A rare case of primary extra-osseous Ewing’s sarcoma presenting in the posterior neck

Year 2019, , 626 - 630, 01.12.2019

Emre Emekli , Uğur Toprak , Suzan Şaylısoy , Lütfiye Demir

https://doi.org/10.21601/ortadogutipdergisi.536096

Abstract

Ewing sarcoma is classified as Ewing’s sarcoma of the bone tissue, extraskeletal Ewing’s sarcoma, peripheral primitive neuroendocrine tumor, malignant small cell tumor of the thoracopulmonary region (Askin) and non-typical Ewing’s sarcoma. Extraosseous Ewing’s sarcoma may be seen throughout the body, however only reported at 4% in the neck. In our case patient has a painless mass growing on his posterior neck for 3 months.On the magnetic resonance imaging, a well-defined, encapsulated, well-demarcated mass lesion between the perivertebral muscles and the subcutaneus fat tissue was detected. Tumor was surgically resected and diagnosis was pathologically Ewing’s sarcoma. 22q11 translocation was detected in the molecular examination. Extraosseous Ewing sarcomas are rarely seen soft tissue masses. Although imaging features are not sufficient to make a specific diagnosis, it is important take the biopsy from the appropriate place and staging the tumor. In addition, complete surgical resection has been shown to associated with better survival rates compared to other Ewing sarcoma family. For this reason, imaging has an important role in the guidance of surgery and resectability of the tumor. Young patients with a fast growing, palpable mass should be evaluated carefully. Although the tumor is thought to be morphologically benign in the first, ekstraosseous Ewing’s sarcoma must be considered in the differential diagnosis of head and neck soft tissue masses.

Keywords

Extraosseous Ewing’s sarcoma, head and neck tumor, magnetic resonance imaging

Ensede Nadir Görülen Ekstraosseöz Ewing Sarkomu Olgusu

Abstract

Ewing sarkomu, kemik dokusunun Ewing sarkomu, iskelet sistemi dışındaki Ewing sarkomu, periferik primitif nöroendokrin tümör, torakopulmoner bölgenin malign küçük hücreli tümörü (Askin) ve tipik olmayan Ewing sarkomu olarak sınıflandırılır. Ekstraosseöz Ewing sarkomu tüm vücutta görülebilir ancak boyunda %4 oranında bildirilmiştir. Olgumuzda, ensesinde 3 aydır giderek büyüyen ağrısız kitlesi olan hastada; manyetrik rezonans görütülemede perivertebral kasların arasında, yağ planlarının arasında büyüyen, kapsüllü, düzgün sınırlı kitle lezyonu saptandı. Cerrahi olarak çıkarılan tümörde patolojik olarak Ewing sarkomu tanısı koyuldu. Moleküler incelemede 22q11 translokasyonu tespit edildi. Ekstraosseöz Ewing sarkomları nadir olarak görülen yumuşak doku kitleleridir. Görüntüleme özellikleri özgün veya tanı koymada yeterli olmasa da, biyopsi için dokunun uygun yerden alınması ve tümörün evrelendirilmesi açısından önemli yer tutar. Ayrıca iskelet dışındaki Ewing sarkomunda, tam cerrahi rezeksiyonun diğer Ewing sarkomu ailesi tümörlerine kıyasla daha iyi sağkalım oranları ile ilişkili olduğu gösterilmiştir. Bu nedenle cerrahiye yön gösterme ve tümörün çıkarılabilirliğinin değerlendirilmesinde görüntüleme önemli yer tutmaktadır. Hızlı büyüyen, ele gelen kitlesi olan genç hastalarda, ilk planda morfolojik olarak benign izlenimi verse de dikkatle değerlendirip baş-boyun yumuşak doku kitlelerinin ayırıcı tanısında düşünülmelidir.

Keywords

Ekstraosseöz Ewins sarkomu, baş boyun kitlesi, manyetik rezonans görüntüleme

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