The records of thirty-two patients who had had an adamantinoma of the
long bones were examined to investigate the relationship between the
clinical presentation, the histological subtype, and the method of
treatment, and the clinical result. All histological patterns of
differentiation that are characteristic of adamantinoma were observed,
including the basaloid, spindle-cell, tubular, squamous, and osteofibrous
dysplasia-like subtypes. Follow-up data were available for twenty-eight (88
per cent) of the thirty-two patients. These patients were followed for a
mean duration of 122 months (range, eleven months to twenty-nine years and
two months). Nine patients (32 per cent), all of whom had been managed with
an intralesional or marginal procedure, had a local recurrence of the tumor
after a mean disease developed in three of the nine patients. In five other
patients, metastasis developed without having been preceded by a local
recurrence. Thus, the over-all rate of metastasis was 29 per cent (eight
patients). The mean duration of survival for the patients who had
metastasis was twelve years and eight months. Statistical analysis of
various clinicopathological variables revealed intralesional or marginal
excision to be the most significant risk factor for a local recurrence or
metastasis (p < 0.001). Two patients who had had a presumed osteofibrous
dysplasia-like adamantinoma, which contained few isolated keratin-positive
epithelial cells within the stroma at the time of presentation, had a
full-blown adamantinoma at the time of the local recurrence. Although the
clinical course that was observed may be the result of a sampling error, it
poses questions as to the regressive nature of osteofibrous dysplasia-like
adamantinoma. On the basis of our findings and the data in the literature,
we believe that an osteofibrous dysplasia-like adamantinoma may be a
precursor lesion of the classic type of adamantinoma.