Angiosarcoma of the nasal septum mimicking reticulohistiocytoma.

Angiosarcomas are rare, aggressive tumors of endothelial cells with a high degree of invasiveness and poor survival. Although they arise in the face and scalp of elderly people, the nose represents a rare location with few reports in the literature. Nasal angiosarcoma resembling benign lesion morphologically has been described, but there is no report of angiosarcoma mimicking benign lesion histologically.Here, we report a case of nasal septum angiosarcoma in which the initial misdiagnosis submitted by the referring pathologist was reticulohistiocytoma. Nevertheless, the nasal septum and anterior nasal spine invasion by the tumor led us to suggest extensive surgical treatment: resection of the caudal septum, the anterior nasal spine, the columella, and the philtrum. Thereafter, an L-strut rib cartilage graft reconstructed the septum defect and was lined with a free radial forearm flap, resulting in a satisfactory functional and aesthetic outcome. Histology showed complete resection of a malignant neoplasm of mesenchymal origin, and immunohistochemistry established the diagnosis of epithelial angiosarcoma. The literature regarding this rare presentation of angiosarcoma was reviewed.

Mucoepidermoid carcinoma of the salivary glands. Review of the literature and clinicopathological analysis of 18 patients.

Salivary gland carcinomas are a rare and clinically diverse group of neoplasms among which mucoepidermoid carcinomas (MEC) are reported to be the most frequently encountered. During the years 1994-2004 18 patients with MEC were treated in our Department. All patients underwent surgery with a curative intent, and in 11 of them treatment was supplemented by radiotherapy. Follow up ranged from 6 to 120 months. Twelve (66.6%) MECs originated from the major salivary glands with the majority located in the parotid. Histologically, 50% of tumors were classified as low grade, 28% as intermediate and 22% as high-grade MECs. Positive surgical margins were documented in six cases (33%) and all in tumors of high or intermediate histological grade. All these patients received adjuvant radiotherapy and one developed local recurrence. Local recurrence occurred in two more patients with histologically free margins. Both received postoperative radiotherapy. Distant metastases were documented in four patients all between 14 and 24 months after surgical treatment. An association between local recurrence and distant metastasis might be suggested since all patients with local recurrences subsequently developed distant metastases. The 5-year overall disease specific survival rate was 85%. Statistical multivariate analysis demonstrated that the factor that significantly correlated with overall survival was the histological grade of tumors (Log Rank test: p=0.013). A trend towards poorer survival was observed in patients aged over 50 years. Our results also suggested a potential benefit of postoperative radiotherapy for patients with positive margins.

Leiomyosarcomas of the oral tissues: clinicopathologic analysis of 50 cases.

PURPOSE: Primary oral leiomyosarcomas are rare tumors. Information regarding the biological behavior, prognosis, and appropriate management of this neoplasm is lacking in the literature. The purpose of this report was to summarize the data of isolated case reports of primary oral leiomyosarcoma that have been published in the English literature during the past 25 years. The cases of 4 additional new patients who have been treated in our department during the past 10 years are also presented. PATIENTS AND METHODS: The data for 46 patients obtained from 32 individual articles retrieved from the English literature were added to our 4 cases and produced a total number of 50 cases of primary leiomyosarcomas of the oral tissues. Patients were analyzed according to demographic data, anatomic location, type of treatment, and survival. RESULTS: Primary oral leiomyosarcoma may affect any age with peaks of occurrence in the third, sixth, and seventh decades of life. There is no gender predilection. Female patients presented the higher incidence in the third decade, whereas males had an even age distribution. The tumor arises in approximately 70% of the cases in the maxillary and mandibular bones. Radical surgery was the treatment of choice. Radiotherapy and chemotherapy when applied in recurrent tumors had little effect. The most adverse prognostic factor was the positive surgical margins. The 5-year survival was 62% (62.9% for females and 52.6% for males, P > .1968). CONCLUSIONS: Cases of oral leiomyosarcoma appear to be associated with major neurovascular structures of the facial skeleton, as evidenced from the imaging studies of our 4 patients. Primary oral leiomyosarcoma is a rare tumor that should be managed with aggressive surgical resection in order to safeguard curability. Histopathologic diagnosis is greatly facilitated with positive immunohistochemical staining for smooth muscle antigenic markers.

Tumors of the submandibular gland: clinicopathologic analysis of 23 patients.

PURPOSE: Tumors of the submandibular gland are rare, comprising less than 2% of head and neck neoplasia. Both benign and malignant lesions show a mild symptomatology, resulting in late presentation and in advanced stage of disease. The purpose of this article was to report our experience in treating submandibular gland neoplasia during the last 10 years. PATIENTS AND METHODS: The medical records of all patients with histologically confirmed epithelial tumors of the submandibular gland were retrospectively reviewed. This review found 23 patients with 9 benign and 14 malignant tumors. The collection of data included demographic data, diagnostic procedures, operative and pathology reports, complications, additional treatment, and follow-up. RESULTS: There were 10 men and 13 women with a mean age of 60 years. Pleomorphic adenoma was the most frequently encountered benign tumor; adenocarcinoma and adenoid cystic carcinoma had an equal presentation in the malignant group of patients. Eleven of the 14 patients with malignant tumors presented in advanced stages of disease (stage III and IV). Surgery was the sole treatment for the benign tumors. There were no recurrences. In the majority of cases, patients with malignant tumors were treated with surgery and postoperative radiotherapy. Eight patients died during the follow-up period, giving a mortality rate of 61.5%. CONCLUSION: Benign submandibular gland tumors manifest a mild course of disease, and local excision along with the gland is a safe and effective method of treatment. Malignant tumors have a poor symptomatology that results in late diagnosis. Radical surgery and postoperative radiotherapy is the treatment of choice. Prognosis depends on the histopathology and biologic behavior of the specific type of malignant tumor.