Primary temporal bone angiosarcoma: a case report

Abstract

SummaryWe present a rare case of temporal bone angiosarcoma diagnosed in a 26-year-old female patient at 36 week of pregnancy. The patient was referred with a 2 months history of left otalgia and tinnitus with a tender swelling above the mastoid. Cranial imaging studies showed a 7 × 5 × 4 cm hypervascularized mass located in the left middle fossa with lysis of the temporal bone and extension to the subcutis. After the baby was delivered by caesarean section, the patient entered the oncology protocol. Selective embolization of the feeding vessels was followed by gross total surgical resection using a combined supra-and infra-tentorial approach. Pathological findings were those of a poorly differentiated, highly malignant sarcoma with a large epitheloid component and immunohistochemical evidence of endothelial differentiation (CD31, Factor VIII related antigen, CD34), consistent with an angiosarcoma with epitheloid features. No extra-cranial tumor was found after extensive staging. The patient received adjuvant radiotherapy followed by a course of chemotherapy consisting of 6 cycles of paclitaxel. At 15 months follow-up, she developed multiple distant metastasis to a left postauricular lymph node and to the lungs and ribs. The patient was given a second line chemotherapy using doxorubicine and ifosfamide. Despite an initial good response, she died with metastatic disease 26 months after diagnosis. We present a rare case of primary temporal bone angiosarcoma and report our experience with a multimode therapeutic approach combining surgery, radiotherapy and chemotherapy.

Metastatic lung angiosarcoma from a femoral subperiosteal origin

“…At the time of diagnosis of an angiosarcoma originating in bone, either a single lesion or multifocal disease may be apparent [1][2][3][4][5][6]. Common symptoms include pain and swelling [1][2][3][4][5][6]. Advanced stage disease carries unfavourable prognosis as in our patient.…”

“…They account for 1-3 % of all sarcomas [1][2][3][4] and common sites of origin include the skin (33%) and deep soft tissues (24%). It also occurs in breast (8%), liver (8%), bone (6%), spleen (4%), heart, large vessels, and the pericardium (3%), eye sockets (3%), ear, nose, and throat (4%), and other organs including the lungs (7%) [1][2][3][4][5]. They may occur at any age but there is a preference for the third and fourth decades [1][2][3][4][5].…”

“…It also occurs in breast (8%), liver (8%), bone (6%), spleen (4%), heart, large vessels, and the pericardium (3%), eye sockets (3%), ear, nose, and throat (4%), and other organs including the lungs (7%) [1][2][3][4][5]. They may occur at any age but there is a preference for the third and fourth decades [1][2][3][4][5]. At the time of diagnosis of an angiosarcoma originating in bone, either a single lesion or multifocal disease may be apparent [1][2][3][4][5][6].…”

Subdural Hematoma Caused by Epithelioid Angiosarcoma Originating from the Skull

“…They are rare tumors accounting for less than 1 % of all sarcomas [9,10]. Primary skull angiosarcomas are particularly rare with less than 20 cases reported in the literature [12][13][14][15], which primarily explains why SDH caused by angiosarcomas is so rare. There seems to be a difference between SDH caused by angiosarcomas and that caused by other common cancers in the clinical presentation.…”

Angiosarcoma of skull base in a 1-year-old child—A case report

“…Angiosarcomas occur even more rarely in paediatric patients, representing 0.3% of paediatric sarcomas [3]. There is a reported overall annual incidence of approximately 2-3 cases per 1,000,000 population [4,5]. These tumours may occur throughout the body but most frequently involve the skin and superficial soft tissue, with half occurring in the head and neck [3,6].…”