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Med Oral Patol Oral Cir Bucal 2 Oral osarcoma Med Oral Patol Oral Cir Bucal 2 Oral osarcoma Oral osarcoma Med Oral Patol Oral Cir Bucal 2 Oral osarcoma Primary leiomyosarcoma of the mandible. A case report João de Jesus Viana Pinheiro , Edson Okuda , Waldir Antonio Jorge , Ruy Gastaldoni Jaeger Ney Soares de Araújo (1) DDS, PhD, Universitary )ospital João de Barros Barreto, Department of Oral Pathology, School of Dentistry, Federal University of Parà (2) DDS., MS. Department of Estomatology, School of Dentistry, University Center of Parà (3) DDS, Oral and Maxillofacial Surgery Clinic, São Paulo Tatuapé )ospital (4) DDS, PhD, Department of Stomatology, School of Dentistry, São Paulo University (5) DDS, PhD, Department of )istology and Embryology, Institute of Biomedical Sciences, São Paulo University Xas useful in making the final diagnosis. A 4yearold Xoman Xas referred to the Stomatology Clinic of São Paulo Tatuapé )ospital, , for evaluation of a lesion on the left side of the mandible. This lesion presented a fast groXth in the last 6 months. Intraoral examination shoXed a firm, fixed, red colored mass measuring, approximately 6mm in diameter. No lymph nodes involvement Xas found. The radiographic examination shoXed a lytic lesion shoXed illdemarcated radiolucent Xith facial and lingual cortical bone destruction. Microscopic examination of the mandibular lesion shoXed a neoplasm composed by interlacing fascicles of spindleshaped cells. Most of the cells presented a blutended elongated shape. A marked cellular pleomor in older adult population and most often arises in the uterus, gastrointestinal tract, and the retroperitonial region(1). Women are more affected than men because of the uterine association. The prognosis is poor, Xith a hight percentage of recurrence or metastasis. The most common sites of me tastasis include the lungs, bone, brain and the lymph node. This neoplasm, is a extremely rare mesenchymal lesion in the oral cavity, Xith unusual bone location because of paucity of smooth muscle in that site(2). )oXever, the definitive histologic diagnosis of leiomyosarcoma is difficult because of the similarity Xith other spindlecell sarcomas(1). The Med Oral Patol Oral Cir Bucal 2 Oral osarcoma Med Oral Patol Oral Cir Bucal 2 Oral osarcoma Med Oral Patol Oral Cir Bucal 2 Oral osarcoma Med Oral Patol Oral Cir Bucal 2 Oral osarcoma The leiomyosarcoma has been divided into three main groups based on geographic occurrence: 1) retroperitoneal and intra�

0E;abdominal 2) cutaneous and subcutaneous and 3) vascular. Each group has certain clinical and biological differences, althought, they are histologically similar. The site of the leiomyosarcoma is more important for prognosis than its histological features. Those arising in a superficial cutaneous locali[ation have the best prognosis (1). Intraoral leiomyosarcoma occurs predominantly in males Xith a ratio of 2:1 and may present at any age, (2) ranging from 1 to  years (35). The mean age of the 1 previously described patients Xith leiomyosarcoma arising from jaX bone Xas 43. years (6). The patient of the present case is a Xoman of 4 years Xith a primary lesion at the mandible. Farman () found only 5 oral cases among ,4 benign smoothmuscle tumors of the Xhole body. In a revieX of the literature, in 1, by Farman and ,ey (), 11 cases of primary leiomyosacomas in the jaXs or in the oral cavity Xere located. A total of 3 cases of primary leiomyosarco ma in the oral cavity have been described during the last 5 years (6, 13). The intraoral locations of these tumors Xere as folloXs: cheek (5), mandible (), gingiva (4), maxilla (4), floor of the mouth (3), tongue (3), soft palate (2), hard palate mucosa (1) (, 13). In the oral cavity, the jaX bones have been the most prevalent location for this tumor, accounting for 45 of all reported cases. Including the current case, only  cases have been reported in the mandible (, 13). There is no distinct clinical feature that may suggest the presence of oral leiomyosarco ma. These tumors can appear as a painless, Xellcircunscri bed mass, firmly adherent to the surrounding tissues (3). )istopathologically, this neoplasm is characteri[ed by sheets of sXeeping, alternating bundles and fascicles of densely packed spindle cells Xith abundant fibrillar eosinophilic cytoplasm and indisctinct cytoplasmic borders. The nucleus is usualy centrally located and bluntended, sRuaredoff or cigarshaped. Occasional cells have perinuclear vacuoles (1,,14,15). In some cases, nuclear hyperchromasia, and pleomorphism are pronounced. Both typical and atypical mitosis, as feX as one or as many as 2 per highpoXer field populate the tumor (,11). The diagnosis of soft tissue tumors of mesenchymal origin can be very difficult and necessitates differentiation from a number of other tumors (16,1). Massons trichrome staining and immuno histochemial evaluation for muscle antigens are helpful in diagnosis separating leiomyosarcoma from other sarcomas (1). Positive reacti

