Spontaneous Conversion of Fibrous Dysplasia Into Osteosarcoma

Abstract

Spontaneous degeneration of sarcomatosis of fibrous dysplasia is a rare phenomenon in adolescence. Fibrous dysplasia is often a deforming and devastating condition that begins in childhood and accounts for approximately 7.5% of the benign bone neoplasms. Approximately 50% to 100% of patients with polyostotic disease and 10% with monostotic disease have craniofacial involvement. This report is about a 16-year-old adolescent boy who had rapid facial mass growth and diplopia at the time of his referral to our center. The patient had been diagnosed with fibrous dysplasia a year earlier, and since the first diagnosis, he was submitted to only 1 surgical intervention, which had been followed by recurrence. The main clinical findings were a bilateral zygomatic mass, saddling of the nasal dorsum, diplopia, superior and inferior gaze, and numbness in the right inferior orbital nerve. The patient had a high level of serum alkaline phosphatase. The diagnosis of osteosarcoma was established by biopsy, and the patient underwent surgical treatment. His postsurgery results were good, and he did not experience a relapse until 1 year after surgery. Unfortunately, 6 months after the recurrence of his condition, he died of uncontrollable local spread.

Conversion of Aneurysmal Bone Cyst into Fibrous Dysplasia: A Rare Pediatric Case Report

“…Despite the unclear relationship between these two entities, the literature describes the development of an ABC (or a malignant lesion outbreak) from a previous FD, or the coexistence of both conditions at the same time [15][16][17][18]. The cystic degeneration of an FD causing rapid enlargement of FD lesions was documented by ClausHermberg et al occurring in a 15-year old patient in 2011 [19].…”

Primary Osteogenic Sarcoma of Zygomatic Arch: A Case Report, With World Literature Review

“…[3,4] Craniofacial osteosarcomas are classified as primary and secondary types. Primary type occurs denovo, secondary type occurs in patients with pre-existing disease like skeletal paget's disease, [3] fibrous dysplasia of bone [5] or prior regional irradiation. [3] Patients with primary craniofacial osteosarcoma are usually present in the second and third decade.…”

Dental perspectives in fibrous dysplasia and McCune–Albright syndrome

“…Pain is common in craniofacial FD, affecting approximately 40% of patients 17, 31 . Rapid growth of jaw FD may also be associated with pathological lesions such as aneurysmal bone cysts, or more rarely malignant transformation to osteosarcoma or other forms of sarcoma 20, 21, 32–36 . Taurodontism, a condition characterized by enlargement of pulp chamber, is often associated with presence of endocrine disorders in FD/MAS 20 , suggesting that FD, endocrinopathies, or a combination of these may impact tooth development in FD/MAS patients.…”

“…Due to the presence of often-complex medical comorbidities, the dental aspects of FD/MAS are frequently overlooked, and dental needs are often underserved. Furthermore, the variable clinical, radiological and histological presentations of FD, as well as apparent risk of malignant transformation, cause some dental practitioners to delay or avoid dental surgical procedures in FD 32, 34–36 . Also, some dental healthcare providers may also feel uneasy about treating FD/MAS patients because of previous subjective reports that dental surgery might exacerbate jaw FD, transforming a quiescent lesion to an aggressively growing lesion 32, 33, 40 .…”