Osteoblastoma is, by definition, a benign bone tumor with a recurrence potential as high as 22%.
24 However, malignant transformation has been reported in rare cases.
25‐
27 All cases of malignantly transformed osteoblastomas reported to date have shown a conversion to osteosarcomas only in recurrent tumors. In the Mayo Clinic files, only one case of malignant transformation from 108 cases of osteoblastoma has been recorded. However, the diagnosis of “malignancy” in a osteoblastoma, recurrent or otherwise, is particularly difficult because of the aggressive behavior of some osteoblastomas. Radiographic findings of cortical destruction (39%) or suggestive of malignancy (12%) are not uncommon.
24 Furthermore, histologic findings do not necessarily correlate with aggressive behavior even in “benign” osteoblastomas.
24 Rather, the clinical outcome correlates with the location such that tumors in short or flat bones or bones of the neuraxis demonstrate more aggressive behavior.
24,
28 The entity “aggressive osteoblastoma” was introduced by Dorfman and Weiss
29 and Lucas et al.
24 to describe lesions with features borderline between osteoblastoma and osteosarcoma. A permeative pattern of growth within intratrabecular spaces and lack of maturation toward the periphery of the lesion have been described as a histologic clue for distinguishing osteoblastoma from osteosarcoma.
24,
30 However, multifocal tumor growth, frequently present in osteoblastomas, may be confused with permeation.
31 To complicate matters further, although the mortality associated with osteoblastoma-like osteosarcoma can be significant from local disease (40% in one series), the metastatic rate of osteoblastoma-like osteosarcoma may be low (9%).
32 In contrast, the conventional osteosarcoma is considered a systemic disease at the time of diagnosis. Therefore, we believe a spectrum of tumors exists, with osteoblastoma at one end and osteoblastoma-like osteosarcoma at the other, all of which are treated with either local or wide excision depending on the degree of clinical aggressiveness. Limited molecular data support a possible progression model in this disease.
25,
33,
34 True transformation to malignancy, as evidenced by distant metastases, is extremely rare but should be considered a separate clinicopathologic entity from osteoblastoma-like osteosarcoma.
25 Osteoblastomas with conversion to osteosarcoma require aggressive surgical management combined with chemotherapy similar to conventional osteosarcomas.