Extranodal T-Cell Lymphoma of Nasal Cavity

Abstract

A rare case of primary extranodal lymphoma of nasal cavity is here with reported in a 11 years old female child. Patient presented with space occupying extensive lesion in nasal cavity with destraction of nasal septum and obliteration of paranasal sinuses. After histological examination and routine stain showed a round cell tumor with provisional diagnosis of large cell non-Hodgkin’s lymphoma was made. Immunohistochemical stain were used to further differentiate from other possibilities mimics as carcinoma and round cell tumors. Immunohistochemical stain confirm the diagnosis of extranodal T-cell lymphoma. We report this case for its rarity in paediatric patients.

Introduction

Extranodal lymphoma arising in the nasal cavity and paranasal sinuses are relatively uncommon although they are most common nonepithelial malignant tumor of nose. Extranodal lymphoma represents 20–30% of all non Hodgkins lymphoma where as nasal and paranasal sinuses lymphoma can account for 13% of all non-hodgkin’s lymphoma. Commonly involves aerodigestive tacts comprising of waldeyers ring but occasionally involves nasal cavity. The mucosa of the nose and paranasal sinuses contains more diffusely distributed lymphoid elements. Apart from the nasal cavity other sites of involvements of extranodal lymphoma are skin, gut, testis, breast, kidney, upper respiratory tracts and rarely eye.

Case Report

A 11 years old female child presented with history of slow growing nodular swelling on right nasal cavity (Fig. 1). Systemic examination were normal. Hematological investigation were unremarkable. Bone Marrow finding were normal. CT-scan of nasal and paranasal sinuses showed a large expansile mass in the nasal cavity extending up to nasal septum with complete obliteration of the right nasal cavity and expanding in the maxillary, ethemoid, sphenoid sinuses on the right side (Fig. 2). Histological examination revealed round cell tumor made up of abnormal lymphoid cells showing irregular large vesicular nuclei with central nucleolus, nuclear outline was round to angulated with scant cytoplasm (Fig. 3). Immunohistochemical evaluation was positive for leukocyte common antigen, CD-3 (Fig. 4) and negative for cytokeratin, desmin,CD-20, CD99 and NSE. We come to the diagnosis of extranodal T-cell lymphoma on the basis of clinical presentation, histological finding and immunohistochemical study.

Fig. 1.

Nodular swelling on right side of nasal cavity

Fig. 2.

CT scan of nasal cavity showed large expansile mass

Fig. 3.

Diffuse population of a typical lymphoid cell (H&E stain ×40)

Fig. 4.

CD3 IHC stain showing diffuse positivity in tumor cells, ×40

Discussion

About 20–30% of non-Hodgkins lymphomas arise from extra nodal site. [1] and predominance of T-cell lymphoma in nasal cavity while most paranasal lymphomas are B-cell type [2]. The nasal and paranasal sinuses lymphomas can accounts for as many as 13% of all non-Hodgkin’s lymphomas [3]. Most upper aerodigestive tract NK/T-cell lymphomas were genotypically of NK cell origin and only few belong to T-cell lineage [4]. Sinonasal tract of non-Hodgkin’s are more common in Asia then in western population and predominantly T-cell lymphoma [5]. Non-Hodgkin’s lymphoma arising from paranasal sinuses presents late and occur relatively in elderly age group. Our case is different because it is reported in paediatric age group. Malignant lymphomas have a predilection for male and tend to occur in younger adults [6]. Within the sinonasal tract the maxillary sinus is more commonly is involved then the nasal cavity in western population, but in Asia then nasal cavity is more commonly involved [7]. Apart from the nasal cavity, other sites of involvement are skin, gut, testis [8] breast [9], kidney, upper respiratory tract and rarely eye. The usual histological type of large cell lymphoma of nasal type which on immunohistochemistry usually turn out to be T-cell lineage [10]. Non-Hodgkin’s lymphoma have a high cure and survival rate with early diagnosis and staging with appropriate treatment. The use of combined chemotherapy and radiotherapy significantly improved the 5 year disease free survival and overall survival rates. Multidrug chemotherapy (CHOP regimen) followed by involved field radiotherapy appears to be the most effective treatment approach [1]. In our case the initial treatment was chemotherapy, followed by radiotherapy with good results. The paediatric non-Hodgkin’s lymphomas most commonly encountered in neck lymph nodes then with in the nose and paranasal sinuses [11]. To conclude, apart from all round cell tumor and tumor of epithelial origen, the possibility of extranodal non-Hodgkins lymphoma should be taken in consideration.