J Korean Med Sci.  2007 Sep;22(Suppl):S159-S163. 10.3346/jkms.2007.22.S.S159.

Primary Pulmonary Ewing's Sarcoma/Primitive Neuroectodermal Tumor in a 67-year-old Man

Affiliations
  • 1Department of Family Medicine, Korea University, College of Medicine, Seoul, Korea.
  • 2Department of Pathology, Korea University, College of Medicine, Seoul, Korea.
  • 3Department of Pulmonology, Korea University, College of Medicine, Seoul, Korea.
  • 4Department of Diagnostic Radiology, Korea University, College of Medicine, Seoul, Korea.
  • 5Department of Family Medicine, Hallym University, College of Medicine, Chunchon, Korea. rohyk@hallym.ac.kr

Abstract

Extraskeletal Ewing's sarcoma (EES) is a branch of neuroectodermal tumor (PNET), which is very rare soft tissue sarcoma. We report a case of EES/PNET arising is the lung of a 67-yr-old man. Computed tomography, bone scintigraphy, and positron emission tomography confirmed the mass to have a primary pulmonary origin. The mass showed positive reactivity in the Periodic Acid Schiff (PAS) stain and MIC-2 immunoreactivity in immunohistochemical stain. Fluorescence in situ hybridization (FISH) was performed, which revealed an EWSR1 (Ewing sarcoma breakpoint region 1) 22q12 rearrangement. The diagnosis was confirmed both pathologically and genetically. The mass lesion was resected, and the patient is currently undergoing chemotherapy.

Keyword

Sarcoma; Ewing's; Neuroectodermal Tumors; Primitive; Peripheral; Lung

MeSH Terms

Aged
Calmodulin-Binding Proteins/genetics
Chromosome Breakage
Chromosomes, Human, Pair 22/genetics
Diagnosis, Differential
Humans
Immunohistochemistry
In Situ Hybridization, Fluorescence
Lung Neoplasms/*diagnosis/genetics/metabolism/pathology
Male
Neuroectodermal Tumors, Primitive,
RNA-Binding Proteins/genetics
Sarcoma, Ewing's/*diagnosis/genetics/metabolism/pathology

Figure

  • Fig. 1 (A, B) An approximately 4 cm round solitary mass is shown at the left lower lung field on posterior-anterior and left lateral chest roentgenograms. (C) The chest CT scan showing a heterogeneously contrast-enhancing mass lesion in the left lung.

  • Fig. 2 (A) Light microscopy of the tumor shows uniformly solidly packed round cells with a distinct unclear membrane (H&E, ×400). (B) Irregularly vacuolated cytoplasm secondary to glycogen deposition (PAS, ×400). (C) Strong membranous MIC2 immunoreactivity (LSAB, ×400).

  • Fig. 3 Well demarcated whitish soft tissue mass having extensive tumoral necrosis inside.

  • Fig. 4 Fluorescence in situ hybridization revealed EWSR1 22q12 rearrangement detected by using LSI® EWSR1 (22q12) Dual Color Break-Apart Rearrangement probe.


Cited by  1 articles

A Case of Long-term Survival in a Patient with Primary Primitive Neuroectodermal Tumor of the Lung
Lae Hyung Kang, Hyeong Jin Kim, Jin Ho Jang, Jun Hyun Kim, Kyoung Un Choi, Doosoo Jeon
Kosin Med J. 2018;33(2):263-270.    doi: 10.7180/kmj.2018.33.2.263.


Reference

1. Tsuji S, Hisaoka M, Morimitsu Y, Hashimoto H, Jimi A, Watanabe J, Eguchi H, Kaneko Y. Peripheral primitive neuroectodermal tumour of the lung: report of two cases. Histopathology. 1998. 33:369–374.
Article
2. Imamura F, Funakoshi T, Nakamura S, Mano M, Kodama K, Horai T. Primary primitive neuroectodermal tumor of the lung: report of two cases. Lung Cancer. 2000. 27:55–60.
Article
3. Mikami Y, Nakajima M, Hashimoto H, Irei I, Matsushima T, Kawabata S, Manabe T. Primary pulmonary primitive neuroectodermal tumor (PNET). A case report. Pathol Res Pract. 2001. 197:113–119.
4. Kahn AG, Avagnina A, Nazar J, Elsner B. Primitive neuroectodermal tumor of the lung. Arch Pathol Lab Med. 2001. 125:397–399.
Article
5. Ewing J. Classics in oncology. Diffuse endothelioma of bone. Proceedings of the New York Pathological Society, 1921. CA Cancer J Clin. 1972. 22:95–98.
6. Tefft M, Vawter GF, Mitus A. Paravertebral "round cell" tumors in children. Radiology. 1969. 92:1501–1509.
Article
7. Kang MS, Yoon HK, Choi JB, Eum JW. Extraskeletal Ewing's sarcoma of the hard palate. J Korean Med Sci. 2005. 20:687–690.
Article
8. Charney DA, Charney JM, Ghali VS, Teplitz C. Primitive neuroectodermal tumor of the myocardium: a case report, review of the literature, immunohistochemical, and ultrastructural study. Hum Pathol. 1996. 27:1365–1369.
Article
9. Danner DB, Hruban RH, Pitt HA, Hayashi R, Griffin CA, Perlman EJ. Primitive neuroectodermal tumor arising in the pancreas. Mod Pathol. 1994. 7:200–204.
10. Koo HL, Jun SY, Choi G, Ro JY, Ahn H, Cho KJ. Primary Primitive neuroectodermal tumor of the kidney: report of two cases. Korean J Pathol. 2003. 37:145–149.
11. Christie DR, Bilous AM, Carr PJ. Diagnostic difficulties in extraosseous Ewing's sarcoma: a proposal for diagnostic criteria. Australas Radiol. 1997. 41:22–28.
Article
12. Shin JH, Lee HK, Rhim SC, Cho KJ, Choi CG, Suh DC. Spinal epidural extraskeletal Ewing sarcoma: MR findings in two cases. AJNR Am J Neuroradiol. 2001. 22:795–798.
13. Gyorke T, Zajic T, Lange A, Schafer O, Moser E, Mako E, Brink I. Impact of FDG PET for staging of Ewing sarcomas and primitive neuroectodermal tumours. Nucl Med Commun. 2006. 27:17–24.
14. Cotterill SJ, Ahrens S, Paulussen M, Jurgens HF, Voute PA, Gadner H, Craft AW. Prognostic factors in Ewing's tumor of bone: analysis of 975 patients from the European Intergroup Cooperative Ewing's Sarcoma Study Group. J Clin Oncol. 2000. 18:3108–3114.
Article
15. Eroglu A, Kurkcuoglu IC, Karaoglanoglu N, Alper F, Gundogdu C. Extraskeletal Ewing sarcoma of the diaphragm presenting with hemothorax. Ann Thorac Surg. 2004. 78:715–717.
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