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2012, Volume 28, Number 3, Page(s) 286-289
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DOI: 10.5146/tjpath.2012.01139 |
Primary Lymphoepithelioma-Like Carcinoma of the Lung: Report of a Rare Case and Review of the Literature |
Neşe Arzu YENER1, Ahmet BALIKÇI2, Rahmi ÇUBUK3, Ahmet MİDİ1, Alpay ÖRKİ4, Aynur EREN TOPKAYA2 |
1Department of Pathology, Maltepe University, Faculty of Medicine, İSTANBUL, TURKEY
2Department of Microbiology, Maltepe University, Faculty of Medicine, İSTANBUL, TURKEY
3Department of Radiology, Maltepe University, Faculty of Medicine, İSTANBUL, TURKEY
4Department of Chest Surgery, Maltepe University, Faculty of Medicine, İSTANBUL, TURKEY |
Keywords: Epstein-Barr virus, Carcinoma, Lymphoepitheliomalike, Lung cancer |
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Primary lymphoepithelioma-like carcinoma of the lung is a rare type
of non-small cell lung carcinoma. In this study, we aimed to present
a 62-year-old smoker male with a primary lymphoepithelioma-like
carcinoma of the hilar region of the left lung. The patient underwent
left pneumonectomy and no adjuvant therapy was given. There were
no other abnormalities on whole body PET/CT scan including the
nasopharyngeal region. The patient showed seropositivity for EBV
IgG but immunohistochemistry and PCR amplification studied
on paraffin-embedded tissue sections of the tumor failed to show
any sign of EBV infection within the tumor cells. He is alive and
disease-free four months after the operation. Although primary
lymphoepithelioma-like carcinoma of the lung is usually reported
in young females with no history of tobacco use and the tumor cells
are infected with EBV, it may rarely be seen in elderly males with a
history of tobacco use and the tumor cells not infected with EBV. |
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Primary lymphoepithelioma-like carcinoma (LELC) has
been described in many organs such as the stomach, thymus,
skin, liver, cervix, and urinary bladder. LELC originating
from the lung was first described by Begin et al in 1987 1.
It preferentially affects non-smoker Asian patients of young
age and is thought to have a better prognosis than the other
types of non-small cell lung carcinomas 2,3. Caucasian
patients are more rarely affected 2,4. In this study, we
aimed to present a rare case of primary LELC of the lung in an elderly smoker male and review the relevant literature
on this rare tumor. |
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Abstract
Introduction
Case Presentation
Disscussion
References
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A 62-year-old man with a 40 pack-year history of smoking
applied to our hospital with complaints of cough and
dyspnea for the last six months. He had a history of
undergoing coronary artery by-pass surgery three months
ago that had resulted in no relief of his symptoms. On chest
computerized tomography (CT) imaging, a hilar mass of 2 cm in size with lobulated margins located 1.5 cm distal to
the bifurcation of the trachea was observed (Figure 1A). At
retrospective evaluation of the baseline radiograph which
had been obtained soon before his coronary artery bypass
operation, the mass was seen as a nodule of less than 5 mm
in diameter.
Click Here to Zoom |
Figure 1: (A) CT scan
revealed a large mass in
the hilar region of the
left lung, (B) and the
head and neck region
was negative for any
nasopharyngeal lesions. |
Fiberoptic bronchoscopy revealed a polypoid tumoral
mass and the bronchus biopsy specimen was reported as
“non-small cell carcinoma”. The patient then underwent
whole body imaging including the nasopharyngeal region
(Figure 1B) but no other lesions or lymphadenomegaly was
identified throughout the body. The patient underwent left
pneumonectomy with nodal staging at the time. Frozen
sections revealed nonmetastatic mediastinal lymph nodes.
His postoperative course was uneventful.
On gross examination of the specimen, a relatively wellcircumscribed
solid mass located in the right main bronchus was observed. On microscopic examination,
the tumor consisted of uncircumscribed solid nests
with the typical syncytial growth pattern. The tumor
cells were undifferentiated epithelial cells with oval
and pleomorphic nuclei. They had vesicular chromatin
with distinct nucleoli. There were no apparent areas of
necrosis and the mitotic count was variable (8/10 HPF).
