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

• Lee YY
• Kim DH
• Lee JH
• Choi JS
• In KH
• Oh YW
• Cho KH
• Roh YK

• KMID: 1785807
• DOI: http://doi.org/10.3346/jkms.2007.22.S.S159

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.

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  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).

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

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

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