Primary primitive neuroectodermal tumors (PNETs) are extremely rare in the lung and especially in adult women. We describe a case of PNET of the lung with aggressive behavior in 31-year-old woman. Diagnosis was based on histopathological and immunohistochemical studies, and confirmed by molecular genetic analysis of chromosome rearrangements in the EWSR1 gene region. Clinical follow-up, post-mortem findings, and differential diagnosis are also discussed.

Department of Clinical and Molecular Pathology and Institute of Molecular and Translational Medicine Faculty of Medicine and Dentistry Palacky University Hněvotínská 3 775 15 Olomouc Czech Republic

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Weiss SW, Goldblum JR. Primitive neuroectodermal tumors and related lesions. In: Weiss SW, Goldblum JR, editors. Soft Tissue Tumors. 5. Maryland Heights: Mosby Elsevier; 2008. pp. 945–987.

Tsuji S, Hisaoka M, Morimitsu Y, Hashimoto H, Jimi A, Watanabe J, Eguchi H, Kaneko Y. Peripheral primitive neuroectodermal tumour of the lung: report of two cases. Histopathology. 1998;33:369–374. doi: 10.1046/j.1365-2559.1998.00485.x. PubMed DOI

Imamura F, Funakoshi T, Nakamura S, Mano M, Kodama K, Horai T. Primary primitive neuroectodermal tumor of the lung: report of two cases. Lung Cancer. 2000;27:55–604. doi: 10.1016/S0169-5002(99)00078-1. PubMed DOI

Baumgartner FJ, Omari BO, French SW. Primitive neuroectodermal tumor of the pulmonary hilum in an adult. Ann Thorac Surg. 2001;72:285–287. doi: 10.1016/S0003-4975(00)02535-2. PubMed DOI

Kahn AG, Avagnina A, Nazar J, Elsner B. Primitive neuroectodermal tumor of the lung. Arch Pathol Lab Med. 2001;125:397–399. PubMed

Mikami Y, Nakajima M, Hashimoto H, Irei I, Matsushima T, Kawabata S, Manabe T. Primary pulmonary primitive neuroectodermal tumor (PNET). A case report. Pathol Res Pract. 2001;197:113–119. PubMed

Takahashi D, Nagayama J, Nagatoshi Y, Inagaki J, Nishiyama K, Yokoyama R, Moriyasu Y, Okada K, Okamura J. Primary Ewing’s sarcoma family tumors of the lung: a case report and review of the literature. Jpn J Clin Oncol. 2007;l37:874–877. doi: 10.1093/jjco/hym108. PubMed DOI

Demir A, Dagoglu N, Gunluoglu MZ, Turna A, Dizdar Y, Kaynak K, Dilege S, Mandel NM, Yilmazbayhan D, Dincer SI, Gurses A. Surgical treatment and prognosis of primitive neuroectodermal tumors of the thorax. J Thorac Oncol. 2009;l4:185–192. doi: 10.1097/JTO.0b013e318194fafe. PubMed DOI

Hancorn K, Sharma A, Shackcloth M. Primary extraskeletal Ewing’s sarcoma of the lung. Interact Cardiovasc Thorac Surg. 2010;10:803–804. doi: 10.1510/icvts.2009.216952. PubMed DOI

Weissferdt A, Moran CA. Primary pulmonary primitive neuroectodermal tumor (PNET): a clinicopathological and immunohistochemical study of six cases. Lung. 2012;190(6):677–683. doi: 10.1007/s00408-012-9405-9. PubMed DOI

Tongaonkar HB, Thyavihally YB, Gupta S, Kurkure PA, Amare P, Muckaden MA, Desai SB. Primitive neuroectodermal tumor of the kidney: a single institute series of 16 patients. Urology. 2008;71:292–296. doi: 10.1016/j.urology.2007.09.051. PubMed DOI

Charney DA, Ghali VS, Charney JM, Teplitz C. Primitive neuroectodermal tumor of the myocardium: a case report, review of the literature, immunohistochemical, and ultrastructural study. Hum Pathol. 1996;27:1365–1369. doi: 10.1016/S0046-8177(96)90352-4. PubMed DOI

Askin FB, Rosai J, Sibley RK, Dehner LP, McAlister WH. Malignant small cell tumor of the thoracopulmonary region in childhood: a distinctive clinicopathologic entity of uncertain histogenesis. Cancer. 1979;43:2438–2451. doi: 10.1002/1097-0142(197906)43:6<2438::AID-CNCR2820430640>3.0.CO;2-9. PubMed DOI

Lucas DR, Bentley G, Dan ME, Tabaczka P, Janet M, Poulik JM, Michael MP. Ewing sarcoma vs lymphoblastic lymphoma a comparative immunohistochemical study. Am J Clin Pathol. 2001;115:11–17. doi: 10.1309/K1XJ-6CXR-BQQU-V255. PubMed DOI

Gamberi G, Cocchi S, Benini S, Magagnoli G, Morandi L, Kreshak J, Gambarotti M, Picci P, Zanella L, Alberghini M. Molecular diagnosis in Ewing family tumors. J Mol Diagn. 2011;13:3. doi: 10.1016/j.jmoldx.2011.01.004. PubMed DOI PMC