Treatment of mandibular malignant fibrous histiocytoma during pregnancy

Abstract

A 30-year-old pregnant woman was referred to the University Hospital at 19 weeks gestation with a chief complaint of a rapidly progressing mass in the right cheek. Clinical examination showed a 2.4 2.4-cm rubbery hard, nontender, slightly mobile mass in the right mental region. The overlying mucosa appeared normal in color. There was no paresthesia of the right inferior alveolar nerve. The pregnancy, which was the patient’s first, had been uneventful up until the onset of the right cheek mass. Neither the history nor family history was significant. A panoramic radiograph showed no evidence of mandibular bone involvement. An incisal biopsy showed an unencapsulated proliferation of polygonal and multinucleated, pleomorphic, histiocytic-like and spindle-shaped fibroblast-like cells with small amounts of intervening collagenous stroma (Fig 1A). The neoplastic elements were arranged in a storiform, or cartwheel-like pattern (Fig 1B). The cells showed large atypical nuclei with multiple nucleoli; there were numerous mitotic figures. Immunohistochemical staining of formalin-fixed sections showed positive reactivity for vimentin, but the tumor cells were negative for cytokeratin, neuron-specific enolase, S100, and epithelial membrane antigen. The compositive histologic features were consistent with a diagnosis of pleomorphic MFH.1 Preoperative computed tomography (CT) showed a slightly raised mass of soft tissue with isodensity overlying the mental foramen (Fig 2). Abdominal magnetic resonance imaging (MRI) and thoracic CT scanning showed no evidence of metastatic disease. With the informed consent of both parents and her husband, surgical treatment was performed under general anesthesia at 23 weeks gestation. Wide resection of the primary tumor and an upper neck dissection were performed. The resulting jaw deformity was reconstructed with titanium plates (Fig 3). On sectioning, the surgical specimen had a dark yellow color. There was evidence of compressive bone resorption near the mental foramen (Fig 4). The tumor appeared to invade the surrounding subcutaneous adipose tissue. Five weeks after tumor surgery, at 28 weeks gestation, Cesarean delivery was performed. Both the mother and child recovered well. Scintigraphs after delivery showed no evidence of distant metastasis. Four weeks postpartum, she received 5 courses of multiagent MAID chemotherapy, which included mesna, doxorubicin, ifosfamide, and dacarbazine (Table 1). At 1-year follow-up, there was no evidence of local recurrence or of metastases.