Spontaneous Malignant Transformation in Craniomaxillofacial Fibrous Dysplasia

Abstract

Spontaneous malignant transformation in craniomaxillofacial fibrous dysplasia (FD) is extremely rare and its clinicopathological characteristics remain largely unknown. Here, we aimed to characterize the epidemiology and clinicopathological features of malignancies arising from preexisting FD by presenting data from a Chinese tertiary referral hospital and review of English and Chinese literatures. The craniomaxillofacial disease registry of Shanghai Ninth People's Hospital was searched and reviewed to collect relevant information for patients diagnosed as malignant transformation in craniomaxillofacial FD between January 1993 and December 2010. An English and Chinese literature review was conducted to retrieve pertinent cases published in the past 2 decades with preset inclusion criteria. All included cases were further analyzed with regard to associated clinical and pathological variables. Three cases with osteosarcoma arising from previous craniomaxillofacial FD were found at our institution and 35 other cases were identified by literature review. These uncommon entities usually occurred in adults with a mean age of 39.8 years and equal gender preponderance. Maxilla remained the most common sites for malignancies followed by mandible and zygoma. Most malignancies were diagnosed as osteosarcoma followed by fibrosarcoma, chondrosarcoma, and malignant fibrous histiocytoma. Radical resection alone or with postoperative radiotherapy/chemotherapy remains the main treatment strategy with unfavorable prognosis due to local recurrence and distant metastasis. Taken together, our findings might for the first time provide the comprehensive information regarding the epidemiology, clinicopathological features, treatment, and prognosis of malignancies in craniomaxillofacial FD. Further investigations are warranted to improve early diagnosis and proper treatment for these uncommon entities.

Strategies for the Optimal Individualized Surgical Management of Craniofacial Fibrous Dysplasia

“…Thus, our patients are being regularly monitored because of the need for additional craniofacial bone surgical interventions and risks of progression, recurrence, appearance of new dysplastic bone lesions, and/or malignant degeneration. 1,2,12,34 Despite these limitations, we believe that the present study along with objective assessment of the results provides additional information that will broaden the field of knowledge of surgical outcomes of craniofacial fibrous dysplasia patients. Further research may confront or ratify our data and try to solve these drawbacks.…”

Malignant Sarcomatous Transformation of Fibrous Dysplasia

“…Yearly X-rays are advocated for screening, and patients must be educated to seek prompt medical attention upon experiencing a sudden change in the quality or severity of their symptoms. Treatment options for sarcomatous transformation of FD include conventional chemotherapy, in addition to radical resection if the tumor location is amenable to surgical excision [8]. …”

Radiation-Induced Dedifferentiated Chondrosarcoma With Orbital Invasion

“…18 Malignant transformation generally occurs in <1% of patients with FD, with the osteosarcoma being the most common histopathological type. 4,19 In a review of all cases of malignant transformation of craniomaxillofacial FD, Cheng et al 19 found a total of 38 cases in the past 2 decades. 4,19 In a review of all cases of malignant transformation of craniomaxillofacial FD, Cheng et al 19 found a total of 38 cases in the past 2 decades.…”

“…19,20 The interval between initial FD and subsequent malignancy is variable, but rarely seen before 2 decades. 4,[18][19][20] The role of radiotherapy in FD transformation remains controversial as some authors supported its pathogenic importance, while others question it. Osteosarcoma was the most common type of neoplasm identified, accounting for 65.6% of all cases.…”

“…4,19 In a review of all cases of malignant transformation of craniomaxillofacial FD, Cheng et al 19 found a total of 38 cases in the past 2 decades. 20,21 In their review, Cheng et al 19 found that 15 of the 38 patients (39.5%) who underwent malignant degeneration had received prior radiation. Fibrosarcoma was second most common at 13.1%, followed by chondrosarcoma 7.9%.…”