Cover Image

Primary Pulmonary Primitive Neuroectodermal Tumor of Lung in a Child

Amitabh Singh, Anirban Mandal, Shruthi Mohan, Rachna Seth

Abstract


Introduction: Peripheral primitive neuroectodermal tumor (pPNET) arising from lung without involvement of chest wall is very rare. Peripheral primitive neuroectodermal tumor are poorly differentiated, highly aggressive tumor of undetermined histogenesis with a tendency toward early metastasis. Diagnosis needs to be established conclusively by histopathology, immunohistochemistry and molecular studies.

Presentation of the cases: A 2 year 10-month old child was investigated for fever, cough and cold and was detected to have right intrathoracic cystic mass on imaging. The mass was excised with possibility of pleuropulmonary blastoma. After excision child was referred to our center for decision on further management. Histopathological examination, immunohistochemistry and molecular study for EWSR gene rearrangement proved the diagnosis of Ewing sarcoma. Metastatic work up for our case was negative. Child is currently undergoing chemotherapy and is asymptomatic.

Conclusion: Primitive neuroectodermal tumor (PNET) of lung without chest wall involvement in pediatric age group is a rare occurrence. Available literature suggests highly aggressive nature of such tumor. This case report summarizes the differential diagnosis of such rare pulmonary neoplasm in children and role of immunohistochemistry and molecular studies to establish the diagnosis conclusively.


Keywords


PNET; molecularstudies;EWSR gene rearrangement;Pediatric PNET lung

References


Christopher DM, Fletcher K, Krishnan U, Fredrik M, World Health Organization classification of tumors: Pathology and genetics of tumors of soft tissue and bone. 2nd edition. Lyon: IARC Press. 2002:297-300

Ewing J. Diffuse endothelioma of bone. Proc N Y Pathol Soc. 1921, 21:17-24

Marley EF, Liapis H, Humphrey PA, et al. Primitive neuroectodermal tumor of the kidney-another enigma: a pathologic, immunohistochemical and molecular diagnostic study. Am J Surg P. 1997, 21:354-359

Kanbour-Shakir A, Sawaday J, Kanbour A, Kunschner A, Stock RJ. Primitive neuroectodermal tumor arising in an ovarian mature cystic teratoma: immuno- histochemical and electron microscopic studies. Int J Gynecol P athol. 1993, 12: 270-275

Banerjee SS, Eyden BP, McVey RJ, Bryden AA, Clarke NW. Primary peripheral primitive neuroectodermal tumor of the urinary bladder. Histopathology. 1997, 30:486-490

Charney DA, Charney JM, Ghali VS, Teplitz C. Primitive neuroectodermal tumor of the myocardium: a case report, review of the literature, immunohisto-chemical, and ultrastructural study. Hum Pathol. 1996, 27:1365-1369

Danner DB, Hruban RH, Pitt HA, Hayashi R, Griffin CA, Perlman EJ. Primitive neuroectodermal tumor arising in the pancreas. Mod Pathol. 1994, 7:200-204

Bernstein M, Kovar H, Paulussen M, et al. Ewing’s sarcoma family of tumors: current management. Oncologist. 2006, 11:503-519

Catalan RL, Murphy T. Primary primitive neuroectodermal tumor of the lung. Am J Roentgenol. 1997, 169:1201-1202

Tsuji S, Hisaoka M, Morimitsu Y, et al. Peripheral primitive neuroectodermal tumour of the lung: Report of two cases. Histopathology. 1998, 33:369-374

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, 27:55-50

Baumgartner FJ, Omari BO, French SW. Primitive neuroectodermal tumor of the pulmonary hilum in an adult. Ann Thorac Surg. 2001, 72:285-287

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

Mikami Y, Nakajima M, Hashimoto H, et al. Primary pulmonary primitive neuroectodermal tumor (PNET). A case report. Pathology Research and Practice. 2001, 197:113-119

Paik SH, Park JS, Koh ES, et al. Primary pulmonary primitive neuroectodermal tumor: CT and skeletal scintigraphic image features with pathologic correlation (2006: 6b). Eur Radiol. 2006, 16:2128-2131

