Understanding the impact of clinicopathologic factors on survival would provide a basis for risk-adapted therapy. Previous studies suggest patients with MFH-B and osteosarcoma have similar survival rates, despite patients with MFH-B showing lower chemosensitivity and being older at presentation [3, 19]. Along with those contradictory reports, most MFH-Bs are purely osteolytic and infrequently show a periosteal reaction . Moreover, patients with an osteosarcoma with a purely osteolytic radiologic finding reportedly have a poor prognosis . We therefore asked (1) whether there is a difference in the initial tumor volume, histologic response, and survival between cohorts with MFH-B and osteosarcoma, and (2) whether histologic responses and survival rates differed between these two groups even after matching for volume and age.
Our study has several limitations that should be considered. First, all patients did not receive the same chemotherapy regimen. Seven (26%) of the 27 patients with MFH-B received a regimen other than the modified T-10 protocol. Three of seven patients received CYVADIC and four of seven had IFO-CDDP. However, all seven patients received two of the three drugs (Adriamycin, CDDP, IFO) known to be effective for treating osteosarcoma. Moreover, a previous report suggests the use of different regimens do not affect the survival rate . Second, there is potential bias in selecting a control group. Although underpowered owing to the small sample size, when selecting the prognostic parameters for matching we identified tumor volume and age as initially significant factors for survival. Third, the data for tumor volume were calculated from the dimensions measured on the MR images by simplified mathematical formulas rather than by using a special built-in software package in the MRI scanner. Therefore, there is potential risk of overestimating or underestimating real tumor volume. However, Shin et al. reported that tumor volume measured by MRI using the ellipsoid mass formula closely correlated with the volume calculated by the built-in software package in the MRI scanner .
Our findings concur with those of other studies regarding MFH-B survival outcome on multidisciplinary treatment and its similarity with osteosarcoma with respect to prognosis (Table 3). However, the percentage of good responders to chemotherapy is similar to that of the osteosarcoma cohort. In previous reports, there is debate regarding whether patients with MFH-B show inferior chemosensitivity [1, 5, 19]. Two factors such as tumor volume and chemotherapy regimen may be related to the response to preoperative chemotherapy. For the chemotherapy regimen, Bacci et al.  and Picci et al.  reported consistently poorer chemosensitivity regardless of the number of drugs used and the dose intensity. However, given a similar chemotherapeutic regimen, two studies [3, 5] reported higher proportions of good responders over those reported by Bacci et al. and Picci et al. Although we are uncertain of the reason for this discrepancy, a possible explanation would be a difference in initial tumor volume. The initial tumor volume tended to be larger in patients in the studies reported by Bacci et al., Picci et al., and others [3, 5] than we observed. Still, despite the larger tumor volume and low response rate, the survival rates reported by Bacci et al. and Picci et al. for patients with MFH-B are similar to others including ours. A large cohort study would be needed to explain this discrepancy.
Our data suggest similar survival rates and histologic responses between patients with MFH-B and osteosarcoma. This implies that despite patients with these two disease entities differing in mean age at diagnosis and radiologic presentation, they show a similar response to contemporary therapy. However, a consistent finding across studies is that good responders with MFH-B seem to have a lower chance of recurrence than good responders with osteosarcoma [3, 11, 19]. We obtained almost the same distribution of good responders among patients with MFH-B and osteosarcoma. Of the 54 matched patients with osteosarcoma, nine (33.3%) of 27 patients with a good response subsequently experienced metastasis, and 10 (40%) of 25 patients with a poor response had subsequent relapse. However, of the 27 patients with MFH-B, none of the 11 who achieved a good response died of the disease, whereas eight (61%) of the 13 who achieved a poor response had metastasis develop. Therefore, there is a possibility that survival is related more with histologic response in MFH-B than in volume-matched osteosarcoma.
Initial clinicoradiographic findings of MFH-B, such as older age at presentation than for patients with osteosarcoma, scant periosteal reaction, a lytic pattern observed on plain radiographs, and the presence of pathologic fracture, can cause physicians to perform erroneous surgical procedures. In the masked analysis of the radiographs of patients with MFH-B, a clinical impression of MFH-B was not the first priority in the differential diagnosis . Therefore, especially for older patients, there is a substantial chance of misdiagnosis if we perform a surgical procedure when we have only a clinical impression. In our study, 18% (eight of 44) of patients with MFH-B underwent an intralesional procedure, which is substantially larger than the 5.4% reported for patients with osteosarcoma [13, 14].
We found patients with MFH-B and osteosarcoma have similar survival rates and histologic responses to chemotherapy. We believe these two disease entities are similar regarding response pattern to contemporary therapy, despite having a different clinical presentation.
We thank J.S. Koh, MD, M.S. Kim, MD, and Yijung Seo, RN for assistance with pathologic reviews and data collection.
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