Reduced S100 Protein Expression in Malignant Ossifying Fibromyxoid Tumors: A Case Report.

Ossifying fibromyxoid tumor of soft parts (OFMT) is a rare mesenchymal neoplasm of uncertain lineage. OMFT normally has a benign clinical course, and malignant variants are considered unusual. Criteria defining malignancy have not yet been clearly identified and universally accepted, and there is diagnostic uncertainty between pathologists as to how best to recognize a malignant variant. We present the case of a 68-year-old male patient who, following initial diagnosis of typical OFMT in the left scapular region, presented to the sarcoma service 9 years later with a short history of a solid lesion in the right calf. Biopsy confirmed metastatic OFMT and further imaging identified three other radiologically similar but distant lesions, which were later resected. The histology of the initial biopsy was reviewed, and the original observations were found to be accurate and due to current diagnostic criteria, the specimen was reported as typical. We propose that this case report contributes to a growing body of literature suggesting that negative S100 expression may be a useful feature in identifying and characterizing malignant OFMT.

Long-Term Outcomes of Smile Reconstruction in Möbius Syndrome.

BACKGROUND: Gracilis muscle transplant is the standard of care for smile reconstruction in children with Möbius syndrome. The authors describe the long-term clinical efficacy, durability, and psychosocial impact of this approach at a mean follow-up of 20 years. METHODS: Patients with Möbius syndrome who underwent gracilis muscle transfer between 1985 and 2005 were included in the study. The authors assessed midfacial movements using photographs and Scaled Measurement of Improvement in Lip Excursion measure, administered the patient-reported Facial Clinimetric Evaluation Scale, and used semistructured interviews. RESULTS: The mean age at surgery was 13.2 ±10.6 years and the mean follow-up was 20.4 ± 4.4 years postoperatively. Twenty-two gracilis muscle transplants were performed, all innervated by the motor nerve to the masseter. Photographic analysis using Scaled Measurement of Improvement in Lip Excursion software demonstrated that movement of the muscle was unchanged 20 years after surgery. Smile symmetry was stable long term. The overall mean Facial Clinimetric Evaluation score of the cohort was 62.3 ± 12.0. Facial comfort (86.4 ± 21.2) and social function (69.9 ± 18.5) subdomains were the highest. Although the facial movement subdomain score was 35.6 ± 12.4, the score related to smiling alone was 84.1 ± 20.2, indicating satisfaction with the midfacial reconstruction. Interview responses indicated high satisfaction with surgery; fulfilled expectations of social acceptance; improved communication; enhanced self-confidence; and a sense of increased facial symmetry, spontaneity, and smile excursion. CONCLUSION: Segmental gracilis muscle transplantation provides long-lasting, durable improvements in objective and patient-reported measures of facial function.