Takahashi D, Nagayama J, Nagatoshi Y, et al. Primary Ewing's sarcoma family tumors of the lung a case report and review of the literature. Jpn J Clin Oncol. 2007, 37:874-877

Lee YY, Kim do H, Lee JH, et al. Primary pulmonary Ewing’s sarcoma/primitive neuroectodermal tumor in a 67-year-old man. J Korean Med Sci. 2007, 22 Suppl: S159-S163

Gaude GS, Patil P, Malur P, Annurshetru S. Primitive neuro-ectodermal tumor of the lung in an adult. Lung India. 2009, 26:89-91

Demir A, Gunluoglu MZ, Dagoglu N, et al. Surgical treatment and prognosis of primitive neuroectodermal tumors of the thorax. J Thorac Oncol. 2009, 4:185-192

Hancorn K, Sharma A, Shackcloth M. Primary extraskeletal Ewing's sarcoma of the lung. Interactive Cardiovascular and Thoracic Surgery. 2010, 10:803-804

Suárez Antelo J, Rodríguez García C, Montero Martínez C, Verea Hernando H. Pulmonary ewing sarcoma/Primitive neuroectodermal tumour: A case report and a review of the literature. Archivos De Bronconeumologia. 2010, 46:44-46

Siddiqui MA, Akhtar J, Shameem M, Baneen U, Zaheer S, Shahid M. Giant extraosseous Ewing sarcoma of the lung in a young adolescent female--a case report. Acta Orthop Belg. 2011, 77:270-273

Ichiki Y, Nagashima A, Chikaishi Y, Yasuda M, Yamamoto I, Toyoshima S. Primary pulmonary Ewing's sarcoma: report of a case. Surg Today. 2012, 42:812-815

Tane S, Nishio W, Hashimoto S, et al. Ewing’s sarcoma family of tumors originating in the main bronchus. Thoracic Cancer. 2012, 3:353-356

Weissferdt A, Moran CA. Primary pulmonary primitive neuroectodermal tumor (PNET): a clinicopathological and immunohistochemical study of six cases. Lung. 2012, 190:677-683

Shet N, Stanescu L, Deutsch G. Primary extraosseous Ewing sarcoma of the lung: Case report and literature review. Radiology Case Reports(Online). 2013, 8:832

Andrei M, Cramer SF, Kramer ZB, Zeidan A, Faltas B. Adult primary pulmonary primitive neuroectodermal tumor: molecular features and translational opportunities. Cancer Biol Ther. 2013, 14:75-80

Deokar KK, Kunjir NG2, Ghorpade S3. Primary ewings sarcoma of the lung. J Clin Diagn Res. 2015, 9:XD01-XD03

Kasturi S, Roda SG, Rendla SB, Reddy Peddareddy N, Raju PS. Ewing’s Sarcoma/Primitive Neuroectodermal Tumor (ES/PNET) in the lung- A Rare Case Report. Global Journal of Research and Review. 2015, 2:58-63

McCarville MB. Malignant pulmonary and mediastinal tumors in children: differential diagnoses. Cancer Imaging. 2010,10:S35-S41

Douglass EC. Chromosomal rearrangements in Ewing’s sarcoma and peripheral neuroectodermal tumor (PNET). Sem Devel Biol. 1990, 1:393-396

Lee CS, Southey MC, Waters K, et al. EWS/FLI-1 fusion transcript detection and MIC2 immunohistochemical staining in the diagnosis of Ewing's sarcoma. Pediatr Pathol Lab Med. 1996, 16:379-392

Kumar S, Pack S, Kumar D, et al. Detection of EWS-FLI-1 fusion in Ewing's sarcoma/peripheral primitive neuroectodermal tumor by fluorescence in situ hybridization using formalin-fixed paraffin-embedded tissue. Hum Pathol. 1999, 30:324-330

Applebaum MA, Worch J, Goldsby R, et al. Clinical features and outcomes in patients with extraskeletal Ewing sarcoma. Cancer. 2011, 117:3027-3032


Full Text: PDF

Refbacks

  • There are currently no refbacks.


AJCC-REP (ISSN 2572-5742)Copyright © 2012-2019. All rights reserved. Published by Ivy Union Publishing, 3204 Valley Rush Dr, Apex, North Carolina 27502, United States