Differentiating Pediatric Rhabdomyosarcoma and Langerhans Cell Histiocytosis of the Temporal Bone by Imaging Appearance

Abstract

Background and purpose: Rhabdomyosarcoma and Langerhans cell histiocytosis are malignant lesions that can affect the skull base with similar radiographic characteristics on CT and MR imaging. We hypothesized that location within the temporal bone determined radiographically can provide useful adjunctive information in differentiating these distinct neoplasms.

Materials and methods: We identified patients with Langerhans cell histiocytosis and rhabdomyosarcoma by using an imaging data base and International Classification of Diseases, Ninth Revision codes at a tertiary care academic medical center. Cross-sectional images were reviewed by a neurotologist and neuroradiologist, who evaluated the location of the lesions and scored each subsite-middle ear, mastoid, petrous apex, retrosigmoid/posterior fossa-on a scale of 0 (no involvement), 1 (partial), or 2 (complete involvement).

Results: We identified 12 patients representing 14 cases of Langerhans cell histiocytosis, and 9 patients representing 9 cases of rhabdomyosarcoma. For patients with Langerhans cell histiocytosis, mastoid involvement was rated 23/28 (82%) compared with 6/18 (33%) with rhabdomyosarcoma (P = .001). Langerhans cell histiocytosis was present in only the anterior portion of the temporal bone (petrous apex and middle ear) in 1 case (7.1%) and in the anterior portion of the temporal bone only in 5/9 (55%) cases of rhabdomyosarcoma (P = .018). The cortical bone was more commonly involved in Langerhans cell histiocytosis, 11/28 (39%) of cases compared with 2/18 (11%) cases in rhabdomyosarcoma (P < .05).

Conclusions: These results indicate that lesions involving only the anterior portion of the temporal bone (petrous apex and middle ear) are more likely to be rhabdomyosarcoma. Lesions involving the mastoid are more likely to be Langerhans cell histiocytosis. This difference in primary location may be helpful in predicting the pathology of these lesions on the basis of imaging.

Fig 1.

A, Axial T1 postcontrast fat-saturation MR image through the temporal bone demonstrates a peripherally enhancing posterior fossa or retrosigmoid LCH lesion (arrow). B, Axial standard algorithm postcontrast correlating CT scan demonstrates the same LCH lesion (arrow) with osseous destructive changes.

Fig 2.

A, Axial fat-saturated postcontrast T1 MR image through the temporal bone shows a heterogeneous avidly enhancing lesion within the right petrous apex (arrow) found to be an RMS. B, Axial postcontrast correlating CT image in a bone window shows the same lesion (arrow) with expansile changes to the right petrous apex.