Background

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

Case report

A 33-year-old male came to our clinic with a 5 month history of recurrent epistaxis of the left nostril described as nasal dripping, previously treated in another center on four occasions by cauterization of the left nasal mucosa with persistence of bleeding. During examination of the nose, we identified a smooth purplish tumor 1.5 cm × 1.5 cm attached to the lateral nasal wall between the inferior and middle turbinates (Fig 1).

Figure 1
figure 1

Nasal endoscopy that shows a tumor in the left nasal wall.

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A CT Scan showed a nonenhancing 3 cm. tumor in the left nasal cavity located between the inferior and middle turbinates at the level of the uncinate process with medial maxillary wall erosion (Fig 2).

Figure 2
figure 2

CT Scan, of paranasal sinus, that shows the tumor in the left nasal cavity.

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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.

Microscopic review of the specimen revealed a vascular neoplasia with multiple vessels comprised of endothelial cells with prominent nuclei and atypia (Fig 3). Elongated fusiform nucleus cells with atypia were also observed between the vascular areas (Fig 4). The final diagnosis was an intermediate-grade angiosarcoma.

Figure 3
figure 3

Image that corresponds to a vascular neoplasia, composed of multiple blood vessels surrounded by endothelial cells with atypical nuclei.

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Figure 4
figure 4

On the solid areas of the tumor we observed elongated atypical cells with fusiform nuclei between vascular areas.

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The patient continues symptom-free and with no evidence of local or distant metastasis 3 years after initial treatment (Fig 5).

Figure 5
figure 5

3 years postoperative CT Scan showing nasal cavity with no presence of tumor.

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Discussion

Upper aerodigestive angiosarcoma is a very rare tumor; only 16 cases have been reported since 1977 (Table 1). Of these, six were located in the paranasal sinuses and four in the nasal cavity. Salomon [5]. from the MD Anderson Cancer Center in Houston reported that angiosarcoma corresponds to 1% of all sarcomas.

Table 1 Reported angiosarcoma cases, 1974–2008
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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 [6]. This contrasts with angiosarcomas of the nasal cavity and paranasal sinus which is usually 7.1 months [7]. Our patient presented with a 5 month history of these symptoms.

Etiology is unknown and has been associated to certain risk factors such as chronic lymphedema, radiotherapy, vinyl chloride exposure, trauma and telangiectatic skin lesions [8, 9]

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 [9]

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 [9]

Free margins are the best prognostic factor for avoiding recurrence [7, 8]

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 [7]

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.

Consent

"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."