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Year : 2013  |  Volume : 56  |  Issue : 4  |  Page : 479-480
Primary primitive neuroectodermal tumor of lung: A case report

1 Department of Pathology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India
2 Department of Cardiovascular and Thoracic Surgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India

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Date of Web Publication18-Jan-2014

How to cite this article:
Amita RN, Sandhyamani S, Balasubramoniam KR. Primary primitive neuroectodermal tumor of lung: A case report. Indian J Pathol Microbiol 2013;56:479-80

How to cite this URL:
Amita RN, Sandhyamani S, Balasubramoniam KR. Primary primitive neuroectodermal tumor of lung: A case report. Indian J Pathol Microbiol [serial online] 2013 [cited 2022 Jun 28];56:479-80. Available from: https://www.ijpmonline.org/text.asp?2013/56/4/479/125397


Ewing's sarcoma (ES) family of tumors are small round cell tumors showing varying degree of neuroectodermal differentiation, the most differentiated ones called as primitive neuroectodermal tumors (PNET). [1] It often arises in the bone and soft-tissues of young adults. There are rare reports of primary ES/PNETs of the kidney, ovary, palate, pancreas and myocardium. [2] Though lung involvement by metastasis is common, primary PNET of lung parenchyma without pleural or chest wall involvement is extremely rare and only very few cases have been reported in the literature. To the best of our knowledge, this is the second documented case of a primary PNET in the lung from India. [3]

A 21-year-old female patient presented with a history of dry cough and loss of appetite for 3 months. Computed tomography scan of the chest showed an enhancing soft-tissue mass with lobulated margins in the apical segment of the upper lobe, left lung [Figure 1]a . The lobectomy specimen showed a well-circumscribed, hemorrhagic mass measuring 3.5 cm × 4.5 cm × 3.5 cm [Figure 1]b, situated 1 cm from the bronchial resection margin.

Microscopy showed monomorphic small round cells arranged in sheets [Figure 2]a. Scattered ill-defined perivascular and true rosettes were seen [Figure 2]b. The cells had scant to moderate amount of clear cytoplasm and vesicular nuclei. Mitotic activity was low. The tumor cells showed cytoplasmic positivity with periodic acid Schiff stain [Figure 2]c and a strong membranous immunoreactivity for MIC-2 [Figure 3]a (using Dako Monoclonal Mouse Anti-Human CD99, clone 12E7 with identical reactivity as monoclonal antibodies HBA-71 and RFB-1). Cells were also positive for S100, vimentin and neuron specific enolase (NSE) [Figure 3[b, c and-d]. Other markers including cytokeratin, synaptophysin, thyroid transcription factor-1, leukocyte common antigen (LCA), CD 20 and desmin were negative.
Figure 1: (a) Computed tomography chest shows an enhancing lobulated soft ti ssue mass in the apical segment of the upper lobe, (b) lobectomy specimen shows a well circumscribed hemorrhagic mass

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Figure 2: (a) Neoplasm composed of monomorphic small round cells, (b) perivascular and true rosett es (a and b, H and E), (c) tumor cells showing periodic acid Schiff positi vity (a-c, ×400)

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Figure 3: (a) Tumor cells shows membrane positi vity for CD99, (b) S100 positive, (c) vimenti n positive, (d) neuron specifi c enolase focal positi ve (a-d, ×400)

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Work-up to exclude primary tumor elsewhere was negative. A final diagnosis of primary PNET, lung was given. Patient has been receiving post-operative radiotherapy and chemotherapy in the last 3 months and is doing well.

The first description of PNET (in ulnar nerve) was given by Stout in 1918 [4] and Ewing sarcoma of bone by James Ewing in 1921. [1] Both are histologically composed of small round cells. Cytogenetic studies have demonstrated a common genetic abnormality in these tumors, and hence are now categorized into a group known as the ES family of tumors.

The diagnosis is based on light microscopy showing a monomorphic small round cell tumor, positive for glycogen, immunohistochemistry showing positivity for NSE, S-100, and MIC-2, and negative for LCA, epithelial membrane antigen, cytokeratin, desmin and characteristic molecular profile. Among these, the detection of p30/32 cell surface antigen (also known as the MIC2 gene product, which can be detected by antibodies such as HBA71 and O13) and reciprocal translocation (11;22)(q24;q12) are believed to be characteristic of this family of tumors. [5]

The differential diagnosis should include other metastatic or primary small round cell tumors such as neuroblastoma, lymphoblastic lymphoma and rhabdomyosarcoma as treatment protocols vary.

Primary pulmonary ES/PNETs are extremely rare and metastasis from elsewhere has to be ruled out before rendering a diagnosis of primary ES/PNET.

   References Top

1.Hancorn K, Sharma A, Shackcloth M. Primary extraskeletal Ewing's sarcoma of the lung. Interact Cardiovasc Thorac Surg 2010;10:803-4.  Back to cited text no. 1
2.Suárez Antelo J, Rodríguez García C, Montero Martínez C, Verea Hernando H. Pulmonary Ewing sarcoma/primitive neuroectodermal tumor: A case report and a review of the literature. Arch Bronconeumol 2010;46:44-6.   Back to cited text no. 2
3.Gaude GS, Patil P, Malur P, Annurshetru S. Primitive neuro-ectodermal tumour of the lung. J Thorac Dis 2011;3.  Back to cited text no. 3
4.Stout AP. A tumor of the ulnar nerve. Proc NY Pathol Soc 1918;18:2-11.  Back to cited text no. 4
5.Lee YY, Kim do H, Lee JH, Choi JS, In KH, Oh YW, et al. Primary pulmonary Ewing's sarcoma/primitive neuroectodermal tumor in a 67-year-old man. J Korean Med Sci 2007;22 Suppl:S159-63.  Back to cited text no. 5

Correspondence Address:
Samvedam Sandhyamani
Department of Pathology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram - 695 011
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.125397

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  [Figure 1], [Figure 2], [Figure 3]

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