mandible is extremely rare (3). Only 4-9% of all ES originate in the head and neck region, usually involving the mandible (4). Most ES lesions in the mandible have been located in the posterior regions (1).…”

Section: Introductionmentioning

confidence: 99%

“…Most ES lesions in the mandible have been located in the posterior regions (1). Most authors claim a better prognosis for ES of the head and neck region as compared to that arising in other anatomic locations (4,5).…”

Section: Introductionmentioning

confidence: 99%