Background: Primary non-Hodgkin's lymphoma of bone, often more
simply referred to as primary lymphoma of bone, is a rare subset of
non-Hodgkin's lymphoma in children. There are only a few small series of
primary lymphoma of bone in children with long-term follow-up, and none have
appeared in the orthopaedic literature.
Methods: A review of our institution's Pediatric Tumor Registry
identified fifteen cases of primary lymphoma of bone among 306 cases of
diagnosed non-Hodgkin's lymphoma between 1970 and 2003. Retrospective
evaluation included collection of demographic, clinical, radiographic,
treatment, and follow-up data. A univariate analysis was used to assess the
prognostic significance of risk factors with respect to survival of patients
from this series and in a summary analysis of data collected from similar
series in the literature.
Results: The patients included ten male and five female patients
with a mean age of 11.6 years. Most patients had a presenting complaint of
pain and had swelling and/or tenderness on physical examination. Eight
children had a solitary bone lesion, and seven had multiple bone lesions.
Overall, the mean number of bones involved was 3.1 per patient. The femur and
the pelvis were the most frequently involved bones. The ten surviving patients
were followed for a mean of 13.6 years. Five patients died: three of disease
progression, one of treatment-related complications, and one of an unrelated
cause. The mean time from diagnosis to death was 2.1 years. Nine patients
received chemotherapy only, whereas six patients received a combination of
chemotherapy and radiation therapy. In the present study, an age of nine years
or less was predictive of poor survival (p < 0.05). In the summary analysis
of cases collected from the literature, advanced stage, young age,
non-large-cell histology, and multiple-bone involvement were predictive of
poor survival (p < 0.05).
Conclusions: On the basis of the present series and a comprehensive
review of similar series in the literature involving patients with primary
lymphoma of bone, it appears that younger age, advanced-stage disease,
multiple-bone involvement, and non-large-cell histology are associated with
decreased survival as compared with older age, localized disease, single-bone
involvement, and large-cell histology, respectively.
Level of Evidence: Prognostic Level IV. See Instructions
to Authors for a complete description of levels of evidence.