Angiosarcoma arising from nasal cavity; A case report

Figures & data

Figure 1. (A) Axial contrast enhanced (CE) computed tomographic (CT) scan, showing opacification of the inferior meatus, partly enhanced. (B) Coronal CE-CT scan. It is difficult to evaluate bone erosion or bone destruction accurately, but neither is apparent. (C) T2-weighted axial magnetic resonance imaging (MRI), showing a heterogeneous high-intensity signal in the same area as on the CT scan. (D) T1-weighted MRI with gadolinium, showing heterogeneous enhancement of the lesion.

Figure 2. (A) The tumor was in the inferior meatus and was in contact with the inferior turbinate and middle turbinate. (B) Appearance after resection of the tumor. The middle turbinate (*) and inferior turbinate (**) were also resected. No characteristics of malignancy were found. (C) The resected tumor. (D) Specimen stained with hematoxylin and eosin (H&E; $\times$40), showing sheets of atypical spindle cells. Scale bar: 50 μm. (E) Stained specimen (H&E, $\times$200), showing several mitotic figures. Scale bar: 100 μm. (F) Immunohistochemical staining for erythroblast transformation-specific-related gene (ERG; $\times$200). Tumor nuclei are positive for ERG, which is highly expressed in endothelial cells. Scale bar: 100 μm.

Table 1. Literature review of 23 cases of sinonasal angiosarcoma, including that of our patient.

Case	Published year	Author	Age	Gender	Location	Metastasis	Symptom	Treatment	Recurrence location	Length of survival
1	1976	McClatchey et al.	26	F	Maxillary sinus	None	Epistaxis	RT	None	7 months
2	1979	Bankaci et al.	68	M	Maxillary sinus	None	Rhinorrhea, facial pain, diplopia	Surgery (maxillectomy)	Local	22 months
3	1979	Sharma et al.	10	M	Maxillary sinus	None	Toothache, swelling	RT + surgery (maxillectomy) + RT	None	Not reported
4	1986	Zakrzewska	58	M	Maxillary sinus	None	Haemorrhagic lesion	RT + surgery (maxillectomy)	Local	14 months
5	1988	Williamson et al.	48	M	Maxillary sinus	None	Painless swelling of cheek	RT	Local	27 months
6	1989	Kurien et al.	38	M	Nasal cavity, maxillary sinus	None	Epistaxis, nasal obstruction	RT + surgery (wide excision)	None	10 months
7	1989	Lanigan et al.	73	M	Maxillary sinus	None	Intraoral swelling, epistaxis	Surgery (hemimaxillectomy) + RT	None	33 months
8	1990	Solomons et al.	33	M	Maxillary sinus	None	Epistaxis, diplopia, diminished sensation	Surgery (radical maxillectomy) + RT	None	27 months
9	1992	Kimura et al.	8	M	Nasal cavity, maxillary sinus	None	Epistaxis	Surgery (medial maxillectomy)	None	8 months
10	2000	Velegrakis et al.	72	M	Maxillary sinus	None	Epistaxis	Surgery (medial maxillectomy) + RT	None	24 months
11	2001	Wong et al.	34	F	Sphenoid sinus	Bone marrow	Headache, epistaxis	Embolization (failed)	None	0.5 month
12	2002	Triantafillindou et al.	50	F	Maxillary sinus	None	Enlarging maxilla	Surgery (medial maxillectomy) + CRT	None	36 months
13	2005	Oliveira et al.	56	M	Nasal columella	None	Epistaxis	Surgery (wide excision)	None	31 months
14	2006	Fukushima et al.	55	M	Nasal cavity	None	Epistaxis	rIL-2 + surgery (lateral rhinotomy) + RT	None	36 months
15	2009	Trevino-Gonzalez et al.	33	M	Nasal cavity	None	Epistaxis	Surgery (wide excision) + RT	None	36 months
16	2013	Gravvanis et al.	68	M	Nasal septum	None	None	Surgery (wide excision)	None	29 months
17	2014	Tomovic et al.	21	F	Frontal sinus	None	Forehead pain, proptosis	Chemotherapy + RT	None	12 months
18	2015	Es-Sibisssi et al.	53	M	Nasal cavity	None	Epistaxis, nasal obstruction	Surgery (ESS) + CRT	Local	33.5 months
19	2015	Deshmukh. et al.	50	F	Nasal cavity, ethmoidal sinus, sphenoid sinus	Not reported	Epistaxis, nasal obstruction	Surgery	None	1 month
20	2015	Mullins et al.	52	M	Maxillary sinus	None	Epistaxis, facial pain, enlarging mass	Surgery	None	53 months
21	2015	Sun et al.	29	F	Maxillary sinus	Lung	Nasal congestion, rhinorrhea	Not reported	Not reported	Not reported
22	2018	Chung et al.	30	F	Maxillary sinus	None	Facial pain, epistaxis	Surgery (wide excision, ESS) + RT	None	48 months
23		Our case	74	F	Nasal cavity	None	Epistaxis	Surgery (ESS) + CRT	None	30 months

rIL-2: cytokine therapy with recombinant interleukin-2, RT: radiotherapy, ESS: endoscopic sinus surgery, CRT: chemoradiotherapy.

Figure 3. Kaplan–Meier survival curve representing the patients in our review, generated with GraphPad Prism 8 (GraphPad Software, San Diego, CA, USA). We calculated the probability of 3 year survival as 63.7%.