A case of Ewing's sarcoma in the mandible and the skull base

Year 2003, Volume: 11 Issue: 5, 144 - 147, 13.10.2003

Mahmut Tayyar Kalcıoğlu Murat Cem Miman Tamer Erdem Semih Öncel Bülent Mızrak

Abstract

A thirteen-year-old boy with Evving’s sarcoma was presented.The treatment was considerably delayed because of the initial diagnostic difficulties due to its insidious presentation and the unvvillingness of the child’s parents to further investigations and treatment. During a three-year delay, the tumor turned out to be a gross painful mass from a painless lesion.Cranial computed tomography and magnetic resonance imaging with contrast showed a destructive lesion extensively involving the right mandible and the skull base, with inva- sion to the intracranial space. Facial and intratemporal portions of the mass were removed totally, but the intracranial extension could not be totally excised because of cavernous sinüs involvement. Histologic and immunohistochemical find- ings were consistent with the diagnosis of Evving’s sarcoma.The patient received systemic chemotherapy and radiothera- py follovving surgery. A month after radiation therapy, radiologic investigations shovved a lung mass suggestive of metastasis. He died two days after hospitalization, from an intracra­ nial hemorrhage associated with the intracranial mass.

Keywords

Child, combined modality therapy, head and neck neoplasms, prognosis, sarcoma, Ewing's/diagnosis/surgery/radiography/chemotherapy

Mandibula ve kafa tabanında Ewing sarkomu: Olgu sunumu

Abstract

On üç yaflında bir erkek çocukta Ewing sarkomuna rast- landı. Tedavi, tümörün bafllangıçta az semptom vermesin- den kaynaklanan tanı güçlükleri ve hastanın anne-babası- nın daha ileri arafltırma ve tedaviyi kabul etmemeleri ne- deniyle önemli ölçüde gecikti. Yaklaflık üç yıllık bir gecik- meden sonra, tümörün ağrısız bir lezyondan ağrılı büyük bir kitleye dönüfltüğü görüldü. Bilgisayarlı tomografi ve kontrastlı manyetik rezonans görüntülerinde sağ mandibu- la ve kafa tabanını yaygın olarak tutan ve kafa içi uzanım gösteren tümör izlendi. Lezyonun yüzdeki ve intratempo- ral bölümleri tümüyle çıkarılırken, kafa içi uzanımı, kaver- nöz sinüs tutulumu yüzünden tümüyle çıkarılamadı. Histo- lojik ve immünkimyasal bulgular Ewing sarkomu tanısını destekler nitelikteydi. Hastaya cerrahi tedaviden sonra sis- temik kemoterapi ve radyoterapi uygulandı. Radyoterapi- den bir ay sonraki radyolojik incelemelerde akciğerde me- tastaz düflündüren bir kitle saptandı. Hasta, yatırılmasın- dan iki gün sonra, kafa içi kitleye bağlı geliflen intrakranyal kanama nedeniyle yaflamını yitirdi

Keywords

Çocuk, kombine tedavi, bafl-boyun neoplazileri, prognoz, sarkom, Ewing/tanı/cerrahi/radyoterapi/kemoterapi

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