Pleochromocytoma is a benign neuroectodermal tumor of the adrenal medulla. Ten to thirty percent of these tumors become metastatic. The bone is the most frequent site for metastasis but jaw involvement is reported rarely. A case of mandibular involvement is reported and a literature review in this topic is presented. Preoperative management of these patients undergoing general anesthesia is not a routine practice in maxillofacial surgery, so this topic is also is explained.

BACKGROUND: Most instances of the parosteal osteosarcoma (OGS) are low-grade tumors. However, some parosteal OGSs undergo dedifferentiated transformation. Dedifferentiated parosteal OGS can cause distant metastasis and poor survival, and preoperative chemotherapy may be warranted. This study provides imaging clues for dedifferentiated parosteal OGS before treatment.
METHODS: The study retrospectively enrolled 23 patients with histologically proven parosteal OGS, including 69.6% (n = 16) low-grade and 30.4% (n = 7) dedifferentiated types. Preoperative images including radiography and magnetic resonance imaging were reviewed. The following imaging parameters and clinical outcomes were evaluated: 1) average age; 2) sex; 3) tumor size; 4) presence of string sign; 5) necrosis; 6) hemorrhage; 7) solid soft tissue component; 8) perforating vessels; 9) ossification grade; 10) marginal ossification; 11) periosteal reaction; 12) sunburst reaction; 13) bone marrow edema; 14) bone marrow invasion; 15) perifocal soft tissue edema; 16) adjacent joint involvement; 17) adjacent neurovascular bundle compression; 18) regional lymph node; 19) bone metastasis; 20) preoperative lung metastasis; 21) follow-up lung metastasis; and 22) recurrence.
RESULTS: The average maximal tumor sizes were 7.1 cm and 10.9 cm in low-grade and dedifferentiated types, respectively (p = 0.033). Sunburst periosteal reaction was visualized in two cases of low-grade type (12.5%) and four cases of the dedifferentiated type (57.1%) (p = 0.025) of parosteal OGS. None of our studied cases revealed preoperative lung metastasis. In the follow-up chest computed tomography, lung metastasis was noted in two cases of conventional type (14.2%), and four cases of dedifferentiated type (57.1%) (p = 0.040) of parosteal OGS. In receiver operating characteristic (ROC) curve analysis, the average tumor size and sunburst periosteal reaction showed good specificity (AUC = 0.070 and 0.072, respectively).
CONCLUSIONS: Compared with low-grade types, dedifferentiated parosteal OGS exhibits a considerably larger tumor size, more sunburst periosteal reaction, and a more frequent development of lung metastasis in the disease course. Tumor size and sunburst periosteal reaction are the most crucial imaging diagnostic factors.