Lymphoepithelial carcinoma (LEC) is most

commonly seen in the nasopharynx. Very rarely it

is found in the salivary gland, preferably in parotid

gland followed by submandibular gland where it

accounts for 0.4% of all malignant salivary gland

tumours. Most commonly it is seen in fifth decade

with female predominance. Significant correlation

has been reported between this tumour and

the Epstein Barr virus (EBV).It has a racial

predilection for Inuits,Chinese and Japanese. Very

rarely it is found In Indians. So we present a case

of LEC of parotid gland in a 23 year old male

Indian patient. As this is a very radiosensitive

tumour, surgery followed by radiotherapy remains

the treatment of choice

Nitin M. Gangane

Sachin A. Badge

Satish M. Sharma

Vitaladevnni B. Shivkumar

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 Journal of Krishna Institute of Medical Sciences UniversityJKIMSU,    Vol. 3, No. 2, July-Dec 2014CASE REPORTISSN 2231-4261Ó ÓAbstract: Lymphoepithelial  carcinoma  (LEC)  is  most commonly seen in the nasopharynx. Very rarely it is found in the salivary gland, preferably in parotid gland followed by submandibular gland where it accounts for 0.4% of all malignant salivary gland tumours. Most commonly it is seen in fifth decade with female predominance. Significant correla-tion has been reported between this tumour and the  Epstein  Barr  virus  (EBV).It  has  a  racial predilection for Inuits,Chinese and Japanese. Very rarely it is found In Indians. So we present a case of LEC of parotid gland in a 23 year old male Indian  patient. As  this  is  a  very  radiosensitive tumour, surgery followed by radiotherapy remains the treatment of choice.Keywords:  Carcinoma,  Lymphoepithelial Carcinoma, Parotid Gland, Salivary Gland.IntroductionLymphoepithelial carcinoma is a specific subtype of undifferentiated carcinoma with characteristic dense  lymphoid  stroma.  The  most  frequent location  is  the  nasopharynx.  Identical  tumours have been rarely described in the major salivary glands  where  they  account  for  approximately 0.4% of all malignant salivary gland tumours [1]. The parotid gland is affected in approximately 80% of the cases, followed by the submandibular gland and minor salivary glands [2]. Very rarely they are found in lungs, thymus, stomach, larynx, trachea and skin [3]. Majority of LECs are seen in   fifth  decade  with  female  predominance  [4].144Lymphoepithelial Carcinoma of Parotid Gland- A Case Report 1*  2  2  2 Sachin A. Badge , Nitin M. Gangane , Vitaladevnni B. Shivkumar , Satish M. Sharma1Department of Pathology, Late Shree Baliram Kashyap Memorial Government Medical College, 2 Jagdalpur - 494001 (Chhattisgarh) India,  Department of Pathology, Mahatma Gandhi Institute of Medical Sciences, Sevagram - 442102, (Maharashtra), India.  It shows a striking racial predilection for Inuits (Eskimo)  in  the  Arctic  regions  (Greenland, Canada,  Alaska),  South-eastern  Chinese,  and Japanese [2]. In Indians it is very rarely found. Significant correlation has been reported between this tumour and the Epstein Barr virus (EBV) [5]. Due to a high radiosensitivity for LEC, surgery combined with Radio Therapy (RT) is considered   as the first choice for treatment of this disease [6]. Very few cases have been reported in medical literature till now. We present a case of LEC of parotid gland in a 23 year old male Indian patient.Case Report:A 23 year old male patient presented with painful swelling of 4x3 cm in left preauricular region. The swelling was hard in consistency, tender, having irregular  borders  and  bosselated  surface.  His facial nerve functions were intact. All finding in general examination and laboratory investigations were  normal.  CT  scan  revealed  a  mass  in  left parotid gland. We had performed whole body CT Scan to rule out metastasis in other organs of the body and on that we didn't get any areas of metas-tasis. Patient was treated with removal of mass by a surgical procedure of superficial parotidectomy.  