Stylianos N Zanakis1*, Vasileios Lyris2, Sotirios Kyriakou1, George Agrogiannis3 and Penelope Korkolopoulou3
1Oral and Maxillofacial Surgeon, Department of Oral and Maxillofacial Surgery, “Hippokration” General Hospital of Athens, Athens 11527, Greece
2Dentist, Private practice, Greece
3Pathologist, Department of Pathology, School of Medicine National and Kapodistrian University of Athens, Greece
Received: 20 February, 2016; Accepted: 29 February, 2016; Published: 03 March, 2016
Dr. Stylianos N Zanakis, Hippokration General Hospital of Athens, 114Vas. Sofias Ave. Athens 115 27, Greece, Tel: +30- 213-208-8000; Fax: +30- 213-208-8000; E-mail:
Zanakis SN, Lyris V, KyriakouS, Agrogiannis G, Korkolopoulou P (2016) Primary Leiomyosarcoma of the Mandibular Gingiva. Glob J Cancer Ther 2(1): 001-004.
© 2016 Zanakis SN, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Leiomyosarcoma; Primary; Oral cavity
Primary oral leiomyosarcomas are exceptionally rare lesions often associated with poor prognosis. We report a case of leiomyosarcoma arising in the anterior mandibular gingiva, which is a non-prevalent site of occurrence. Clinically, the tumor was far from being firm and solid as it is usually described in the literature. Light microscopy failed to confirm the true nature of the lesion on the biopsy specimen.
Benign smooth muscle tumors, or leiomyomas, are common, well-circumscribed neoplasms that can arise from smooth muscle cells anywhere in the body, but are encountered most commonly in the uterus . Leiomyosarcomas (LMS), theirs malignant counterpart, comprise 7% of head and neck sarcomas . Primary LMSs rarely occur in the oral and maxillofacial region, probably due to paucity of smooth muscle tissue in the area [1,3]. Source of smooth muscle in the oral cavity may be the arterial tunica media, lingual duct, circumvallate papillae and pluripotential mesenchymal cells . We present a case of oral LMS which exhibits an uncommon clinical appearance in a non-prevalent site and which also presents difficulties in the histopathologic diagnosis of the incisional biopsy.
An 81-year-old Caucasian female attended the outpatient department, complaining of a painless mass in the region of her anterior mandibular ridge, causing difficulty in mastication. On examination the mass measured 4x3x3cm, was bluish-red in color, soft and friable in consistency and boss elated in appearance. The lesion was broad-based and firmly attached to the underlying tissues. The patient was partially edentulous and the only teeth remaining in the arch, were the 31,32,33. The three teeth were embedded in the mass with a grade 4 mobility. Extra orally, there were no clinical signs of cervical lymphadenopathy. The orthopantomogram was negative for bone lesion. Past medical history included a partial colectomy for an adenocarcinoma 10 months earlier. The clinical impression was that of an exophytic squamous cell carcinoma arising from the dentoalveolar mucous membrane. An incisional biopsy was carried out and the histopathology report indicated a pseudosarcomatous lesion. The lesion was excised along with the teeth and the basal bone of the mandible was curetted (Figure 1). The histopathology report was a surprise, as indicated a leiomyosarcoma. The margins were free but the under surface of the lesion was infiltrating the bone. A complete workup followed, consisting of CT scans (face, neck, chest), upper and lower abdomen ultra sound and a skeletal scanning. The investigation proved negative for lesions elsewhere in the body. Consequently, the patient underwent a wider excision and a peripheral mandibulectomy, preserving the continuity of the mandible, since a partial mandibulectomy was denied by the patient. A split thickness skin graft was used to cover the inner surface of the lower lip and the anterior floor of the mouth. The post-operative period was uneventful. The histopathology report indicated both free soft (confirming the previous report) and hard tissue margins. The surgical ablation was followed by radiotherapy in another institution and according to their files the patient received 66 gray (Gy) locally in 33 fragments and 52 Gy in the neck in 26 fragments. Six months postoperatively the patient presented with osteoradionecrosis of the jaw, a pathologic fracture of the mandibular symphysis and edema of the lower lip. At that time, biopsies were taken, with negative results. The patient refused admission as well as the scheduled hyperbaric oxygen therapy; simultaneously, underwent a complete CT workout (brain, face, neck, chest, upper and lower abdomen), that was negative for pathology (LMS or colon adenocarcinoma). Two months later the patient admitted urgently in the hospital for an ischaemic necrosis in the lower face and upper neck region present for 15 days (Figure 2). On admission some basic laboratory parameters were as follows: White blood count 43.758, neutrophils 96,6%, urea 129 mg/dl, creatinine 1,9 mg/dl, C Reactive Protein 195,6 mg/dl). The patient’s health deteriorated rapidly by developing renal and respiratory insufficiency, and died 5 days after admission.
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