Diffuse epithelioid angiosarcoma of the oral cavity: clinical, radiological and immunohistochemical presentation of a case

• ¹ University of Bari Aldo Moro, Interdisciplinary Department of Medicine, Italy

Aim. Angiosarcomas are extremely rare tumors arising in vascular endothelium. They comprise only 1-2% of soft tissues sarcomas and usually occur in the skin and subcutis, or in visceral sites such as breast, liver and great vessels. Metastatic spread via bloodstream at the time of diagnosis is common.Epithelioid angiosarcoma (EA) is a poorly differentiated morphological variant of this sarcoma, with a histological carcinoma-like aspect. Thirty-three percent out of all angiosarcomas are EA. The literature reports about 1% of angiosarcomas arising in the mouth and in rare cases are a metastatic manifestation. To date about 30 cases of primary oral angiosarcomas are reported. Metastatic involvement of the lung (90%) is more common than primary pulmonary involvement. In the literature at least 3 cases of primary maxillary angiosarcomas with pulmonary metastasis were recorded. Nevertheless, there are no radiological and immunohistochemical differences between PPA and metastatic angiosarcoma of the lung. Materials and Methods. A 46 years-old female patient referred to the Department of Oral Medicine of the Policlinico in Bari, in February 2018, for investigation and management of multifocal gingival and mucosal lumps. The patient had first noticed swellings during November 2017, which quickly enlarged and increased in number. Her recent past medical history revealed the presence of respiratory distress linked to a suspect pulmonary tumor. In December 2017 she got a thoracoabdominal CT scan, that showed multiple masses scattered all over the right pulmonary ileum-mediastinum and in the lower lobe of the lung. Abnormal regional lymph nodes were also displayed by the CT. A bronchoscopy with transbronchial biopsy was performed. This exam revealed a histological diagnosis of "undifferentiated carcinoma of the lung/possible adenocarcinoma". At the time of our first visit, the patient was pale, asthenic, hyper-pyretic and in weight loss. Intra-oral examination revealed soft, red-bluish exophytic masses localized on the premolars regions gingiva and on the hard and soft palate. The neoplasms with bleeding tendency caused dysphagia and hindered oral hygiene practices. Full laboratory investigations including complete blood count (CBC), assessment of liver and renal functions, EKG, HIV-test, OPG X-Ray, and head and neck CT scan were requested. Kaposi Sarcoma, haematological malignancies, oral melanoma, sarcomas and metastatic localization of other neoplasms were considered in differential diagnosis. HIV- test was negative, OPG X-Ray did not show pathological signs, while the CT scan evidenced multiple nodular lesions bordering the right maxillary sinus floor, and multiple lymphadenopathies in submandibular, deep-superficial lateral cervical and bilateral supraclavicular regions. CBC revealed normocytic anaemia (Hgb=8.7 g/dl) and neutrophilic leucocytosis (WBC=21,11 x103/uL). The haematological check excluded a diagnosis of leukaemia and other blood neoplasms. Blood transfusion was given in order to perform the biopsy of the oral lesions. Results. Two different oral lesions were biopsied in order to obtain a definitive diagnosis. The histological exam revealed a malignant tumor with epithelioid cells characterized by strong pleomorphism and abnormal vasoformative growth pattern. Immunohistochemical investigations showed immunoreactivity for CD31, CD10, VIII Factor, R-Ag, FLI-1, while S-100, CD45, TTF-1, Melan-A, HMB45, EMA, CD56, CD34, CD30, AE1/AE3 CKs, 7CK, synaptophysin and chromogranin were negative. A diagnosis of Epithelioid Angiosarcoma was provided and the revised histological pulmonary exam agreed with our diagnosis. The patient underwent to weekly chemotherapy treatment at the Oncology Department in Bari, according to the current protocols. To date, the patient is still receiving the polychemotherapy. Discussion. The present study reports a rare case of oral EA and its complex diagnostic pathway. The angiosarcomas’ clinical, radiological and histological resemblance to other neoplasms, makes the immunohistochemical exam mandatory, in order to provide a correct diagnosis. The prognosis of this tumor is poor: life expectance ranges from 6 months to 3 years. Debate exists about the elective treatment for this neoplasm. Polychemotherapy and radiotherapy are usually provided in association with the surgical treatment, when it is viable.