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Primary nasal lymphoma (NSL) and nasopharyngeal lymphoma (NPL) are uncommon extranodal lymphomas that have often been grouped together in studies published in the literature. It is unclear whether or not NSL and NPL are biologically different entities. We reviewed the records of 25 NSL and 19 NPL patients who were managed between 1985 and 1995, to evaluate and compare their clinical characteristics and treatment outcome. Clinical variables, including age, sex, stage, tumour bulk and treatment modality, were assessed for their prognostic influence on survival. Nasal obstruction (80%), nasal discharge (64%) and epistaxis (60%) were the predominant symptoms in NSL patients; neck swelling/mass (42%), nasal obstruction (37%) and hearing impairment (32%) were common findings in NPL patients. Limited stage disease (I/II) was present in all NSL and in 80% of the NPL patients. Thirty-six per cent of the NSL and 32% of the NPL patients had bulky disease. The majority of the immunophenotyped NSLs were of T-cell lineage (75%, 12/16) and most immunophenotyped NPLs were of B-cell lineage (69%, 11/16). The patients were treated with radiotherapy alone (14%), chemotherapy alone (23%) or chemotherapy plus radiotherapy (64%). NSL patients showed a lower complete remission rate and higher relapse rate than NPL patients, although the difference did not reach statistical significance. Both the 5-year disease-free survival (36% versus 76%; P = 0.007) and overall survival (33% versus 82%; P = 0.003) were significantly worse in NSL compared with NPL patients. Advanced age (>60 years; P = 0.03) and bulky disease (P = 0.04) were also associated with inferior survival times. The analysis of sex, stage and type of therapy showed no evidence of significant impact on the survival. Despite the close anatomical relationship in origin, NSL and NPL were noted to behave as biologically distinct entities, in which the NSLs demonstrated a higher frequency of T-cell tumours and a much worse prognosis than NPL. In addition to the primary site, advanced age and bulky disease were also associated with reduced survival.

An unusual case of aggressive Stage IIE(B) primary natural killer cell lymphoma of the caecum is described in a 16-year old Chinese girl. The immunophenotype of the tumour cells was CD2+, CD3-, CD4-, CD5-, CD7+, CD8-, CD45RO+, CD45RA-, CD56+, CD57-. Southern blot analysis showed a normal germline arrangements of the T-cell antigen receptor and immuno-globulin heavy chain genes. This lymphoma pursued a highly aggressive clinical course, with the rapid development of an extensive local recurrence after an apparently complete resection and combination cytotoxic therapy. The patient died 7 months after diagnosis, despite receiving salvage treatment. Given the aggressiveness and poor prognosis in this biologically distinct primary gastrointestinal lymphoma, a more vigorous systemic therapy should be considered in addition to surgery.

We describe an unusual occurrence of bilateral conjunctival tumours in a 25-year-old woman. This was the first sign of relapse of acute monoblastic leukaemia. There was also both marrow relapse and subsequent skin infiltration. No central nervous system involvement was detected. The tumours appeared as pink raised lesions in the upper conjunctivae of both eyes. They were not associated with pain or visual impairment. Conjunctival tumour biopsy revealed a dense mononuclear cell infiltration. Complete remission (conjunctival tumours, skin infiltration and bone marrow) was attained following systemic chemotherapy in combination with intrathecal chemotherapy.