Scheduled follow-up programs failed to detect relapses in 50% of cases presented here. Survival after relapse is not affected by whether relapse was detected at a scheduled or an unscheduled visit.
A 16-year-old youth was referred to our hospital for evaluation of a scalp lesion associated with cervical lymphadenopathy. Six months previously he had presented elsewhere with painful cervical lymphadenopathy and fever. A biopsy of the lymph node was inconclusive. On presentation to us he had a bald scalp area over a deeply infiltrating tumour mass. Cranial magnetic resonance imaging showed a densely enhancing, mushroom-like soft-tissue mass involving the pericranium, the aponeurotic coverings (left) and the dura mater (left). The external cerebral surface and the adipose layer of the scalp were apparently intact. The tumoral tissue had grown through a large osteolytic area of the left parietal bone, which could easily be seen using three-dimensional computerized tomography (right).A biopsy from the parietal lesion showed an anaplastic large cell lymphoma (ALCL). Malignant cells on immunohistochemical investigation were positive for CD30, epithelial membrane antigen, ALK and BH9. A study of the t(2;5)(p23;q35) investigated using reverse transcription-polymerase chain reaction was negative. Extensive staging investigations found no other evidence of disease. Primary osseous ALCL was diagnosed and polychemotherapy was started. Clinical response was complete and is still ongoing 1 year after diagnosis.Anaplastic large cell lymphoma, mainly affecting adolescents and young adults, represents approximately 10-15% of childhood lymphomas. Presentation can be varied and in some patients spontaneous regression followed by rapid progression is observed before a diagnosis is made. Combination chemotherapy is effective but skin involvement with nodal disease, visceral or mediastinal involvement and high lactate dehydrogenase levels are associated with an increased risk of failure.
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