Ganglion cells in Ewing's sarcoma following chemotherapy: a case report.
A case of Ewing's sarcoma of the bone, arising in the right radius of a 12-year-old girl, which showed unique histologic features after pre-operative treatment, is reported. The light microscopic features of a biopsy sample were those of a small round cell tumor showing positive immunoreaction with antibodies against the product of the MIC 2 gene (O13), neuron-specific enolase, neurofilament, and synaptophysin, but no morphological differentiation. The patient received combined intensive multi-drug chemotherapy and radiation before surgery. Examination of the surgical specimen showed that the tumor was less cellular than that in the biopsy specimen, and was composed mainly of loosely textured large cells mimicking ganglion cells, occasionally forming Homer-Wright rosettes. An immunohistochemical study revealed that neural differentiation was enhanced. Immunoreactivity for Leu-7 also became positive. Although the patient underwent postoperative chemotherapy, she died of multiple lung and bone metastases 30 months after the diagnosis. Autopsy showed that metastatic foci were made up of densely packed small round cells like those seen in the biopsy samples, but associated with prominent Homer-Wright rosettes. To the authors' knowledge, this is the first report of a tumor being replaced almost entirely by ganglion cells after pre-operative chemotherapy and radiotherapy.
Orthopedic Division, National Cancer Center Hospital, Tokyo, Japan.
G
G
Maeda
F
F
Masui
R
R
Yokoyama
T
T
Shimoda
Y
Y
Matsuno
K
K
Mukai
K
K
Ohtomo
Y
Y
Beppu
H
H
Fukuma
D000071443:12E7 Antigen; D015703:Antigens, CD / Q000378:metabolism; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects*; D001859:Bone Neoplasms / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology*; D018959:CD57 Antigens / Q000378:metabolism; D015815:Cell Adhesion Molecules / Q000378:metabolism; D002471:Cell Transformation, Neoplastic / Q000139:chemically induced* / Q000378:metabolism / Q000473:pathology; D002648:Child; D017931:DNA Primers / Q000737:chemistry; D017809:Fatal Outcome; D005260:Female; D005724:Ganglia / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D009362:Neoplasm Metastasis; D009419:Nerve Tissue Proteins / Q000378:metabolism; D010751:Phosphopyruvate Hydratase / Q000378:metabolism; D016133:Polymerase Chain Reaction; D011859:Radiography; D018714:Radiotherapy, Adjuvant; D011884:Radius; D012512:Sarcoma, Ewing / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology*
D000071443:12E7 Antigen; D015703:Antigens, CD; D018959:CD57 Antigens; C061828:CD99 protein, human; D015815:Cell Adhesion Molecules; D017931:DNA Primers; D009419:Nerve Tissue Proteins; D010751:Phosphopyruvate Hydratase