Ewing's sarcoma family of tumor arising in the adrenal gland--possible diagnostic pitfall in pediatric pathology: histologic, immunohistochemical, ultrastructural, and molecular study.
We present an adrenal Ewing's sarcoma family of tumor (ESFT) arising in an 11-year-old Japanese boy. Although intensive chemoradiotherapy and radical surgery were performed, the patient died of obstinate disease 1 year and 3 months after the initial presentation. The primary site (adrenal gland) with radiologic findings (with foci of calcification), high titer of serum neuron specific enolase, and sheets of monotonous primitive rounded cells on histology mostly favored neuroblastoma. However, a diagnosis of ESFT was confirmed by immunohistochemical profile, including MIC2-positivity and molecular study disclosing EWS-FLI1 chimera gene verified by direct sequencing. Recognition of adrenal ESFT and use of newly developed diagnostic techniques are required for differential diagnosis of undifferentiated small round cell tumor of the adrenal gland.
Kato
K
K
Division of Pathology, Kanagawa Children's Medical Center, Yokohama, Japan.
Kato
Y
Y
Ijiri
R
R
Misugi
K
K
Nanba
I
I
Nagai
J
J
Nagahara
N
N
Kigasawa
H
H
Toyoda
Y
Y
Nishi
T
T
Tanaka
Y
Y
D000071443:12E7 Antigen; D000310:Adrenal Gland Neoplasms / Q000737:chemistry / Q000175:diagnosis* / Q000235:genetics / Q000648:ultrastructure; D015703:Antigens, CD / Q000032:analysis; D014408:Biomarkers, Tumor / Q000032:analysis; D001859:Bone Neoplasms / Q000737:chemistry / Q000175:diagnosis* / Q000235:genetics / Q000648:ultrastructure; D002114:Calcinosis / Q000473:pathology; D015815:Cell Adhesion Molecules / Q000032:analysis; D002648:Child; D004273:DNA, Neoplasm / Q000032:analysis; D003937:Diagnosis, Differential; D006801:Humans; D007124:Immunoenzyme Techniques; D008297:Male; D009363:Neoplasm Proteins / Q000032:analysis; D009447:Neuroblastoma / Q000175:diagnosis; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D010751:Phosphopyruvate Hydratase / Q000097:blood; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS; D020133:Reverse Transcriptase Polymerase Chain Reaction; D012512:Sarcoma, Ewing / Q000737:chemistry / Q000175:diagnosis* / Q000235:genetics / Q000648:ultrastructure; D014157:Transcription Factors / Q000235:genetics
D000071443:12E7 Antigen; D015703:Antigens, CD; D014408:Biomarkers, Tumor; C061828:CD99 protein, human; D015815:Cell Adhesion Molecules; D004273:DNA, Neoplasm; C106031:EWS-FLI fusion protein; D009363:Neoplasm Proteins; D015514:Oncogene Proteins, Fusion; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS; D014157:Transcription Factors; D010751:Phosphopyruvate Hydratase