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Lymphoepithelial carcinoma of the nasolacrimal duct
Otolaryngology–Head and Neck Surgery (2010) 142, 144-145
CASE REPORT
Lymphoepithelial carcinoma of the nasolacrimal duct Yuan-Yun Tam, MD, Li-Yu Lee, MD, and Kai-Ping Chang, MD, PhD, Taoyuan, Taiwan No sponsorships or competing interests have been disclosed for this article.
L
ymphoepithelial carcinoma (LEC), an uncommon lesion in the head and neck, consists of undifferentiated carcinoma cells within the lymphoid stroma. This malignancy has been found in the major salivary glands,1-3 but it has also been sporadically reported in the larynx and soft palate.1 Some studies suggested that LEC of the salivary gland and Epstein-Barr virus (EBV) are strongly correlated. To the best of our knowledge, this neoplasm derived from the nasolacrimal duct has never been addressed in any English literature. We present a rare case of a LEC originating from the nasolacrimal duct and address its differential diagnosis, management, and association with EBV. Institutional Review Board approval was obtained. A 61-year-old woman visited our clinic with symptoms of right progressive nasal obstruction, rhinorrhea, intermittent epistaxis, and nasal stuffiness for six months. She also complained of frequent right epiphora and a slowly enlarging mass below the right medial canthus, which was diagnosed as dacryocystocele by an ophthalmologist. Nasal endoscopy revealed an exophytic tumor located inferiorly and laterally to the inferior turbinate. Transnasal biopsy of the lesion revealed an LEC. MRI of the head and neck disclosed a lesion that emerged from the right nasolacrimal duct (Fig 1) with extension to the inferior meatus. Physical examination and systemic work-up for metastasis showed negative results. We performed an en-bloc resection with medial maxillectomy through right lateral rhinotomy. For better locoregional control, the patient underwent adjuvant radiotherapy with 64 Gy in 32 fractions over seven weeks. At the 33-month follow-up (the time of writing), there were no signs of locoregional or distant tumor relapse. Grossly, the tumor was polypoid, whitish, and firm, and measured 2.3 ⫻ 1.3 ⫻ 1.3 cm, with invasion of the maxillary bone. Histopathologic examination of the tumor revealed a circumscribed mass containing aggregates of malignant epithelial cells bathed in a background of dense lymphocytic infiltrates and fibrous stroma (Fig 2). Low magnification revealed indistinct epithelial components and distinct heavy in-
filtration of lymphoid cells. At higher magnification, nests of carcinoma cells consisting of scanty cytoplasm with ill-defined cell borders, large nuclei, and prominent nucleoli appeared. Both in situ hybridization to identify small EBV-encoded RNA (EBER) and polymerase chain reaction (PCR) amplification for the EBV latent membrane protein-1 (LMP-1) oncogene were performed with positive results.4 Serum IgA antibodies against the EBV capsid antigen (EBV VCA IgA) and EBV early antigen and nuclear antigen-1 (EBV EA⫹ NA1 IgA) were also detected at 1:40 and 45.523 EU/mL, respectively.
Discussion Tumors derived from the lacrimal drainage system are very rare. Only a few tumors, such as lymphoma, Kaposi’s sarcoma, adenocarcinoma, angiofibroma, and squamous cell
Figure 1 Coronal view of T2-weighted MRI revealed a mass occupying the right nasolacrimal duct (arrow).
Received January 22, 2009; revised March 31, 2009; accepted May 29, 2009.
0194-5998/$36.00 © 2010 American Academy of Otolaryngology–Head and Neck Surgery Foundation. All rights reserved. doi:10.1016/j.otohns.2009.05.035
Tam et al
Lymphoepithelial carcinoma of the nasolacrimal duct
145
followed by radiotherapy.3 Moreover, in previous reports of tumors of the nasolacrimal duct, an en-bloc excision was usually applied.5 Therefore, en-bloc resection along with adjuvant radiotherapy was recommended in this case. We present the clinicopathologic manifestations, the association with EBV, and the management of this rare case to remind clinicians that vigilance and proper diagnosis, recognition of its distinct nature from other neoplasms in the lacrimal drainage system, and appropriate treatments are the cornerstones of management for this aggressive malignancy.
Author Information Figure 2 Hematoxylin and eosin stain showed the typical histopathologic features of lymphoepithelial carcinoma, characterized by islands of undifferentiated large carcinoma cells permeated and enveloped by an admixture of lymphocytes.
