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Extraskeletal Ewing sarcoma, a high-grade soft tissue tumor, is extremely rare in the stomatognathic region. We report the case of a 47-year-old man with probable extraosseous Ewing sarcoma of the maxillary gingiva that occurred concurrently during chemoradiotherapy for non-small cell lung cancer. Owing to the rapid growth of the mass, debulking surgery was performed for the suspected round cell tumor under intravenous anesthesia. The tumor was considered as having extraosseous Ewing sarcoma showing unusual features based on the following features: it was not continuous with the maxilla; histopathological examination revealed proliferation of small round cells with pleomorphic and voluminous round nuclei in the fibrous connective tissue; tumor cells were immunohistochemically positive for CD99 and ETS-related gene (ERG); and fluorescence in situ hybridization analysis showed EWSR1 gene translocation. Despite satisfactory postoperative recovery, the patient died of respiratory failure due to rapid lung cancer deterioration on postoperative day 16. To our knowledge, in the English literature, there are only 17 reported cases of extraskeletal Ewing sarcoma in the stomatognathic region, and this is the only one with concurrent other organ cancer. More data on cases of extraskeletal Ewing sarcoma in the oral region are needed to establish its pathophysiology, prognosis, and treatment guidelines. [ABSTRACT FROM AUTHOR]

Introduction Localized amyloidosis in the head and neck is a rare and generally benign condition. In the oral cavity, amyloidosis usually involves the tongue or buccal mucosa. We present the second case of oral amyloidosis arising in the gingiva ever reported, to the best of our knowledge. Case presentation A 73-year-old White Spanish man presented a persistent nodular mass involving his upper gingiva. The lesion was surgically resected and the histological examination revealed a subepithelial, multinodular amorphous and fibrillar accumulation. Staining of the specimen for Congo red proved positive, exhibiting a reddish colour under light microscopy and applegreen birefringence under polarized light. With immunohistochemical tests, pentagonal amyloid component was demonstrated. An extensive study excluded any systemic involvement; a diagnosis of localized primary amyloidosis was made. After 2 years of follow-up, no clinical progression to systemic amyloidosis or local recurrence was observed. Conclusions Localized amyloidosis of the gingiva is an extremely rare condition that seems to show no clinically distinct feature. Histologic examination is the first step towards diagnosis, followed by immunohistochemical tests. The diagnosis of localized amyloidosis should always be integrated with blood tests, a bone marrow biopsy, echocardiography and digestive endoscopy to intercept systemic involvement. [ABSTRACT FROM AUTHOR]

Leiomyosarcoma of the oral cavity is a very rare tumor type associated with aggressive clinical behavior and a low survival rate. However, this tumor may also be encountered as a slow-growing, non-ulcerated, painless mass in its early stage. We report a case of leiomyosarcoma, affecting the gingival mucosa of a 24-year-old male. The tumor mimicked an epulis, because it was encountered as a slow-growing, discreet, firm, non-ulcerated, painless mass without any evidence of resorption of the maxillary bone. Excision of the "epulis" resulted in a histological diagnosis of leiomyosarcoma. The histological findings of a wider excision did not indicate the presence of any tumor cells. The patient has since remained tumor-free for 3 years. A literature review, related to the gingiva of the maxilla or mandible, indicated only a few cases wherein the tumor behavior was similarly mild and benign as it was in this case. Thus, when the tumor is diagnosed at an early stage, while it is small and non-aggressive, successful treatment can lead to a good prognosis. [ABSTRACT FROM AUTHOR]

Summary: Squamous cell carcinoma of the superior gingival–buccal complex are rare and few English-language data have been published on their biological behaviour. Reported in this paper are the clinical behaviour and treatment outcomes of squamous cell carcinoma of the upper gingival–buccal complex. We reviewed the charts of 110 patients with squamous cell carcinoma restricted to the upper gingiva, superior gingival–buccal sulcus and adjoining buccal mucosa, seen between 1997 and 2001. Separate outcome analyses were carried out among 86 patients who had undergone surgery, and 24 patients treated by radiotherapy or chemo-radiation. Disease-free survival at 2 and 5 years was 48.9% and 36%, respectively, and was independent of epicentre of disease. Five-year, disease-free survival was 48.8% and 0% for surgical treatment and non-surgical treatment groups. T stage (p =0.024) and extra-capsular spread of disease (p =0.036) were independent predictors of disease-free survival on multivariate analysis. Adequate surgical resection and adjuvant treatment, in the first instance, offers the best chance of disease control. [Copyright &y& Elsevier]

Amelobasltoma is a benign neoplasm of the jaw bones that originate from the odontogenic epithelium. They are more common on the mandible than the maxilla. Rarely such tumours arise outside these bones, when they are termed extraosseous or peripheral ameloblastoma. We report a case of extraosseous ameloblastoma in a 30 year old woman, who presented with a painless lesion on the upper gingiva. The lesion was excised completely and the histopathology was suggestive of extraosseous ameloblastoma. [ABSTRACT FROM AUTHOR]