Children ultimately diagnosed with malignancy are referred to pediatric rheumatology clinics with provisional rheumatic diagnoses. We aimed to distinguish the features in these patients that lead to the correct diagnosis of malignancy.
A retrospective review of the case records of 29 children (19 boys and 10 girls, aged 1 to 15.5 years) with malignancy who were referred to 2 pediatric rheumatology centers between 1983 and 1997.
The suspected diagnoses on referral were: juvenile rheumatoid arthritis (12), nonspecific connective tissue disease (4), discitis (3), spondyloarthropathy (3), systemic lupus erythematosus (2), Kawasaki disease (2), Lyme disease (1), mixed connective tissue disease (1), and dermatomyositis (1). The final diagnoses were leukemia (13), neuroblastoma (6), lymphoma (3), Ewing's sarcoma (3), ependymoma (1), thalamic glioma (1), epithelioma (1), and sarcoma (1). Patients had features typical of many rheumatic disorders including musculoskeletal pains (82%), fever (54%), fatigue (50%), weight loss (42%), hepatomegaly (29%), and arthritis (25%). Features that were suggestive of malignancy included nonarticular "bone" pain (68%), back pain as a major presenting feature (32%), bone tenderness (29%), severe constitutional symptoms (32%), clinical features "atypical" of most rheumatic disease (48%), and abnormal initial investigations (68%). The atypical features included night sweats (14%), ecchymoses and bruising (14%), abnormal neurologic signs (10%), abnormal masses (7%), and ptosis (3%). Initial investigations with abnormal findings included complete blood count/smear (31%), discordant erythrocyte sedimentation rate and platelet count (28%), elevated lactate dehydrognease level (24%), plain skeletal x-ray films (28%), bone scan (21%), and abdominal ultrasonography (17%). Findings of investigations done before referral to the rheumatology clinic were not recognized as abnormal in 11 (40%) patients.
Patients with a diverse group of malignancies, other than leukemia, may present to the pediatric rheumatologist. Pediatric care providers should be familiar with typical features of childhood rheumatic disorders, and rheumatic diagnoses should be reevaluated in the presence of any atypical or discordant clinical features.