From the Departments of Otolaryngology–Head and Neck Surgery (Drs Tam and Chang) and Pathology (Dr Lee), Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Taoyuan, Taiwan. Corresponding author: Dr Kai-Ping Chang, MD, PhD, Department of Otolaryngology–Head and Neck Surgery, Chang Gung Memorial Hospital and Chang Gung University College of Medicine, No. 5 Fu-Shin Street, Kwei-Shan, Taoyuan, Taiwan, 33333. E-mail address: [email protected].
carcinoma have ever been reported in this particular location. Most of these patients presented with the symptoms of epiphora, blood-tinged tears, and epistaxis.5 In our case, right epiphora and dacryocystocele also suggested that the lacrimal drainage system was obstructed and that neoplastic diseases should be taken into consideration in the differential diagnosis. LEC has a unique racial prevalence in Inuits and Asians, and an association has been suggested between LEC and EBV infection.1,2 As in nasopharyngeal carcinoma, which is frequently diagnosed in southeastern Asians, most of the EBV-associated tumors including LEC often share similar characteristic histologic patterns, such as syncytial sheets of undifferentiated malignant cells with a lymphoid infiltrate background.2 In the present case, both in situ hybridization for the presence of EBER and PCR amplification for the LMP-1 gene showed positive results. These findings might collectively suggest the possibility of a carcinogenic relationship between EBV and the occurrence of LEC from the nasolacrimal duct. Owing to the rarity of LEC of the nasolacrimal duct, the optimal therapy remains unknown. The therapeutic strategy in this patient was founded on clinical experience from LEC of the salivary gland, for which Hsiung et al demonstrated the best prognosis was obtained with surgical resection
Author Contributions Yuan-Yun Tam, data collection, preparation of draft; Li-Yu Lee, analysis and interpretation of data; Kai-Ping Chang, article modification and editing of manuscript.
Disclosures Competing interests: None. Sponsorships: None.
References 1. Wu DL, Shemen L, Brady T, et al. Malignant lymphoepithelial lesion of the parotid gland: a case report and review of the literature. Ear Nose Throat J 2001;80:803– 6. 2. Leung SY, Chung LP, Yuen ST, et al. Lymphoepithelial carcinoma of the salivary gland: in situ detection of Epstein-Barr virus. J Clin Pathol 1995;48:1022–7. 3. Hsiung CY, Huang CC, Wang CJ, et al. Lymphoepithelioma-like carcinoma of salivary glands: treatment results and failure patterns. Br J Radiol 2006;79:52–5. 4. Chang KP, Hao SP, Lin SY, et al. The 30-bp deletion of Epstein-Barr virus latent membrane protein-1 gene has no effect in nasopharyngeal carcinoma. Laryngoscope 2006;116:541– 6. 5. Sakaida H, Kobayashi M, Yuta A, et al. Squamous cell carcinoma of the nasolacrimal duct. Eur Arch Otorhinolaryngol 2009;266:455– 8.
CASE REPORT
Lymphoepithelial carcinoma of the nasolacrimal duct Yuan-Yun Tam, MD, Li-Yu Lee, MD, and Kai-Ping Chang, MD, PhD, Taoyuan, Taiwan No sponsorships or competing interests have been disclosed for this article.
L
ymphoepithelial carcinoma (LEC), an uncommon lesion in the head and neck, consists of undifferentiated carcinoma cells within the lymphoid stroma. This malignancy has been found in the major salivary glands,1-3 but it has also been sporadically reported in the larynx and soft palate.1 Some studies suggested that LEC of the salivary gland and Epstein-Barr virus (EBV) are strongly correlated. To the best of our knowledge, this neoplasm derived from the nasolacrimal duct has never been addressed in any English literature. We present a rare case of a LEC originating from the nasolacrimal duct and address its differential diagnosis, management, and association with EBV. Institutional Review Board approval was obtained. A 61-year-old woman visited our clinic with symptoms of right progressive nasal obstruction, rhinorrhea, intermittent epistaxis, and nasal stuffiness for six months. She also complained of frequent right epiphora and a slowly enlarging mass below the right medial canthus, which was diagnosed as dacryocystocele by an ophthalmologist. Nasal endoscopy revealed an exophytic tumor located inferiorly and laterally to the inferior turbinate. Transnasal biopsy of the lesion revealed an LEC. MRI of the head and neck disclosed a lesion that emerged from the right nasolacrimal duct (Fig 1) with extension to the inferior meatus. Physical examination and systemic work-up for metastasis showed negative results. We performed an en-bloc resection with medial maxillectomy through right lateral rhinotomy. For better locoregional control, the patient underwent adjuvant radiotherapy with 64 Gy in 32 fractions over seven weeks. At the 33-month follow-up (the time of writing), there were no signs of locoregional or distant tumor relapse. Grossly, the tumor was polypoid, whitish, and firm, and measured 2.3 ⫻ 1.3 ⫻ 1.3 cm, with invasion of the maxillary bone. Histopathologic examination of the tumor revealed a circumscribed mass containing aggregates of malignant epithelial cells bathed in a background of dense lymphocytic infiltrates and fibrous stroma (Fig 2). Low magnification revealed indistinct epithelial components and distinct heavy in-
filtration of lymphoid cells. At higher magnification, nests of carcinoma cells consisting of scanty cytoplasm with ill-defined cell borders, large nuclei, and prominent nucleoli appeared. Both in situ hybridization to identify small EBV-encoded RNA (EBER) and polymerase chain reaction (PCR) amplification for the EBV latent membrane protein-1 (LMP-1) oncogene were performed with positive results.4 Serum IgA antibodies against the EBV capsid antigen (EBV VCA IgA) and EBV early antigen and nuclear antigen-1 (EBV EA⫹ NA1 IgA) were also detected at 1:40 and 45.523 EU/mL, respectively.
