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

Yıl 2019, , 626 - 630, 01.12.2019

Emre Emekli , Uğur Toprak , Suzan Şaylısoy , Lütfiye Demir

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

Öz

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.

Anahtar Kelimeler

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

Ensede Nadir Görülen Ekstraosseöz Ewing Sarkomu Olgusu

Öz

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.

Anahtar Kelimeler

Ekstraosseöz Ewins sarkomu, baş boyun kitlesi, manyetik rezonans görüntüleme

Kaynakça

• Ambros I, Ambros PF, Strehl S, ve ark. MIC2 is a specific marker for Ewing’s sarcoma and peripheral primitive neuroectodermal tumors. Evidence for a common histogenesis of Ewing’s sarcoma and peripheral primitive neuroectodermal tumors from MIC2 expression and specific chromosome aberration. Cancer 1991; 67: 1886–1893.
• Lewis TB, Coffin CM, Bernard PS, Differentiating Ewing’s sarcoma from other round blue-cell tumors using a RT-PCR translocation panel on formalin-fixed paraffin-embedded tissues. Mod Pathol 2007; 20: 397–404.
• Resnick D, Kyriakos M, Greenway G. Tumors and tumor-like lesions of bone: imaging and pathology of specific lesions. In: Resnick D, Niwayama G, eds. Diagnosis of bone and joint disorders. Philadelphia, Pa: Saunders, 2002; 4060–4073.
• Galyfos G, Karantzikos GA, Kavouras N, Sianou A, Palogos K, Filis K. Extraosseous Ewing Sarcoma: Diagnosis, Prognosis and Optimal Management. Indian J Surg. 2016 Feb; 78(1): 49-53.
• Olson MD, Van Abel KM, Wehrs RN, Garcia JJ, Moore EJ. Ewing sarcoma of the head and neck: The Mayo Clinic experience. Head Neck. 2018 Sep; 40(9): 1999-2006.
• Murphey MD, Senchak LT, Mambalam PK, Logie CI, Klassen-Fischer MK, Kransdorf MJ. From the radiologic pathology archives: ewing sarcoma family of tumors: radiologic-pathologic correlation. Radiographics. 2013 May; 33(3): 803-31.
• Javery O, Krajewski K, O’Regan K, Kis B, Giardino A, Jagannathan J, Ramaiya NH. 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 Dec; 197(6): W1015-22
• Cho SI, Park YH, Cho JH, ve ark. Extraskeletal Ewing’s sarcoma of the head and neck presenting as blindness. Korean J Intern Med. 2007; 22(2): 133-7.
• Choi SH, Kim YJ, Kim H, Kim HJ, Nam SH, Choi YW. The Rare Presentation of Extraskeletal Ewing’s Sarcoma on the Forehead. Arch Plast Surg. 2015; 42(1): 100-2.
• Yang F, Zhao Y, Huang S, Sun R, Lei L. [Four cases of extraskeletal Ewing’s sarcoma in the head and neck and literature review]. Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2013 Sep; 27(18): 1000-2, 1005. Review. Chinese. PubMed PMID:24459926.
• Shin JH, Lee HK, Rhim SC, Cho KJ, Choi CG, Suh DC. Spinal epidural extraskeletal Ewing’s sarcoma: MR findings in two cases. AJNR Am J Neuroradiol 2001; 22(4): 795-98.
• Kennedy JG, Eustace S, Caulfield R, Fennelly DJ, Hurson B, O’Rourke KS. Extraskeletal Ewing’s sarcoma: a case report and review of the literature. Spine (Phila Pa 1976). 2000 Aug 1; 25(15): 1996-9.
• Robbin MR, Murphey MD, Jelinek JS, Temple HT. Imaging of soft tissue Ewing sarcoma and primitive neuroectodermal tumor. Radiology 1998; 209(P): 311.
• Huh J, Kim KW, Park SJ, Kim HJ, Lee JS, Ha HK, Tirumani SH, Ramaiya NH. Imaging Features of Primary Tumors and Metastatic Patterns of the Extraskeletal Ewing Sarcoma Family of Tumors in Adults: A 17-Year Experience at a Single Institution. Korean J Radiol. 2015 Jul-Aug; 16(4): 783-90.
• Biermann JS. Updates in the treatment of bone cancer. J Natl Compr Canc Netw. 2013 May; 11(5 Suppl): 681-3.