We received superficial parotidectomy specimen measuring  5x4x2.5  cm.  On  cut  section  a  gray white tumour measuring 4x3x2.5 cm was seen along  with  areas  of  hemorrhage  and  necrosis. Histopathological  examination  showed  salivary gland  with  tumour  composed  of  tumour  cells growing in nests and trabecular sheets intermin-gled with lymphoid stroma (Fig.1)Fig. 1: Tumour Cells Growing in Nests Inter-mingled with Lymphoid Stroma(H &E,10X)Neoplastic cells exhibited syncytial appearance with  indistinct  cell  border,  eosinophilic  cyto-plasm,  large  vesicular  nuclei  and  prominent nucleoli (Fig. 2).Fig.  2:  Neoplastic  Cells  Exhibited  Syncytial Appearance  with  Indistinct  Cell  Border, Eosinophilic  Cytoplasm,  Large  Vesicular Nuclei and Prominent Nucleoli (H&E, 40X)JKIMSU,    Vol. 3, No. 2, July-Dec 2014 Sachin A. Badge et. al. Journal of Krishna Institute of Medical Sciences University Ó Ó 145Few mitosis and areas of hemorrhage and necrosis were also seen. In immunohistochemistry epithe-lial cells and lymphocytes showed positivity for cytokeratin  and  leukocyte  common  antigen respectively. So the diagnosis of LEC of parotid gland was given. We didn't get the patient for follow  up  to  opine  about  the  outcome  of  the treatment.Discussion:According  to  the  classification  by  Ellis  and Auclair,  undifferentiated  carcinomas  of  the salivary glands can be subtyped further into small cell undifferentiated carcinoma, large cell undif-ferentiated  carcinoma,  and  LEC  [7].  LEC accounts for approximately 0.4% of malignant salivary gland tumours [1]. This tumour affects the parotid gland in approximately 80% of the cases [2]. Most cases occur in the fifth decade of life with age range of first to ninth decade. It is most commonly  seen  in  females  [4]. There  are  few reports showing its occurrence in males as seen in our case [8]. Most of parotid LEC cases arises de novo, but they may rarely develop within lympho-epithelial sialadenitis [9]. The exact origin and pathogenesis of parotid LEC remain unknown. Among the possible etiologies are a malignant transformation of the glandular and ductal inclu-sions  in  the  intra-parotid  lymph  nodes  and  a malignant  transformation  of  the  epi-myoepithelial island [3, 10]. It seems there is a complex  interaction  between  genetic  factors, environmental factors, and EBV infection in the oncogenic process of LEC of the salivary glands [2]. EBV may be directly shed from lymphocytes into the salivary gland or may replicate in epithe-lial cells. The viral episome is maintained in the infected epithelial cell that continues to proliferateand  does  not  differentiate.  This  focus  of  latent infected, metaplastic epithelial cells rapidly become malignant  and  invade  the  basement  membrane. Parotid  LEC  usually  presents  as  an  enlarging parotid lump, occasionally painful and with facial nerve involvement in approximately 20% of cases [4]. Distant metastases usually involve the lung, liver, bone, and brain [1]. Macroscopically, these tumours are firm, 1–10 cm masses, multinodular, circumscribed, or clearly infiltrative into adjacent salivary gland, fat, muscle, or skin, with a cut surface that varies from a grey-tan to yellow-gray. Histologically, it is characterized by a syncitial growth pattern and a dense stroma made of non-neoplastic  lymphoplasmacytic  cells;  the  lym-phoid cells include a mixture of B and T cells and are sometimes associated with germinal centers. The epithelial component is composed of irregu-lar  shaped  islands,  cords,  trabeculae  of pleomorphic, large, malignant cells with abundant lightly  eosinophilic  cytoplasm  and  vesicular nuclei. Mitotic rate is variable. LEC is indistin-guishable from undifferentiated nasopharyngeal carcinoma which is much more common or other LECs that develop in various parts of the body [4].Therefore, to confirm the diagnosis of primary LEC  in  the  major  salivary  glands,  metastatic nasopharyngeal carcinoma to the salivary glands should be eliminated through examination of the upper aerodigestive tract with endoscopy, CT scan and even random biopsy of the nasopharynx [4]. Nasopharynx was absolutely normal on CT scan in our case. In reality, the parotid gland is the predominant site of occurrence of LEC and an exceptional site of JKIMSU,    Vol. 3, No. 2, July-Dec 2014 Journal of Krishna Institute of Medical Sciences University Ó Ó 146Sachin A. Badge et. al.metastasis  from  nasopharyngeal  carcinoma, which more typically metastasizes to the cervical or  sub-mandibular  lymph  nodes  [1].  