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A Bump on the Head - An Unusual Presentation of Extraosseus Ewing Sarcoma in a 3-month-old Male
T Dao MD†, T Beydoun DO†, P Cornejo MD†, S Taylor PA‡, D Carpentieri MD‡, M Patel MD†
Department of Medical Imaging†, Department of Pathology‡
Phoenix Children's Hospital
Phoenix, Arizona, USA
Abstract:
Extraosseus Ewing sarcoma arising in the head and neck is extremely rare, and even more rare in the neonatal period. We present a case of extraosseus Ewing sarcoma involving the right eyelid in a 3-month-old male with a progressively enlarging mass involving the right forehead that was present since 2 weeks of age. The mass was initially thought to be a hemangioma and treated as such, but did not respond to propranolol and steroid treatment. Excisional biopsy was performed and pathology demonstrated Ewing sarcoma. Extraosseus Ewing sarcoma is a rare entity in the neonatal population and should be included in the differential diagnosis of head and neck soft tissue masses despite patient age.
References
1. Murphey MD, Senchak LT, Mambalam PK et-al. From the radiologic pathology archives: ewing sarcoma family of tumors: radiologic-pathologic correlation. Radiographics. 2013;33 (3): 803-31. http://pubs.rsna.org/doi/10.1148/rg.333135005?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%3dpubmed
2. Javery O, Krajewski K, O'Regan K et-al. A to Z of extraskeletal Ewing sarcoma family of tumors in adults: imaging features of primary disease, metastatic patterns, and treatment responses. AJR Am J Roentgenol. 2011;197 (6): W1015-22. http://www.ajronline.org/doi/a....bs/10.2214/AJR.11.66
3. El Weshi A, Allam A, Ajarim D et-al. Extraskeletal Ewing's sarcoma family of tumours in adults: analysis of 57 patients from a single institution. Clin Oncol (R Coll Radiol). 2010;22 (5): 374-81. http://www.clinicaloncologyonl....ine.net/article/S093
4. Kennedy JG, Eustace S, Caulfield R et-al. Extraskeletal Ewing's sarcoma: a case report and review of the literature. Spine. 2000;25 (15): 1996-9. http://www.ncbi.nlm.nih.gov/pubmed/10908947


MSK gynecologic cancer experts describe surgery to remove the ovaries and fallopian tubes of women at high risk of developing ovarian cancer.
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{partial transcript}
Let’s say we have a woman who has a BRCA1 or BRCA2 mutation. The hot topic is: should she have her ovaries taken out? Talk to me about that.
For patients that have a known genetic mutation, their lifetime risk of developing ovarian cancer is increased substantially. General population-based risk for a woman in this country is 1.7 percent – that is the chance that in her lifetime, at some point, she’ll develop ovarian cancer. For BRCA1 or BRCA2 gene mutation carriers, that risk can be as high as 40 to 60 percent lifetime risk. That’s huge.
So really, it goes to understanding your family history. If there are triggers, then meet with a genetic counselor to get the genetic testing because that can be so important for family members who are not infected with the disease in terms of what they can do to help protect themselves.
We’ve already talked about how there’s not a very effective screening tool – the best that we have is CA 125 and the pelvic ultrasound. We do recommend that until the completion of childbearing and at that point, we start to have a discussion about prophylactic or preventative removal of the ovaries because of their extraordinary high risk. That does confer about a 90 to 95 percent risk reduction – so it really goes go a long way to decrease one’s risk.


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