Cranio-maxillofacial non-Hodgkin’s lymphoma: Clinical and histological presentation

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Abstract

Non-Hodgkin’s lymphoma represents about 5% of all malignant lesions of the head and neck. In this study we retrospectively evaluated clinical presentation, histological subtype and long-term prognosis of 42 patients with non-Hodgkin’s lymphoma involving the craniofacial area. The mean age at diagnosis was 64 years. More than half of the patients presented with disseminated disease at multiple sites (55%, n = 23). In 62% (n = 26) the first manifestation was extranodal. The most common affected region was the oral cavity (65%, n = 17). Treatment consisted of local therapy, including surgical resection and radiation, as well as chemotherapy with or without local therapy. Recurrence occurred in 31% (n = 13) of the treated patients. Mean survival after first diagnosis varied from 17 months in patients presenting with diffuse large B-cell lymphoma (DLBCL), to 8.5 years in patients with follicular lymphoma. The most common histological subtype is DLBCL. Standard treatment for DLBCL consists of chemotherapy combined with CD 20 monoclonal antibody, even after total resection of the tumour. There is high risk of systemic disease in patients presenting with non-Hodgkin’s lymphoma and high risk of post therapy recurrence.

Introduction

Malignant lymphoma represents approximately 5% of all malignant lesions of the head and neck. It is the second most common malignant lesion of the head and neck. The most common malignancy is squamous cell carcinoma (DePena et al., 1990, Vega et al., 2005). Lymphoma has traditionally been categorised as either Hodgkin’s and non-Hodgkin’s lymphoma. Non-Hodgkin lymphoma (NHL) originates from B- or T-cell of lymphatic tissue, with plasmocytoma being a special clinical entity manifesting primarily in the bone marrow. Up to 40% of non-Hodgkin lymphomas present at an extranodal site. Extranodal non-Hodgkin lymphoma most frequently occurs in the gastrointestinal tract. The head and neck is the second most frequent site of extranodal manifestation (Vega et al., 2005). Primarily extranodal manifestations often involve regional or generalized nodal disease (Epstein et al., 2001). The World Health Organisation’s classification of non-Hodgkin’s lymphoma has been accepted by most pathologists and clinicians as a worldwide consensus classification system (Jaffe, 2009, Tan, 2009). It is based on distinct disease entities that can be defined by specific morphological, immunological, genetic and clinical parameters (Stein, 2000).

Section snippets

Material and methods

In this study, 42 patients with malignant non-Hodgkin’s lymphoma were included. They presented first at the Department of Oral and Maxillofacial Surgery of the University Hospital with unspecific swelling in the head and neck area in the time from 1993 to 2010. All data were retrospectively reviewed. Patient data obtained are shown in Table 1. All patients underwent surgical biopsy of the tumour. Histopathological analysis and immunhistochemical staining were performed by the Department of

Results

All patients in this study were submitted directly to the department of oral and maxillofacial surgery. The histological diagnosis was made in our department for all patients. All referrals were based on clinical findings of unspecific swelling in the head and neck area by the general practitioner or dentist. The mean age at diagnosis was 64 years (range 1–91 years, SD 18 years). Twenty-three patients (55%) were female. In 26 patients (62%) the first clinical presentation of NHL was extranodal, 9

Discussion

Malignant NHL manifested at a nodal site in about 70% of all cases, only 30% manifested at an extranodal site (Zucca et al., 2002).

Most cases of extranodal B-cell lymphoma present in the gastrointestinal tract as MALT-lymphoma (mucosa-associated lymphoid tissue). The head and neck region is the second most common site of extranodal lymphomas (Vega et al., 2005).

In our study, the most common first clinical presentation was a single extranodal site (60%). In 10 of 42 patients the first clinical

Conclusion

Non-Hodgkin lymphoma is a frequent differential diagnosis in tumours of the head and neck, representing about 5% of all malignant tumours in the area. Therefore, it should be considered as a possible diagnosis by oral and maxillofacial surgeons as well as dentists who see patients with unspecific head and neck swelling.

We therefore strongly recommend that patients with NHL should be treated by oncologists according to standard treatment protocols. Good interdisciplinary cooperation between

Conflict of interest

All authors disclose any financial and personal relationships with other people or organisations that could inappropriately influence or bias our work.

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