Historia Clinica

Leukemia & Lymphoma, December 2008; 49(12): 2263–2271

REVIEW

Non-Hodgkin lymphoma of the Waldeyer’s ring: clinicopathologic and therapeutic issues

SIDDHARTHA LASKAR1, PRANSHU MOHINDRA1, SUDEEP GUPTA2, TANUJA SHET3, & MARY ANN MUCKADEN1
Department of Radiation Oncology, 2Department of Medical Oncology, and 3Department of Pathology, Tata Memorial Hospital, Parel 400012, Mumbai, India(Received 16 August 2008; accepted 18 September 2008)
1

Abstract Extranodal non-Hodgkin lymphoma (NHL) in head and neck region is most commonly seen in the Waldeyer’s ring. Waldeyer’s ring is a unique subtype of mucosa associated lymphoid tissue (MALT), which shows rarity of low-grade or MALT-type lymphomas and a high incidence of diffuse large B cell lymphoma (DLBCL). The commonest histology isDLBCL with natural history similar to primary nodal NHL. However, high association with gastrointestinal involvement is reported. The diagnostic workup is similar to that of the usual nodal NHL, and in absence of a specific staging system, the Ann Arbor staging is followed. As compared with T-cell subtypes, B-cell phenotypes are less likely to present with mucosal ulceration, epitheliotropism andangioinvasion. Stage of disease, histology and use of combined modality treatment have been proposed as significant prognostic factors. Treatment has evolved from the use of extended field radiotherapy (RT) alone to the use of combined chemotherapy and RT leading to almost doubling of survival. Advances in pathology and further risk stratification of patients into prognostic groups could lead to thedevelopment of novel therapeutic strategies to improve outcome.

Keywords: Lymphoma, Waldeyer’s ring, prognostication, treatment

Introduction There has been an increase in the incidence of nonHodgkin lymphoma (NHL) across the globe ranging from 2 per 100 000 in regions of Asia to *10 per 100 000 in the United Kingdom [1–3]. Multiple sites of involvement, often with non-contiguous spread as wellas involvement of extranodal sites are common. Extranodal presentation is seen in 25–40% patients of NHL [4,5]. The criteria for diagnosis of a primary extranodal NHL (E-NHL) requires involvement of one or more extra nodal sites at presentation, either alone or with minor accompanying nodal involvement, suggesting the extranodal site as the primary site of origin [4]. Head and neck region is oneof the commonest extranodal sites of involvement. The Waldeyer’s ring (WR) is the most frequent site of involvement (i.e. nasopharynx, tonsils, oropharynx

and soft palate) followed, in descending order, by oral cavity, thyroid gland, paranasal sinuses, nasal cavity and larynx [6–8]. The most common histology seen at these sites is diffuse large B cell lymphoma (DLBCL) with natural history notvery different from that of primary nodal NHL. NHL is a radio and chemo-sensitive malignancy with high rates of response in the majority of cases. Waldeyer’s ring NHL Within the head and neck region, WR has been reported to be the commonest site of involvement accounting for 5–16% of all E-NHL and 60–70% of all E-NHL of the head–neck region [9,10]. Higher incidence has been reported in the Asianpopulation as reported in different series from Korea, Japan and Hong Kong [11–13]. An incidence of 15.5% has

Correspondence: Siddhartha Laskar, Department of Radiation Oncology, Tata Memorial Hospital, Dr. Ernest Borges Road, Parel, Mumbai 400012, India. Tel: þ91-22-24177167. Fax: þ91-22-24146937. E-mail: laskars2000@yahoo.com ISSN 1042-8194 print/ISSN 1029-2403 online Ó 2008 Informa HealthcareUSA, Inc. DOI: 10.1080/10428190802493686

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S. Laskar et al. of dysphagia, nasal obstruction or symptoms of Eustachian tube obstruction or may accidentally notice a mass in the region. Systemic symptoms are not very common occurring in less than 10–20% patients [22,25]. Occasionally, these patients may present with an ulcerative lesion mimicking a squamous cell carcinoma [19,32]....