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

Abstract

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.

Adamantinoma-Like Ewing Sarcoma Mimicking Basal Cell Adenocarcinoma of the Parotid Gland: A Case Report and Review of the Literature

“…Similar to other EFTs, AES were initially described to mainly affect the long bones of the extremities and only rarely other areas like the head and neck [7,[25][26][27]. However, cases genotypically and phenotypically compatible with this tumor have also been reported to affect soft tissue and even viscera with or without associated osseous involvement [5,[28][29][30].…”

Small round blue cell tumors of the sinonasal tract: a differential diagnosis approach

“…a Pituitary hormones and/or pituitary transcription factors, but may include or peptides and hormones (ADH, oxytocin). Based on data aggregate in part from Bahrami et al, 36 Bell et al, 51 Bishop et al, 74 Bourne et al, 143 Chapman-Fredricks et al, 88 Folpe et al, 151 Hafezi et al, 150 Hicks et al, 30 Nikitakis et al, 166 Thompson et al, 7 Thompson et al, 124 Thompson et al, 159 and Wooff et al 57 …”

“…Tumors develop slightly more often in males, most commonly in young patients, but older patients may be affected. [148][149][150] The skull and jaws are much more commonly affected than the sinonasal tract. 150 Symptoms are non-specific, but tend to develop rapidly, with imaging studies showing orbital or intracranial extension.…”

“…A strong and diffusive membranous CD99 reactivity (Figure 14) is the most sensitive marker for this tumor; nuclear FLI1 and ERG are also commonly identified, but are not specific. 6,50,81,153,154 Importantly, pancytokeratin may be expressed in a dot-like pattern (up to 30% of cases), but more often in adamantinoma-like tumors; 150,152,155 p63 is infrequently identified; 57 neural markers may be seen (NSE, S100 protein, synaptophysin, chromogranin); CD117 is uncommon, and desmin is rarely coexpressed. A FISH EWSR1 break-apart probe helps to identify the class of tumor, although it does not confirm the translocation partner (which is usually FLL1).…”

“…The 50-75% 5-year survival for sinonasal Ewing sarcoma is much better than other sites, but local recurrence and metastases, when they develop are usually soon (2 years) after initial presentation. 147,150 Tumors are managed with multimodality therapy, but there is a risk of posttreatment sarcoma development. Poor prognosis may be related to p53 aberrations.…”