Epstein Barr Virus-Associated Lymphoepithelial Carcinoma in
Middle Ear
1Leh-Kiong Huon MD, 1,2,3Pa-Chun Wang MD, MSc, 4Shih-Hung Huang MD
Affiliations/ Institution
1.
Department of Otolaryngology, Head and Neck Surgery, Cathay General Hospital, Taipei, Taiwan
2.
School of Medicine, Fu Jen Catholic University, Taipei, Taiwan.
3.
School of Public Health, China Medical University, Taichung, Taiwan.
4.
Department of Pathology, Cathay General Hospital, Taipei, Taiwan
Financial support: none
Word count:631
Correspondence: Shih-Hung Huang MD
Department of Pathology, Cathay General Hospital,
280 Sec.4 Jen-Ai Rd, 106 Taipei, Taiwan Tel: 8862-27082121 ext 3221
Fax: 8862-27074949
CASE REPORT
Histo-pathologically, lymphoepithelial carcinoma (LEC) is characterized by
individual, sheets, or nests of poorly to un-differentiated epithelial cells. The
malignant epithelial cells are surrounded and infiltrated by prominent components of
small mature lymphocytes and plasma cells. The most frequent site of LEC
occurrence is nasophayrnx (nasopharyngeal carcinoma, NPC), where it is almost
invariably associated with Epstein-Barr virus (EBV) infection. NPC is an endemic
disease prevalent in south-eastern coastal provinces of China. Isolated
non-nasopharyngeal LEC occurrence is rare, and its association with EBV remains
controversial.
Primary temporal bone LEC is a rare disease entity. 1 To the best of our
knowledge, only 4 patients have been reported in the literatures to date, all with
confirmed EBV infection. 2 In this report we present the diagnosis and management of
a rare LEC case that originated from middle ear. Its association with EBV is also
addressed.
presented to our clinic with aural fullness and hearing loss on her right ear for 1 year.
Otoscopic examination revealed a lobulated mass extending out from middle ear
space (Fig 1). There was no history of otorrhea. Her brother had had cured NPC for
several years. The pure-tone audiometry (PTA 45 dB) revealed a mixed type hearing
loss with 30 dB air-bone gap on the right side. The left side PTA was 18dB, showing a
high-tone sensori-neural loss with no conductive component. High resolution
temporal bone computerized tomography showed a middle ear mass with no bone or
ossicle erosion.
Exploratory tympanotomy revealed that the multi-lobulated lesion was originated
from promontory wall, filling whole meso-tympanum and partially extending into
Eustachian tube. The tumor was removed piece-by-piece without disrupting ossicular
chain. Patient received adjuvant radiotherapy (62 Gy, 31 fractions) over a period of 7
weeks.
Histo-pathology examination demonstrated irregular sheets of undifferentiated
carcinoma intermingled with abundant lymphoid infiltrate (Fig 2).
Immuno-histochemical staining was positive for keratin and negative for leukocyte
EBV encoded RNA was strongly positive. However, serum IgA antibodies against
EBV capsid antigen (EBV VCA IgA) was negative. Nasopharynx biopsy reveal no
evidence of LEC. Magnetic resonance imaging (MRI) excluded extra-temporal
invasion and distant metastasis.
DISCUSSION
Tumor occurrences at temporal bone region are not commonly seen; most of
them are squamous cell carcinoma. Primary LEC of middle ear is a rare entity.
Non-nasopharyngeal LEC has been previously reported under various names,
including undifferentiated carcinoma of nasopharyngeal type, undifferentiated
carcinoma with lymphoid stroma, lymphoepithelioma, and lymphoepithelioma-like
carcinoma. The profound infiltration of lymphoid cells in stroma makes it difficult to
indentify the malignant epithelial cells with routine hematoxylin and eosin-stain.
The association between EBV and nasopharyngeal LEC has been well
established2. Serum EBV titer check is a routine NPC screening test in the endemic
area of China. However, the role of EBV in the pathogenesis of non-nasopharyngeal
demonstrate EBV infection in the present case using highly sensitive PCR
amplification technique. The presence of EBV encoded RNA in the middle ear
suggests that EBV infection may indeed play some role in the development of middle
ear LEC.
Owing to the paucity of middle ear LEC, the optimal therapy regimen remains
unknown. Non-nasopharyngeal LEC of head and neck is radiosensitive with high
regional control rates. 3 From the published data, surgery and post-operative
radiotherapy is considered the appropriate protocol for middle ear LEC.
In this report we present the clinico-pathologic manifestations of middle ear LEC
and its association with EBV. Further study is warranted to establish the appropriate
radiation field and dose for the treatment of middle ear LEC.
The preparation of this report is in compliance with the Institution Review Board
AUTHOR INFORAMTION
From the Department of Otolaryngology, Head and Neck Surgery, Cathay
General Hospital, Taipei, Taiwan (Drs. Houn, Wang); School of Medicine, Fu Jen
Catholic University, Taipei County, Taiwan (Dr.Wang); School of Public Health,
China Medical University, Taichung, Taiwan (Dr.Wang); and Department of
Pathology, Cathay General Hospital, Taipei, Taiwan (Dr.Huang)
Corresponding author: Shih-Hung Huang, Department of Pathology, Cathay General
Hospital. 280 Sec.4 Jen-Ai Rd, 106 Taipei, Taiwan.
E-mail: drshhuang@gmail.com
AUTHOR CONTRIBUTION
Leh-Kiong Huon: principle manuscript drafting, patient care.
Pa-Chun Wang: patient care, manuscript editing and finalization
Shih-Hung Huang: histo-pathological confirmation of diagnosis
FINANCIAL DISCLOSURE
None
REFERENCES
1.Devaney KO, Boschman CR, Willard SC, et al. Tumours of the external ear and
temporal bone. Lancet Oncol 2005;6:411-20
2.Clark MP, Westerberg BD, Berean KW. Primary middle ear epstein-barr
virus-related lymphoepithelial carcinoma: casereports and systematic review.
Laryngoscope 2010;120:172-7
3.Dubey P, Ha CS, Ang KK, et al. Nonnasopharyngeal lymphoepithelioma of the head
and neck. Cancer 1998;82:1556-62
FIGURE LEGEND
Fig 1. Otoscopic examination revealed a lobulated mass extending from middle ear
space
Fig 2. Hematoxylin and eosin stain demonstrated irregular sheets of undifferentiated
FIGURES
