ORAL AND MAXILLOFACIAL PATHOLOGY Editor: Alan R. Gould Oral alveolar soft-part sarcoma: Review of literature and case report with immunohistochemistry study for prognostic markers Kelen Christine do Nascimento Souza, DDS, MSc, a Paulo Roge ´rio Faria, DDS, MSc, a Ivan Miguel Costa, DDS, b Antonio Francisco Duriguetto Ju ´nior, DDS, PhD, b and Adriano Mota Loyola, DDS, PhD, b Uberaba and Uberla ˆndia, Brazil FACULTY OF MEDICINE TRIANGULO MINEIRO AND FEDERAL UNIVERSITY OF UBERLA ˆ NDIA Objective. To describe a case of alveolar soft-part sarcoma (ASPS) affecting the tongue of a child and to study prognostic imunohistochemical markers for the disease. Study design. Tissue sections were incubated with primary antibodies reactive to neuron-specific enolase (NSE), vimentin, desmin, S-100 protein, cytokeratins AE1-AE3, EMA, neurofilament, synaptophysin, and muscle-specific actin (MSA), and for prognostic markers, including Ki-67, p53, bcl-2, bax, and nm23. Results. Histologically, the tumor showed a proliferation of large polygonal cells with PAS-positive diastase-resistant intracytoplasmatic material, arranged in an alveolar growth pattern. Diffuse positive reaction for neuron specific enolase (NSE), focal reactivity for desmin and S-100 protein, strong positivity for nm23 and bax, but weak reaction for p53 and Ki-67 were found. No bcl-2epositive cells were noted. Conclusion. These immunohistochemical findings may reflect the less aggressive behavior of ASPS in oral tissues. (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005;99:64-70) Alveolar soft-part sarcoma (ASPS) is a rare malignant tumor of unknown histogenesis first described by Christopherson et al 1 in 1952. Most of the tumors occur in adolescents and young adults, with a predilection for females. 2,3,4 In young adults, the neoplasm often develops in the upper and lower extremities. However, in children and adolescents, this tumor commonly orig- inates in the head and neck where the orbit and tongue are most favored sites. 5-7 Usually, ASPS presents as a slow-growing lesion, but frequently it is often associated with a high incidence of pulmonary metastases early in the course of the disease. 4,8 Prognosis is unfavorable. For the nonmetastatic cases, survival is approximately 38% in 10 years of follow-up; for metastatic cases, most patients die within a short period of time. 6,7 Notwith- standing, the predictive value of prognostic markers, including proliferative indices (p53 and Ki-67), poten- tial of metastasis (nm23), and apoptosis (bcl-2 and bax) in ASPS of the oral cavity, is unknown. 9 The purpose of this paper is to report a case of ASPS arising on the dorsum of the tongue in a 13-year-old girl that had no metastases at the time of diagnosis, and to review the literature of case reports involving the oral tissues. Additionally, we describe the clinicopathologic and the immunohistochemical significance of prognos- tic markers. CASE REPORT A 13-year-old white girl was referred to the Oral Diagnosis Clinic at the School of Dentistry, Federal University of Uberla ˆndia, in March 2000, with an asymptomatic swelling on the dorsal portion of the tongue with a provisional diagnosis of hemangioma. According to the mother of the patient, the lesion was first detected 9 years beforehand. Unfortunately, no material was available to confirm this duration. During 6 months preceding the diagnosis, the lesion had grown in size. Upon oral examination, an erythematous, painless, nontender nodule approximately 3 cm in diameter on the left side of the dorsum of the tongue was observed (Fig 1, A). Extraoral a Fellow, Department of General Pathology, School of Medicine, FMTM Uberaba, Brazil. b Professor, Department of Oral Pathology, School of Dentistry, Federal University of Uberla ˆndia, Brazil. Received for publication Sep 3, 2003; returned for revision Oct 21, 2003; accepted for publication Jul 5, 2004. Available online 29 September 2004. 1079-2104/$ - see front matter Ó 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.tripleo.2004.07.002 64 Vol. 99 No. 1 January 2005