Head pleomorphic sarcoma showing murine double minute 2 amplification without a well-differentiated liposarcoma component in a pediatric patient.

BACKGROUND: Murine double minute 2 (MDM2) is an oncogene that inhibits p53, leading to decreased apoptosis. Sarcomas showing MDM2 amplification are rare among pediatric patients. CASE: A 14-year-old boy presented with pleomorphic sarcoma of the head showing MDM2 amplification without a well-differentiated liposarcoma component. Although chemotherapy was initially performed to reduce the tumor size before surgery, the tumor did not shrink. The patient underwent complete surgical resection. Microscopic examination revealed a positive surgical margin; thus, postoperative proton-beam radiotherapy was performed. 3 years after the therapy, no sign of recurrence was observed. CONCLUSION: Macroscopic surgical resection combined with adjuvant postoperative radiotherapy was effective against MDM2-amplified pleomorphic sarcoma refractory to neoadjuvant chemotherapy in a pediatric patient.

Alveolar soft part sarcoma of the larynx: a case report of an unusual location with immunohistochemical and ultrastructural analyses.

BACKGROUND: Alveolar soft part sarcoma (ASPS) is a rare mesenchymal neoplasm of uncertain origin. In this article, we report a case of ASPS occurring in the larynx, an extremely rare location for this rather unusual tumor. METHODS AND RESULTS: The patient was a 34-year-old Japanese woman who requested an examination for hoarseness. The tumor showed a proliferation of large polygonal cells with periodic-acid-Schiff-positive diastase-resistant intracytoplasmic granules, arranged in an alveolar growth pattern. The cytoplasm of the tumor cells was eosinophilic. Tumor cells were positive for vimentin and titin. Nuclear immunoreactivity for TFE3 was observed, and the Ki-67 labeling index was 14.7%. Ultrastructurally, electron-dense rod-shaped crystals were infrequently observed in the cytoplasm. This case was finally diagnosed as ASPS of the larynx. CONCLUSION: We discuss the histogenesis and differential diagnosis of ASPS with immunohistochemical and ultrastructural findings. TFE3 immunohistochemistry was found to be a very useful marker for the diagnosis of ASPS.

Velopharyngeal function after microsurgical reconstruction of lateral and superior oropharyngeal defects.

OBJECTIVES/HYPOTHESIS: Defects of the lateral and superior oropharyngeal wall are difficult to reconstruct because of their complicated anatomy and the possibility of causing velopharyngeal incompetence. The objective was to investigate problems of reconstruction and postoperative velopharyngeal function. STUDY DESIGN: Defects were classified into three types (I, II, and III) according to their extent. Four operative procedures were performed: the Patch, Jump, Denude, and Gehanno methods, which include a lateral-posterior pharyngeal advancement flap. Speech intelligibility, velopharyngeal function, and wound dehiscence between the flap and the remaining soft palate were evaluated. METHODS: Forty patients who had undergone resection of the lateral and superior oropharyngeal walls and subsequent reconstruction were reviewed. RESULTS: Most patients with type I or II defects had satisfactory velopharyngeal function. However, in patients with type III defects, speech function was worse and severe velopharyngeal incompetence was more common. The type of defect and the presence of wound dehiscence were related to postoperative function. The rates of wound dehiscence were lower with the Patch and Gehanno methods. CONCLUSIONS: Postoperative function in patients with type III defects can be affected by various factors. We suggest that the Gehanno method be the treatment of choice for reconstruction of extensive defects of the oropharynx. However, patients in whom more than two-thirds of the superior and posterior oropharyngeal walls has been resected are poor candidates for reconstruction because of the difficulty of maintaining both nasal airway patency and velopharyngeal function.