Original Article
Pulmonary extranodal NK/T-cell lymphoma, nasal type: a clinicopathological analysis of eleven cases
Xianzheng Gao, Jianguo Wei, Shenglei Li, Guannan Wang, Jing Han, Wugan Zhao, Yilin Xie, Wencai Li
Published 2019-06-08
Cite as Chin J Pathol, 2019, 48(6): 433-437. DOI: 10.3760/cma.j.issn.0529-5807.2019.06.002
Abstract
ObjectiveTo investigate the clinicopathological features, diagnosis and differential diagnosis of pulmonary extranodal NK/T-cell lymphoma, nasal type (ENKTL-NT).
MethodsEleven cases of newly diagnosed (10 puncture biopsies and 1 transbronchial biopsy), previously untreated pulmonary ENKTL-NT were collected at the First Affiliated Hospital of Zhengzhou University, from August 2013 to November 2018. The clinicopathological features including histomorphology, immunohistochemistry and in situ hybridization were collected and analyzed.
ResultsAmong the 11 cases, 8 were males and 3 were females, with a male to female ratio of 8∶3.The age range was from 30 to 74 years, with an average of 48 years and a median of 43 years. Tumors involved bilateral lung lobes in 8 cases, the upper left lobe in 1 case, lower left lobe in 1 case, and upper right lobe in 1 case. Main clinical symptoms included fever, often accompanied by cough, and bloody sputum in most cases. All cases were stage Ⅳ E. Histological features included scattered or focal aggregates of marked pleomorphic tumor lymphocytes, accompanied by necrosis and heavy admixture of inflammatory cells. In a few cases, diffuse neoplastic lymphocytes or vascular central and destructive infiltrations were seen. Tumor cells in most cases expressed CD3ε, CD3, CD43, CD56, TIA-1, granzyme B, but did not express CD20, CD79a, and CD5. Ki-67 index ranged from 40%to 90%.All cases were positive for EBER by in situ hybridization. Four of five patients died during follow-up with a survival period of only 1 week to 13 months.
ConclusionsPulmonary ENKTL-NT is rare, high grade malignancy with a poor prognosis. Misdiagnosis is common due to lesional necrosis and heterogeneous cell components. Immunohistochemistry and EBER in situ hybridization are essential for accurate diagnosis.
Key words:
Lung neoplasms; Lymphoma, extranodal NK-T-cell; Immunohistochemistry
Contributor Information
Xianzheng Gao
Department of Pathology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China
Jianguo Wei
Department of Pathology, Shaoxing People′s Hospital of Zhejiang Province, Shaoxing 312000, China
Shenglei Li
Department of Pathology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China
Guannan Wang
Department of Pathology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China
Jing Han
Department of Pathology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China
Wugan Zhao
Department of Pathology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China
Yilin Xie
Department of Pathology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China
Wencai Li
Department of Pathology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China