The authors present a case of a rare highly malignant condition that initially appeared clinically and histologically to be the relatively common and benign condition necrotizing gingivitis. Conditions that do not follow the expected clinical course mandate further investigation because rare malignant disease is not foremost in the mind of dentists and oral and maxillofacial surgeons.
Soft tissue sarcomas represent less than 1% of malignant tumours and little is known about their aetiology or incidence . Angiosarcoma is a rare, highly malignant soft tissue sarcoma. Its occurrence in the oral cavity is extremely rare, comprising only 1–2% of all angiosarcomas. This tumour is composed of malignant cells with many of the functional and morphological properties of normal vessels and endothelium and it presents difficult diagnostic and therapeutic problems . The clinical presentations share aggressive behaviour, leading to a short survival time with a less than 12% 5-year overall survival rate.
The authors describe a patient with an angiosarcoma involving primarily the gingiva, which initially led to the microscopic and clinical misdiagnose of acute necrotizing ulcerative gingivitis. This case illustrates the difficulty of establishing a definitive histological and clinical diagnosis of oral angiosarcoma.
A 76-year-old white man presented with a 2-month history of persistent ulceration of the buccal aspect of the upper right lateral incisor, first molar and lower right second premolar teeth. Examination revealed ulceration with central necrosis ( Fig. 1 A ). The management included biopsies, oral penicillin and metronidazole in addition to oral hygiene instruction and rinsing with chlorhexidine. A course of periodontal treatment was completed that included regular scaling and root planning. The histologically proven diagnosis was that of necrotizing gingivitis. Despite further treatment, the ulcerating and necrotic lesions were progressive, affecting multiple sites of the maxilla and mandible. After 2 months of unsuccessful treatment, the patient was referred to the authors’ department. A dental panoramic radiograph showed bone loss in both the maxilla and mandible ( Fig. 1 B). The disease extended from the upper right second molar to the midline of the maxilla, from the upper left canine to the upper left first molar and from the lower right lateral incisor to the lower right first molar. Clinical signs at presentation were halitosis and deep, ulcerated, painful, necrotic gingivae with localized bleeding and signs of infection. There was bilateral cervical lymphadenopathy. Testing for HIV, hepatitis B and C, classical and protoplasmic anti-neutrophil cytoplasmic antibody tests were negative. The patient had no other signs or symptoms. A week later, the periodontal soft tissue showed extensive recession and ulceration with localized bleeding ( Fig. 2 ).
There was concern that the normal clinical course of the preliminary (and histologically confirmed) diagnosis was not being followed, so biopsies were taken from the gingival mucosa lesions at multiple sites. The histological features demonstrated atypical cells, haemorrhage and numerous well-formed vascular spaces lined with endothelial cells ( Fig. 3 A ). Immunohistochemical staining demonstrated reactivity of tumour cells to CD31, but not to CD34, CD45, CD68, CK18, CK-HMW, S-100, KP-1, MPO, or factor VIII-related antigen ( Fig. 3 B). Lymphoepithelial lesions were not detected in the routinely stained section. The characteristic pathological findings of lymphoma follicular colonization were not found. Features such as endothelial atypia, mitotic activity, collagen dissection, and infiltrative growth pattern differentiated the tumour from benign lesions. Lack of cytoplasmic hyaline globules, spindled cells with distinctive cytoplasmic borders, and scattered plasma cells distinguished Kaposi’s sarcoma from angiosarcoma. The vascular markers separated angiosarcoma from carcinoma and melanoma, although keratins may be observed in epithelioid endothelial tumours. A diagnosis of primary multifocal gingival angiosarcoma was made, as whole body staging revealed no other tumour location. The immunohistochemical staining and the histological type of the tumour indicated a high-grade endothelial angiosarcoma with solid and papillary areas. Magnetic resonance imaging (MRI) of the head and neck, with and without gadolinium enhancement, showed a diffuse oedematous swelling of the right lateral pterygoid muscle and a mass around the alveolar arch of the maxilla and mandible extending into the right pterygopalatine fossa. There was suspicious cervical lymphadenopathy by imaging criteria. Resection of the tumour by lower maxillectomy and marginal mandibulectomy, combined with neck dissection was performed. Owing to the growth pattern of the angiosarcoma the resection margins were profusely involved in the surgical field after tumour resection. The findings suggested preoperatively by the MRI could not be confirmed at the site of operation and the surgeon decided not to extend the resection. Histology confirmed the diagnosis of an angiosarcoma with two lymph node metastases confirmed by two independent histopathological centres. Postoperative non-surgical treatment was planned with a combination of radiation (70 Gy) and chemotherapy with Paclitaxel intravenously at a dose of 80 mg/m 2 . 3 weeks later, another MRI, carried out to plan the radiation fields, revealed extension of the tumour to the sphenoid bone and in the direction of the foramen rotundum. The patient started both therapies, but only radiation therapy was performed after the first administration of Paclitaxel because of the toxic side effects of the chemotherapy and the patient’s rapidly declining general condition. 6 months later, the patient died. Permission for an autopsy was denied.