A significant lymphoplasmacytic infiltration within and
around the tumor cells was also observed (Figure 2A, B).
Immunohistochemically, tumor cells were positive for
pancytokeratin and negative for latent membrane protein
of EBV. The lymphocytes infiltrating the tumor cells were
almost exclusively positive for CD8, whereas the rest of
them were positive for CD20. EBV serology revealed
positivity for EBV IgG, which suggested a prior infection.
However, the tumor cells were negative for EBV DNA with
PCR amplification.
Click Here to Zoom |
Figure 2A, B: Irregular solid epithelial cell islands infiltrated by dense lymphoplasmacytic infiltration (H&E, x100). |
A final diagnosis of primary LELC of the lung was made
with these findings. He was not given any adjuvant therapy.
He is doing well and a standard chest radiograph revealed
no evidence of recurrent disease at the fourth month of
follow-up. |
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Abstract
Introduction
Case Presentation
Disscussion
References
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The commonest site of involvement for LELC is the
nasopharynx where it is almost invariably associated with
EBV infection. Primary LELC of the lung is a rare type of
non-small cell lung cancer and it comprises about 0.9%
of the other types of lung cancer 5. To recognize it as
having a pulmonary origin, careful examination of the
nasopharyngeal region should be done to exclude any
primary nasopharyngeal tumor 2,5-7. LELC is a distinct
type of non-small cell lung carcinoma in many aspects. It
commonly affects patients younger than those typically
affected by bronchogenic carcinoma 7. A female sex
predilection was notified in several reports 5,7,8 whereas
not in the others 2,6. It has no strong association with
smoking 2,5,8. EBV infection of the tumor is variable,
and still much stronger in Asians than in whites 2,5,6,8.
The patients usually present at an early stage of the
disease 2. Our case was a 62-year-old smoker nonAsian
(Turkish) man with the disease at stage I (T1N0M0) and
with serological evidences of a former EBV infection.
However, the percentage of seroprevalance for EBV is about
95% in adults irrespective of country 9. Although DNA
degradation in paraffin-embedded tissues may obscure
the results of PCR amplification results 10, there was
no evidence of EBV particles within the tumor cells in our
case and we still consider that an unknown carcinogenic
agent possibly related to tobacco use may play a role in the
formation of LELC of pulmonary origin.
Radiographic, CT and MRI features of the primary
pulmonary LELC at an early stage are reported as
nonspecific7. The characteristic CT findings in advanced
stage disease are the central location of the tumor with welldefined,
smooth borders and with variable dimensions11.
Peribronchovascular lymph node involvement and vascular
encasement were also considered to be specific CT features
for advanced stage LELC11. Our case was at an early
stage and CT findings were suggestive of any type of lung
malignancy without any LELC-specific feature.
Histopathologically, the tumor is identical to its
nasopharyngeal counterpart and it is characterized by two
patterns of tumor growth as well-defined epithelial nests
separated by broad areas of lymphocytic reaction and a
diffuse infiltration of malignant epithelial cells infiltrated with non neoplastic lymphocytes3. Lymphocytes
infiltrating the tumor cells may represent the enhanced
immunity, suggesting a better prognostic indicator12. In
our case, we observed these two patterns, mainly the welldefined
nests of malignant epithelial cells. The abundant
lymphocytes infiltrating the tumor cells forming these nests
were of the T cell phenotype.
The mainstay of the treatment for LELC of the lung is surgery2,3,5-7. Adjuvant chemotherapy or radiotherapy may be
added especially in patients in advanced stage disease13.
Similarly, palliative chemotherapy and/or radiotherapy
should be employed in patients with unresectable lesions14. Given the rarity of primary LELC of the lung, the
choice of chemotherapy treatment remains empirical14.
Based on the long term follow-up of the cases reported
in the literature, the patients with LELC of the lung at an
early stage seems to have a more favorable prognosis than
do patients with other types of non-small cell carcinoma of
similar stage3,6-8.
