Angiosarcoma of the nasal cavity: a case report
© Treviño-González et al; licensee BioMed Central Ltd. 2009
Received: 29 September 2008
Accepted: 29 January 2009
Published: 29 January 2009
Angiosarcomas are malignant neoplasias of rapid growth that develop from endothelial cells. They represent 2% of all sarcomas and only 1–4% are located in the aerodigestive tract. Since 1977, only 16 cases have been reported.
We present a 33-year-old male with spontaneous epistaxis that was refractory to cauterization. During physical examination, a smooth purplish tumor of 1.5 cm × 1.5 cm was identified. A CT scan showed a nonenhanced tumor in the left nostril on the uncinate process. A biopsy revealed an intermediate-grade angiosarcoma. Surgical removal followed by radiation therapy was performed with good result. Aerodigestive angiosarcomas have a better prognosis than angiosarcomas of other locations due to better cell differentiation and the presence of early symptoms. Recurrence can occur because of tumor tissue left during resection. Our patient continues tumor free after three years.
Angiosarcomas are malignant neoplasias of rapid growth that originate from endothelial cells [1, 2]. They represent 2% of all sarcomas, and of these, only 1–4% are localized in the upper aerodigestive mucosa [3, 4].
They appear during middle age and prognosis depends on location, size and degree of tissue invasion.
We present a case of angiosarcoma of the nasal cavity
Resection with maxillary antrostomy, ethmoidectomy, frontal and ipsilateral sphenoidotomy was performed. Free margins were reported on the surgical specimen. Thirty radiation cycles were applied until a total dose of 55 Gy was achieved.
Reported angiosarcoma cases, 1974–2008
Time of evolution before diagnosis
Dysphagia and dysphonía
Total pharyngolaryngectomy + RT
Maxillectomy + RT
Maxillectomy + Rhinotomy
7.5 × 4.5 cm
pharyngopalatectomy + RT
Pain and malar edema
RT + Maxillectomy
Epistaxis, pain and diplopia
Maxillectomy + RT
molar pain and maxillary edema
Maxillectomy + RT
Maxilla and maxillary sinus
5 × 3 cm
Intraoral mass and hemorrhage
Hemimaxillectomy + RT
4 × 2 cm
RT + Maxillectomy
5 × 4 cm
Cephalalgia and epiphora
Cranial-facial Resection + RT
Recombinant IL-2 + Rhinotomy + PO RT
Ucinate process and lateral nasal wall resection, partial turbinectomy of the middle turbinate + RT
We did not find any accurate report of angiosarcoma in the nasal cavity after a review of the literature.
Nasal obstruction, malar edema and recurrent epistaxis are usually present at an early phase of the disease. The period of time for the presence of symptoms of angiosarcomas of the head and neck is short, usually one month . This contrasts with angiosarcomas of the nasal cavity and paranasal sinus which is usually 7.1 months . Our patient presented with a 5 month history of these symptoms.
The histology of aerodigestive angiosarcomas is similar to that of angiosarcomas at other locations. It is characterized by multiple vascular anastomosis, sometimes having solid necrotic or hemorrhagic areas, particularly in high-grade tumors. The neoplastic vessels present atypical endothelium with prominent hyperchromatic nuclei and can be classified in high or low grade based on their microscopic appearance 
Well-differentiated tumors present vascular channels with anastomosis, endothelial cells with large hyperchromatic nuclei, a low mitotic index and few pseudopapillary projections into the vessel lumen contrary to poorly-differentiated neoplasias, which are characterized by solid areas with fusiform cells [10, 11]
Diagnosis of angiosarcoma is established by pathological examination of the biopsy using H&E. However, immunohistochemical staining with CD34, Ulex europaeus agglutinin I and factor VIII antigen is often required. Factor VIII-like antigen is synthesized in vascular endothelial cells and CD34 is specifically stained in vascular endothelial cells [12–14].
Radical surgery with ample margins is the treatment of choice in patients with head and neck angiosarcoma.
There is no standard treatment for nasal cavity and paranasal sinus angiosarcoma. Based on a literature review, 97% of studies reported wide resection with free margins followed by radiotherapy (4000–5000 rads) as the treatment of choice. Chemotherapy has a low impact on the disease. Kazuto Fukushima et al. reported favorable results with the use of recombinant interleukin 2, combined with surgery 
Angiosarcoma of the skin or soft tissue of the head and neck is associated with a 50% mortality rate within the first 25 months and a 12% survival rate at 5 years, compared to nasal cavity or paranasal sinus angiosarcoma, which have a 22% survival rate at 5 years according to grade of differentiation and early diagnosis 
A literature review reported a 20.8 month survival rate. Cervical metastasis occurs in 10–15% of cases and distal metastasis to bone, liver, lungs or skin occurs in 30% of patients during the first 24 months of the disease.
Radical surgery followed by radiotherapy is still the treatment of choice. Due to the lack of experience with these tumors, we believe that a better approach should be sought in order to unify criteria and provide patients with better results.
"Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal."
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