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Primary pulmonary primitive neuroectodermal tumor (PNET). A case report.
Pathol Res Pract. 2001; 197(2):113-119; discussion 121-2.PR

Abstract

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.

Authors+Show Affiliations

Department of Pathology, Kawasaki Medical School Hospital, Kurashiki, Okayama,. mika@med.kawasaki-m.ac.jpNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

11261815

Citation

Mikami, Y, et al. "Primary Pulmonary Primitive Neuroectodermal Tumor (PNET). a Case Report." Pathology, Research and Practice, vol. 197, no. 2, 2001, pp. 113-119; discussion 121-2.
Mikami Y, Nakajima M, Hashimoto H, et al. Primary pulmonary primitive neuroectodermal tumor (PNET). A case report. Pathol Res Pract. 2001;197(2):113-119; discussion 121-2.
Mikami, Y., Nakajima, M., Hashimoto, H., Irei, I., Matsushima, T., Kawabata, S., & Manabe, T. (2001). Primary pulmonary primitive neuroectodermal tumor (PNET). A case report. Pathology, Research and Practice, 197(2), 113-119; discussion 121-2.
Mikami Y, et al. Primary Pulmonary Primitive Neuroectodermal Tumor (PNET). a Case Report. Pathol Res Pract. 2001;197(2):113-119; discussion 121-2. PubMed PMID: 11261815.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Primary pulmonary primitive neuroectodermal tumor (PNET). A case report. AU - Mikami,Y, AU - Nakajima,M, AU - Hashimoto,H, AU - Irei,I, AU - Matsushima,T, AU - Kawabata,S, AU - Manabe,T, PY - 2001/3/23/pubmed PY - 2001/6/22/medline PY - 2001/3/23/entrez SP - 113-119; discussion 121-2 JF - Pathology, research and practice JO - Pathol Res Pract VL - 197 IS - 2 N2 - 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. SN - 0344-0338 UR - https://www.unboundmedicine.com/medline/citation/11261815/Primary_pulmonary_primitive_neuroectodermal_tumor__PNET___A_case_report_ L2 - https://medlineplus.gov/lungcancer.html DB - PRIME DP - Unbound Medicine ER -