In conclusion, as a rare type of non-small cell lung cancer,
primary LELC of the lung should be kept in mind even in
nonendemic areas for EBV. Nasopharyngeal region as a
possible primary site of origin should be ruled out before
considering the tumor as primary lung origin. |
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Abstract
Introduction
Case Presentation
Discussion
References
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1) Begin LR, Eskandari J, Joncas J, Panasci L. Epstein-Barr virus related lymphoepithelioma-like carcinoma of the lung. J Surg Oncol 1987,36:280-283. [ PubMed ]
2) Chan JK, Hui PK, Tsang WY, Law CK, Ma CC, Yip TT, Poon YF.. Primary lymphoepithelioma-like carcinoma of the lung. A clinicopathologic study of 11 cases. Cancer 1995,76:413-422. [ PubMed ]
3) Han A, Xiong M, Gu Y, Lin S, Xiong M. Lymphoepithelioma-like carcinoma of the lung with a better prognosis. A clinicopathologic study of 32 cases. Am J Clin Pathol 2001,115:841-850. [ PubMed ]
4) Bildirici K, Ak G, Peker B, Metintas M, Alatas F, Erginel S, Ucgun I. Primary lymphoepithelioma-like carcinoma of the lung. Tuberk Toraks 2005, 53: 69-73. [ PubMed ]
5) Chang YL. Wu CT, Shih JY, Lee YC. New aspects in clinicopathologic and oncogene studies of 23 pulmonary lymphoepithelioma-like carcinomas. Am J Surg Pathol 2002,26:715-723. [ PubMed ]
6) Castro CY, Ostrowski ML, Barrios R, Green LK, Popper HH, Powell S, Cagle PT, Ro JY. Relationship between Epstein-Barr virus and lymphoepithelioma-like carcinoma of the lung: A clinicopathologic study of 6 cases and review of the literature. Hum Pathol 2001,32:863-872. [ PubMed ]
7) Hoxworth JM, Hanks DK, Araoz PA, Elicker BM, Reddy GP, Webb WR, Leung JW, Gotway MB. Lymphoepithelioma-like carcinoma of the lung: radiologic features of an uncommon primary pulmonary neoplasm. AJR 2006,186:1294-1299. [ PubMed ]
8) Yoshino N, Kubokura H, Yamauchi S, Ohaki Y, Koizumi K, Shimizu K.. Lymphoepithelioma-like carcinoma of the lung; case in which the patient has been followed up for 7 years postoperatively. Jpn J Thorac Cardiovasc Surg 2005,53:653-656. [ PubMed ]
9) Ozkan A, Kilic SS, Kalkan A, Ozden M, Demirdag K, Ozdarendeli A. Seropositivity of Epstein-Barr virus in Eastern Anatolian Region of Turkey. Asian Pac J Allergy Immunol 2003,21:49-53. [ PubMed ]
10) Charef S, Jrad BB, Mahfouth W, Zakhama A, Kassab A, Driss N, Chouchane L. Detection of EBV by PCR in fresh and paraffin embedded samples of cavum tumour. Arch Inst Pasteur Tunis 2006,83:41-47 (abstract). [ PubMed ]
11) Ooi GC, Ho JC, Khong PL, Wang MP, Lam WK, Tsang KW. Computed tomography characteristics of advanced primary pulmonary lymphoepithelioma-like carcinoma. Eur Radiol 2003,13:522-526. [ PubMed ]
12) Kobayashi M, Ito M, Sano K, Honda T, Nakayama J. Pulmonary lymphoepithelioma-like carcinoma: Predominant infiltration of tumor-associated cytotoxic T lymphocytes might represent the enhanced tumor immunity. Intern Med 2004,43:323-326. [ PubMed ]
13) Chan ATC, Teo PML, Lam KC, Chan WY, Chow JHS, Yim APC, Mok TSK, Kwan WH, Johnson PJ. Multimodality treatment of primary lymphoepithelioma-like carcinoma of the lung. Cancer 1998,83:925-929. [ PubMed ]
14) Ho JC, Lam WK, Wong MP, Wong MK, Ooi GC, Chan-Yeung M, Tsang KW. Lymphoepithelioma-like carcinoma of the lung: Experience with ten cases. Int J Tuberc Lung Dis 2004,8:890-895. [ PubMed ] |
Top
Abstract
Introduction
Case Presentation
Discussion
References
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