Discussion Tumors derived from the lacrimal drainage system are very rare. Only a few tumors, such as lymphoma, Kaposi’s sarcoma, adenocarcinoma, angiofibroma, and squamous cell
Figure 1 Coronal view of T2-weighted MRI revealed a mass occupying the right nasolacrimal duct (arrow).
Received January 22, 2009; revised March 31, 2009; accepted May 29, 2009.
0194-5998/$36.00 © 2010 American Academy of Otolaryngology–Head and Neck Surgery Foundation. All rights reserved. doi:10.1016/j.otohns.2009.05.035
Tam et al
Lymphoepithelial carcinoma of the nasolacrimal duct
145
followed by radiotherapy.3 Moreover, in previous reports of tumors of the nasolacrimal duct, an en-bloc excision was usually applied.5 Therefore, en-bloc resection along with adjuvant radiotherapy was recommended in this case. We present the clinicopathologic manifestations, the association with EBV, and the management of this rare case to remind clinicians that vigilance and proper diagnosis, recognition of its distinct nature from other neoplasms in the lacrimal drainage system, and appropriate treatments are the cornerstones of management for this aggressive malignancy.
Author Information Figure 2 Hematoxylin and eosin stain showed the typical histopathologic features of lymphoepithelial carcinoma, characterized by islands of undifferentiated large carcinoma cells permeated and enveloped by an admixture of lymphocytes.
From the Departments of Otolaryngology–Head and Neck Surgery (Drs Tam and Chang) and Pathology (Dr Lee), Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Taoyuan, Taiwan. Corresponding author: Dr Kai-Ping Chang, MD, PhD, Department of Otolaryngology–Head and Neck Surgery, Chang Gung Memorial Hospital and Chang Gung University College of Medicine, No. 5 Fu-Shin Street, Kwei-Shan, Taoyuan, Taiwan, 33333. E-mail address: [email protected].
carcinoma have ever been reported in this particular location. Most of these patients presented with the symptoms of epiphora, blood-tinged tears, and epistaxis.5 In our case, right epiphora and dacryocystocele also suggested that the lacrimal drainage system was obstructed and that neoplastic diseases should be taken into consideration in the differential diagnosis. LEC has a unique racial prevalence in Inuits and Asians, and an association has been suggested between LEC and EBV infection.1,2 As in nasopharyngeal carcinoma, which is frequently diagnosed in southeastern Asians, most of the EBV-associated tumors including LEC often share similar characteristic histologic patterns, such as syncytial sheets of undifferentiated malignant cells with a lymphoid infiltrate background.2 In the present case, both in situ hybridization for the presence of EBER and PCR amplification for the LMP-1 gene showed positive results. These findings might collectively suggest the possibility of a carcinogenic relationship between EBV and the occurrence of LEC from the nasolacrimal duct. Owing to the rarity of LEC of the nasolacrimal duct, the optimal therapy remains unknown. The therapeutic strategy in this patient was founded on clinical experience from LEC of the salivary gland, for which Hsiung et al demonstrated the best prognosis was obtained with surgical resection
Author Contributions Yuan-Yun Tam, data collection, preparation of draft; Li-Yu Lee, analysis and interpretation of data; Kai-Ping Chang, article modification and editing of manuscript.
Disclosures Competing interests: None. Sponsorships: None.
References 1. Wu DL, Shemen L, Brady T, et al. Malignant lymphoepithelial lesion of the parotid gland: a case report and review of the literature. Ear Nose Throat J 2001;80:803– 6. 2. Leung SY, Chung LP, Yuen ST, et al. Lymphoepithelial carcinoma of the salivary gland: in situ detection of Epstein-Barr virus. J Clin Pathol 1995;48:1022–7. 3. Hsiung CY, Huang CC, Wang CJ, et al. Lymphoepithelioma-like carcinoma of salivary glands: treatment results and failure patterns. Br J Radiol 2006;79:52–5. 4. Chang KP, Hao SP, Lin SY, et al. The 30-bp deletion of Epstein-Barr virus latent membrane protein-1 gene has no effect in nasopharyngeal carcinoma. Laryngoscope 2006;116:541– 6. 5. Sakaida H, Kobayashi M, Yuta A, et al. Squamous cell carcinoma of the nasolacrimal duct. Eur Arch Otorhinolaryngol 2009;266:455– 8.