Osteosarcomas of the skull. Clinical remarks on 19 cases

Abstract

Background. Osteosarcomas are typically long bone tumors and rarely affect the skull, with most articles reporting single cases. As elsewhere in the body, these lesions may be classified as primary or secondary, chiefly post‐Paget and post–radiation therapy. Methods. The authors reviewed the osteosarcomas of the skull diagnosed at the Division of Neurosurgery of “La Sapienza” University of Rome. The patients were placed into two groups, according to the treatment received (prechemotherapeutic era and chemotherapeutic era). In the last five patients, a diagnostic‐therapeutic protocol was adopted. Results. The most effective investigations are plain radiographs, computed tomography (CT) with bone windows, and magnetic resonance imaging (MRI), with the latter two allowing assessment of the extraosseous extent of the disease. Chemotherapy has changed the prognosis dramatically, achieving cure in some cases (especially in de novo osteosarcomas). Before the age of chemotherapy, the median survival length was 16 months, but since its introduction, five of nine patients in this study are alive 2 years after diagnosis. The onset of metastases, chiefly to the lung, does not necessarily imply a poor prognosis. The authors propose a schedule for the diagnosis and treatment of new cases of osteosarcoma of the skull. Conclusions. Plain radiographs, CT targeted on the bone, and MRI are the most important diagnostic tools. Aggressive chemotherapy together with surgery (eventually including local radiation therapy in nontotal macroscopic surgical removal of the lesions) can drastically modify the prognosis of de novo and post‐radiation therapy osteosarcomas.

Osteosarcoma of the cranial vault and skull base in pediatric patients

“…59 Like osteosarcoma of the long bones, craniofacial osteosarcomas often present as slow-growing masses. 51 Unlike long bone lesions, however, craniofacial tumors are frequently painless or only mildly tender.…”

“…51 Unlike long bone lesions, however, craniofacial tumors are frequently painless or only mildly tender. 32,51,59 Common presenting symptoms include headache, cranial nerve palsies, exophthalmos, and visual impairment. 22,51 Elevation of the serum alkaline phosphatase level is common, although not uniformly observed.…”

“…22,51 Elevation of the serum alkaline phosphatase level is common, although not uniformly observed. 59 Imaging with MRI and CT may reveal bone growth with lytic regions and periosteal remodeling, as seen in osteosarcomas of other areas. 58 The most common skull masses in children are dermoid cysts and Langerhans cell histiocytosis, which may be difficult to distinguish from malignant tumors such as osteosarcoma or Ewing sarcoma.…”

“…38 Tumors arising secondary to a pathological process are typically more aggressive and have higher recurrence rates than those arising de novo. 54,59 Malignancy resulting from these predisposing factors is also more likely to appear at a more advanced age, 32 contributing to the low incidence of cranial osteosarcoma in the pediatric population. 32,33,59 Traumatic injury has also been implicated in the development of osteosarcoma.…”

“…54,59 Malignancy resulting from these predisposing factors is also more likely to appear at a more advanced age, 32 contributing to the low incidence of cranial osteosarcoma in the pediatric population. 32,33,59 Traumatic injury has also been implicated in the development of osteosarcoma. 41 We report our experience treating cranial osteosarcoma in three 14-year-old boys and review the available literature regarding the management of these rare, aggressive tumors.…”