Abstract
Primary sinonasal angiosarcomas are very rare tumors. They typically occur in the nasal cavity of middle-aged patients. They are classically highly aggressive. Primary treatment is surgical excision when feasible. We describe a unique case of angiosarcoma in a young woman arising from the frontal sinus with distant metastasis. This case represents the first report of angiosarcoma arising from the frontal sinus in the English literature. The traditional treatment options for the advanced nature of her disease and overall poor prognosis are discussed. We further review the literature and discuss alternative treatments options. Newer chemotherapeutic regiments on the horizon show promise in helping to control this disease.
1
Introduction
Angiosarcomas are aggressive tumors of vascular endothelial origin. These tumors tend to occur in the middle-aged population and are managed with surgical excision with radiation and chemotherapy traditionally reserved for unresectable disease or positive margins.
This case is unique in that it is the first reported case in the English literature of a paranasal sinus angiosarcoma 1) arising in the frontal sinus, 2) with distant metastatic spread, and 3) staged using positron emission tomography (PET). In addition, due to the extent of disease careful thought and discussion were given to the optimal treatment modality. Interestingly, with the recent advent of newer targeted treatments more consideration has been given to the role of an antibody to vascular endothelial growth factor (anti-VEGF) to help control and limit disease.
2
Illustrative case
A previously healthy 21-year-old female with no prior history of radiation exposure presented with two months of progressing left sided forehead tingling, pain and proptosis with no significant vision changes that were refractory to antibiotics. A computed tomography (CT) scan showed a calcified mass centered in the left lateral frontal sinus with evidence of cortical bone destruction and invasion of the left frontal lobe and superior left orbit with opacification of the ethmoid sinus and destruction of the cribriform plate and medial orbital wall ( Fig. 1 A&B ). A magnetic resonance imaging (MRI) showed an enhancing heterogeneous mass centered in the left frontal sinus extending into the left superolateral aspect of the orbit with diffuse dural enhancement towards the vertex and left temporal lobe ( Fig. 1 C&D).
The patient was taken to the operating room for nasal endoscopy and biopsy, which revealed a purple friable necrotic tumor obstructing the left middle meatus ( Fig. 2 ). Histology was consistent with a high grade angiosarcoma, invading bone with hemorrhagic necrosis ( Fig. 3 ) Malignant endothelial cells were epithelioid type with nuclear pleomorphism and mitoses. The tumor cells were immunoreactive to vimentin and endothelial markers such as, CD31 and CD34, Fli-1 and Factor VIII.
A postoperative PET/CT was performed and showed a lyticlesion of the left frontal bone extending to the superolateral aspect of the left orbit with a maximum SUV of 10.5. The ethmoid sinus had opacification with a maximum SUV of 11.7 and the left maxillary sinus had a maximum SUV of 7.7. There was also a lesion of 1.1 cm in the apex of the right lung with an SUV of 2.5, a right level II cervical lymph node with an SUV of 2.7 and a C2 vertebral body lesion with an SUV of 3.4, all considered metastatic.
Given the distant disease as well as extensive local disease, initial treatment with chemotherapy including bevicizumab and radiation was recommended with the consideration for salvage surgery. The patient was subsequently treated with docetaxel and gemcitabine for six months with regression of the tumor, however the tumor regrew and the patient was started on radiation therapy. She is currently one year out from diagnosis with full functional capacity.
2
Illustrative case
A previously healthy 21-year-old female with no prior history of radiation exposure presented with two months of progressing left sided forehead tingling, pain and proptosis with no significant vision changes that were refractory to antibiotics. A computed tomography (CT) scan showed a calcified mass centered in the left lateral frontal sinus with evidence of cortical bone destruction and invasion of the left frontal lobe and superior left orbit with opacification of the ethmoid sinus and destruction of the cribriform plate and medial orbital wall ( Fig. 1 A&B ). A magnetic resonance imaging (MRI) showed an enhancing heterogeneous mass centered in the left frontal sinus extending into the left superolateral aspect of the orbit with diffuse dural enhancement towards the vertex and left temporal lobe ( Fig. 1 C&D).
