Malignant transformation of fibrous dysplasia in McCune-Albright syndrome is observed in less than 1% of cases, thus osteosarcoma is developing more frequently. According to the search in the PubMed database over the last 5 years, 13 publications were found, but none of them described cerebral cranium damage.
MATERIAL AND METHODS
A clinical observation of a 27-year-old patient with polyostotic fibrous dysplasia, skin manifestations and prolactin+HGH-secreting pituitary adenoma — McCune-Albright syndrome — without GNAS gene mutation is described. The patient had a rapidly increasing formation in the left temporoparietal region, which reached 18×20×15 cm, accompanied by local pain and fever 4 months prior to hospitalization. Diagnosis of osteosarcoma was verified by biopsy. Neoadjuvant chemotherapy in the presence of intracranial hypertension and giant tumor has been refused.
RESULTS
Preoperative embolization of tumor vessels with polyvinyl alcohol emboli was moderately effective. Tumor node has been removed, blood loss amounted to 5500 ml, blood autodonation, cell saver, 1 dose of donor erythrocytes were used. On the 1st day, the patient was transferred to the clinical unit, pain and fever ceased. He was discharged on the 8th day in satisfactory condition. Nevertheless, chemotherapy in the home area was not performed and the patient died from the disease progression in 1.5 months. The algorithm of care in osteosarcoma — neoadjuvant chemotherapy, tumor resection and adjuvant treatment.
However, the results of such treatment remain generally unsatisfactory. The algorithm of treatment for patients with fibrous dysplasia malignancy in McCune-Albright syndrome is not presented in the literature. The surgical intervention resulted in the removal of the main tumor volume and regression of intracranial hypertension, but did not significantly affect the patient’s fate in the absence of adjuvant chemotherapy.
CONCLUSION
The possibility of malignant neoplasms development from fibrous dysplasia in McCune-Albright syndrome must be taken into consideration. The development of an algorithm for care in such patients requires accumulation of material and its analysis.