Immuno-histochemistry shows neoplastic cells that stain positive for cytokeratin and epithelial membrane antigen [9]. Lymphoid cells are reactive for leuko-cyte common antigen, CD20 and CD3 markers. Histologically,  the  LEC  must  be  distinguished from benign lesions such as lymphoepithelial sia-ladenitis and from other malignancies, such as primary or metastatic poorly differentiated squa-mous  cell  carcinoma,  adenocarcinoma,  amela-notic melanoma and large cell or anaplastic types of  lymphoma  [7].  Lymphoepithelial  carcinoma seems to have a better prognosis than the other undifferentiated  carcinomas  of  the  salivary glands, in part because of the lymphoid stroma that has a role in limiting the aggressiveness of this carcinoma. Advanced  disease,  the  presence  of metastases on diagnosis, and histological features such as high mitotic rate, anaplasia, and necrosis are predictors of a worse prognosis [1, 2]. How-ever, the presence or absence of EBV in LEC does not appear to be of prognostic significance [2, 7]. Because  most  LECs  of  the  parotid  gland  are radiosensitive, the optimal management is com-plete  excision  with  clear  surgical  margins  fol-lowed by adjuvant radiotherapy to the tumour bed and neck. In conclusion, LEC of parotid gland is a very rare tumour to found in Indian population that requires surgical excision and postoperative radiotherapy.1. Schneider M, Rizzardi C. Lymphoepithelial carcinoma of the parotid glands and its relationship with benign lymphoepithelial lesions. Arch Pathol Lab Med 2008; 132(2):278-282.2. Sheen  TS,  Tsai  CC,  Ko  JY,  Chang YL,  Hsu  MM. Undifferentiated  carcinoma  of  the  major  salivary glands. Cancer 1997; 80(3):357-363.3. Kountakis  SE,  SooHoo  W,  Maillard  A. Lymphoepithelial  carcinoma  of  the  parotid  gland. Head Neck 1995; 17(5):445-450.4. Manganaris A, Patakiouta F, Xirou P, Manganaris T. Lymphoepithelial carcinoma of the parotid gland: is an association  with  Epstein-Barr  virus  possible  in nonendemic areas? Int J Oral Maxillofac Surg 2007, 36(6):556-559.5. Kuo  T,  Hsueh  C.  Lymphoepithelioma-like  salivary gland carcinoma in Taiwan: a clinicopathological study References:*Author for Correspondence: Dr. Sachin A. Badge, Department of Pathology, Late Shree Baliram Kashyap Memorial Government Medical College, Jagdalpur - 494001, (Chhattisgarh) India. Cell: 08962356271 Email: sachin.579@rediffmail.comJKIMSU,    Vol. 3, No. 2, July-Dec 2014 Journal of Krishna Institute of Medical Sciences University Ó Ó 147Sachin A. Badge et. al.of nine cases demonstrating a strong association with Epstein-Barr virus. Histopathology 1997, 31(1):75-82.6. Abdulla AK, Mian MY. Lymphoepithelial carcinoma of salivary glands. Head Neck 1996, 18(6):577-581.7. Ellis GL, Auclair PL. Atlas of tumor pathology: tumors of the salivary glands. Washington, DC: Armed Forces Institute of Pathology1996: 311-318.8. Borg MF, Benjamin CS, Morton RP, Llewellyn HR. Malignant  lymphoepithelial  lesion  of  the  salivary gland:  a  case  report  and  review  of  the  literature. Australas Radiol 1993; 37(3):288-291.9. Gravanis MB, Giansanti JS. Malignant histopathologic counterpart of benign lymphoepithelial lesion. Cancer 1970; 26(6):1332-1342.10. Batsakis  JG,  Bemacki  EG,  Rice  DH,  Stebler  ME. Malignancy and the benign lymphoepithelial lesion. Laryngoscope 1975, 85(2):389-399.

Lymphoepithelial Carcinoma of Parotid Gland- A Case Report

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