Abstract
Introduction/Objective
Angiosarcomas are aggressive neoplasms of the vascular endothelium and comprise approximately 2% of all soft tissue sarcomas. The occurrence in the sinonasal cavity is rare. A case of epithelioid angiosarcoma of the maxillary sinus in a 19-year-old patient is presented and its clinical presentation, histologic, immunohistochemical, and molecular features are discussed.
Methods/Case Report
A 19-year-old male evaluated at Rutgers University Hospital for 3 months of left-sided epistaxis. He reports left side nasal obstruction, facial pain, and epiphoria. Computed tomography revealed a heterogeneous soft tissue mass within the left maxillary sinus; with dehiscent segments extending into the premaxillary space, infratemporal fossa, and the left orbital floor. Bilateral rigid nasal endoscopy showed bowing of the left maxillary sinus medial wall, exposing a mucosalized mass. He underwent left maxillary antrostomy and left subtotal maxillectomy. Positron emission tomography scan showed no metastasis.
The tumor was within the submucosa of the respiratory epithelium and focally demonstrated anastomosing vascular spaces lined by atypical endothelial cells. The neoplastic cells showed epithelioid morphology with increased nuclear size, prominent nucleoli, and atypical mitotic figures up to 22 mitoses per 10 high power fields. The tumor cells were strongly and diffusely positive for CD31, CD34, FLI1, ERG, vimentin, and cytokeratin AE1/AE3. The tumor was negative for HMB45, desmin, HHV8, CAMTA1, FOSB, and S-100 and retained the expression of INI-1. The proliferation index was 60-70%.
A next generation sequencing disclosed stable tumors microsatellite status and a mutational burden of 1 mutation/Mb. Numerous variants of uncertain significance were identified including amplification of BRIP1, GNA13, and PRKAR1A as well as various point mutations including FLT4 G1296S and G71R, MET R359Q, ROS1 R442Q, DOT1L R757W, PDCD11 R1610L, TLL2 R849C, FANCD2 D400E, LEF1 D177V, PRDM1 A408P, FLT1 F989L, and MAP3K1 S939C.
Results (if a Case Study enter NA)
NA
Conclusion
Sinonasal angiosarcoma is incredibly rare with a poor overall prognosis. Few lesions have been reported in the pediatric population. Angiosarcoma may be confused with other types of malignant neoplasms when epithelioid morphology is present. The use of multiple vascular and epithelial markers with clinical correlation will be helpful in the diagnosis.