ons for desmin, vimentin, smoothmus cle actin and hcaldesmon have been demonstrated in this The case presented fulfilled these criteria and confirmed the diagnosis of leiomyosarcoma. Surgical resection, hemimandibullectomy or hemimaxillec tomy, partial maxillectomy, are the prefered initial treat ments for cases of leiomyosarcoma primary in the jaXbones (15, 13). Regional lymph node investigation is recommended if there is clinical evidence of lymphadenopathy (21). A hemimandibullectomy Xas the treatment of the choice in the present case. The patient has been folloXing by 11 months and no local recurrence has been observed. The cervical and sumandibular regions remain free of palpable lymph nodes. Systemic examinations revealed no other site affected by this malignant neoplasm. To summari[e our report, Xe should emphasi[e the impor tance of an early diagnosis on the survival of the patient. This diagnosis can only be achieved if a representative fragment of the lesion is obtained. In addition, immunohis tochemistry Xas very important to establish a final diagnosis of leiomyosarcoma. 1. En[inger FM, Weis SW, eds. Soft Tissue Tumors: St. -ouis, MO, Mosby :ear Book Inc 15. p. 4151. 2. Schenberg ME, SlootXeg PJ, ,oole R. -eiomyosarcoma of the oral cavity. Report of four cases and revieX of the literature. J Craniomaxillofac Surg 1321:342. 3. I[umi ,, Maeda T, Cheng J, Saku T. Primary leiomyosarcoma of the maxila Xith regional lymph node metastasis: Report of a case and revieX of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 4. Freedman PD, Jones AC, ,erpel SM. Epithelioid leiomyosarcoma of the oral cavity: Report of tXo cases and revieX of the literature. J Oral Maxillofac Surg 1351:232. 5. )aedicke G, ,aban -B. Smoothmuscle tumors of the oral cavity. Plast Reconstr Surg 11:264. 6. Carter -C, Aguirre A, Boyd B, De-acure MD. Primary leiomyosarcoma of the mandible: Report of a case and revieX of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1:44. . Farman AG. Benign smoothmuscle tumours. S Afr Med J 15 . Farman AG, ,ey S. Oral leiomyosarcoma: Report of a case and revieX of the pertainnig to smoothmuscle tumors of the oral cavity. Oral Surg Oral Med Oral Pathol 143:42. . Bass B, Archard ), Sussman R, Stern M. Saunders V. Clinicopathologic conferences, case 62: expansile radiolucent lesion of the mandible. J Oral Maxillofac Su

rg 1644:3. 1. Nishi M, Mimura T, Senba I. -eiomiosarcoma of the maxila. J Oral Maxillofac Surg 145:64. 11. Poon C,, ,Xan PC, :in NT. -eiomyosarcoma of gingiva: Report of a case and revieX of the lierature. J Oral Maxillofac Surg 145:2. 12. Mindel RS, Calcaterra TC, Ward P). -eiomyosarcoma of the head and neck: A revieX of the literature and report of tXo cases. -aryngoscope 13. ,arlis V, ;asloX M, MinkoXit[ G, Glickman RS. -eiomyosarcoma of the mandible: Report of a case and literature revieX. J Oral Maxillofac Surg 1654:1123. 14. Allen CM, Neville B, Damm DD, Marsh W. -eiomyosarcoma me tastatic to oral region: Report of three cases. Oral Surg Oral Med Oral Pathol 136:526. 15. ,ratochvil FJ, MacGregor SD, Budnick SD, )eXan-oXe ,, Allsup ). -eiomyosarcoma of the maxila: Report of a case and revieX of the literature. Oral Surg Oral Med Oral Pathol 1254:6455. 16. -loriaBenet M, Bagan JV, -loria de Miguel E, BorjaMorant A, Alonso S. Oral leiomyoma: a case report. Med Oral 23:215. 1. Sanche[ Jimene[ J, Dean Ferrer A, Alamillos Granados F, Rui[ Ma sera JJ, Villar Pastor C, Garcia -ope[ A, Penalba Manegold M. Adult rhabdomyoma in the masticatory area. NeX case presentation and revieX of the literature. Med Oral 216:64. 1. -ack EE. Primary leiomyosarcoma of adrenal gland. Case report Xith immunohistochemical and ultrastructural study. Am J Surg Pathol 1. -o Mu[io -, Favia G, Mignogna MD, Piattelli A, Maiorano E. Primary intraoral leiomyosarcoma of the tongue: An immunohistochemical study and revieX of the literature. Oral Oncol 236:5124. Med Oral Patol Oral Cir Bucal 2 Oral osarcoma Med Oral Patol Oral Cir Bucal 2 Oral osarcoma Med Oral Patol Oral Cir Bucal 2 Oral osarcoma Med Oral Patol Oral Cir Bucal 2 Oral osarcoma Medicina Oral S.- . Email: medicina!medicinaoral.com 2. MesRuita RA, Migliari DA, De Sou[a SOM, Alves MR. -eiomyo sarcoma of the buccal mucosa: A case report. J Oral Maxillofac Surg 21. Watanabe ,, ,usakabe T, )oshi N, Saito A, Su[uki T. hCaldesmon in leiomyosarcoma and tumours Xith smooth muscle celllike differentia tion: its specific expression in the smooth muscle cell tumor. )um Pathol