Primary pulmonary primitive neuroectodermal tumor (PNET). A case report.
We describe a rare case of a primary primitive neuroectodermal tumor (PNET) in the lung of a 17-year-old girl. Grossly, the tumor, located in the right lower lobe, was relatively well-circumscribed and whitish to yellowish in color with scattered hemorrhagic necrosis. Microscopically, the tumor was composed of ovoid to polygonal cells with a high nuclear to cytoplasmic ratio and relatively scant cytoplasm, arranged in solid sheets with intervening fine fibrovascular stroma. Immunohistochemically, the tumor was positive for the MIC2 gene product, whereas AE1/AE3, CAM5.2, and a variety of neuroendocrine markers such as chromogranin A, synaptophysin, and ProGRP, were negative. Three months after the lobectomy, recurrent tumors were noted in the mediastinum and right thoracic wall, and she died despite combined chemotherapy and radiation therapy. In this case cytogenetic analysis showed a hypertriploid karyotype with multiple numerical and structural chromosomal aberrations, but failed to disclose distinct evidence of translocation between chromosome 11 and 22. However, the reverse transcriptase-polymerase chain reaction (RT-PCR) demonstrated EWS/FLI-1 fusion transcripts, confirming the histopathologic diagnosis of PNET. This case indicates that the primary pulmonary PNET is a highly aggressive neoplasm occurring at a young age, and should prompt combined systemic chemotherapy, even though it is organ-confined.
Department of Pathology, Kawasaki Medical School Hospital, Kurashiki, Okayama,. mika@med.kawasaki-m.ac.jp
Y
Y
Mikami
M
M
Nakajima
H
H
Hashimoto
I
I
Irei
T
T
Matsushima
S
S
Kawabata
T
T
Manabe
D000293:Adolescent; D018288:Carcinoma, Small Cell / Q000175:diagnosis; D003131:Combined Modality Therapy; D017931:DNA Primers / Q000737:chemistry; D004273:DNA, Neoplasm / Q000032:analysis; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007621:Karyotyping; D008175:Lung Neoplasms / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000235:genetics / Q000473:pathology* / Q000601:surgery / Q000628:therapy; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS; D013902:Radiography, Thoracic; D020133:Reverse Transcriptase Polymerase Chain Reaction; D014057:Tomography, X-Ray Computed; D014157:Transcription Factors / Q000235:genetics; D014407:Tumor Cells, Cultured
D017931:DNA Primers; D004273:DNA, Neoplasm; C106031:EWS-FLI fusion protein; D015514:Oncogene Proteins, Fusion; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS; D014157:Transcription Factors