Haematolymphoid tumours

Hodgkin lymphoma

Hodgkin lymphoma only rarely shows primary involvement of the nasopharynx {1274,1602,1756,1763,1922}. The patients usually present with nasal obstruction or otitis media, and frequently have low stage (stage I/II) disease. Most of the tumours are of mixed cellu-larity and nodular sclerosis subtypes. The majority of cases involving the nasopharynx are associated with Epstein-Barr virus {1274,1756}. Please refer to 'Hodgkin lymphoma' in 'WHO classification of tumours: Tumours of haematopoietic and lymphoid tissues' for details.

Non-Hodgkin lymphoma Definition

Primary non-Hodgkin lymphoma (NHL) of the nasopharynx is defined as a lym-phoid cell neoplasm in which the bulk of disease occurs in this site.

Epidemiology

Nasopharyngeal NHL accounts for 2.5% of all extranodal NHLs {809}. Most cases have been reported in the literature in combination with either NHL of the nasal cavity or NHL of the Waldeyer ring, rendering it difficult to extract the specific details on nasopharyngeal NHL {420,1704,2250,2849}. In some cases, there is simultaneous involvement of both the nasopharynx and nasal cavity, precluding determination of the site of origin of the NHL.

In the West, nearly all cases of nasopha-ryngeal NHL are of B-cell lineage (most commonly diffuse large B-cell lymphoma, DLBCL) {1704}. The situation is different in Asia, where B-cell lymphomas account for only 50-60% of cases {420,2849}, due to a higher frequency of extranodal NK/T cell lymphomas and peripheral T-cell lymphomas. Most patients with nasopharyngeal NHL are adults. Patients with extranodal NK/T cell lymphoma of nasal-type have a male to female ratio of 3:1, and a median age of 53 years {420}. Patients with B-cell lymphomas are generally one decade older (median age of 63 years), and the male to female ratio is only 1.2:1 {420}. Burkitt lymphoma occurs more frequently in children and young adults {2826}.

Etiology

The etiology is unknown, except that extranodal NK/T cell lymphoma of nasaltype is strongly associated with Epstein-

Barr virus (EBV) (>95%) Irrespective of the ethnic background of the patients {1195}. The association of nasopharyngeal DLBCL with EBV is weak {376}.

Clinical features

The patients present with nasal obstruction, epistaxis, hearing impairment, dysphagia, headache or neck mass, similar to the presenting symptoms of nasopha-ryngeal carcinoma. A small proportion of patients have concurrent cervical lym-phadenopathy, a feature seen more frequently in DLBCL than extranodal NK/T cell lymphoma.

Tumour spread and staging

The majority (80%) of patients have localized disease (Stage IE/IIE) at presentation {420,1500,1505,1550}. Extranodal NK/T cell lymphoma tends to disseminate to various sites, such as skin, gastrointestinal tract, liver, lymph node and testis, during the course of disease. There is a propensity for DLBCL to spread to the cervical lymph nodes {420}.

Histopathology

DLBCL and extranodal NK/T cell lymphoma of nasal-type occurring in the nasopharynx are morphologically and

Nasal Lymphoma Cell

Fig. 2.29 Primary non-Hodgkin lymphoma of the nasopharynx. A Diffuse large B-cell lymphoma. A diffuse infiltrate of large lymphoid cells with high nuclear-cyto-plasmic ratio and mitotic figures (left). There is strong CD20 immunoreactivity, confirming the B-cell immunophenotype (right). B Extranodal NK/T-cell lymphoma. The neoplastic cells infiltrate the vascular wall (left), and show immunoreactivity for CD3e (right).

Fig. 2.29 Primary non-Hodgkin lymphoma of the nasopharynx. A Diffuse large B-cell lymphoma. A diffuse infiltrate of large lymphoid cells with high nuclear-cyto-plasmic ratio and mitotic figures (left). There is strong CD20 immunoreactivity, confirming the B-cell immunophenotype (right). B Extranodal NK/T-cell lymphoma. The neoplastic cells infiltrate the vascular wall (left), and show immunoreactivity for CD3e (right).

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Responses

  • morgan
    What does this mean haematolymphoid lineage?
    1 year ago

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