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Vol.46 No.1 contents | Japanese/English |
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- Case Report -
A Case of Primary Pulmonary Primitive Neuroectodermal Tumor (PNET)
Miyako Hiramatsu1,4, Sakae Okumura1, Fumiyoshi Ohyanagi2, Makoto Nishio2, Yuichi Ishikawa3, Ken Nakagawa11Thoracic Center, Surgical Oncology, 2Medical Oncology, Cancer Institute Ariake Hospital, Japan, 3Department of Pathology, Cancer Institute, Japan, 4Department of Surgery, Tokyo Jikei University, Japan
Background. Primitive neuroectodermal tumor (PNET) and Ewing's sarcoma are relatively aggressive malignant tumors of bone and soft tissue. Their typical chromosomal translocation and chimeric protein, as a result of gene fusion, have proved their similarity and now they are thought to be identical. Since the 1980, many protocol studies have been carried out and accumulated data have demonstrated that intensive chemotherapy combined with local control is crucial to improve survival. Since primary pulmonary PNET is very rare and there are only a few cases reported in detail, there is no standard therapy for this disease and the prognosis is also unknown. Case. A 31-year old Japanese man presenting a mass adjacent to the right hilum of lung on an annual chest X-ray check up was admitted to our hospital. CT-guided needle biopsy demonstrated small, round cell proliferation and both MIC2 and PAS staining were positive, resulting in a diagnosis of PNET. Chromosomal translocation (t(11; 22) (q24; q12)) together with fusion gene (EWS-FLI 1 Exon 7/Exon 5) confirmed the diagnosis. After 3 courses of chemotherapy, we performed right upper lobectomy and he recently finished an adjuvant chemotherapy course that extended over 47 weeks.
key words: Primitive neuroectodermal tumor, Ewing's sarcoma, Chromosomal translocation, Transfusion gene, Lung
Received: June 8, 2005
Accepted: October 31, 2005
JJLC 46 (1): 33-39, 2006
