PMC5301122_01
a A large vascular polypoid mass completely filling the left nasal cavity. b The nasal septum is excluded to the right side.
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F1
At the endoscopic examination, a large vascular polypoid mass was found to completely fill the left nasal cavity and the nasal septum was excluded to the right side (Fig. 1a, b).
a, b CT and MRI (T1) showed a large hypervascular mass involving the left nasal airway, maxillary antrum, and anterior ethmoid cells. There was no bony erosion or contiguous spread, and the remaining sinuses, orbit, and cranial fossa were uninvolved.
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F2
There was no bony erosion or contiguous spread, and the remaining sinuses, orbit, and cranial fossa were uninvolved (Fig. 2a, b).
a Angiography of left maxillary artery. b Gelatin was injected into the left maxillary artery. c A lateral rhinotomy approach was performed to remove the tumor.
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F3
We embolized the mass (Fig. 3a) and subsequently performed surgical resection. Angiography of the left maxillary artery showed deep dyeing of the mass and gelatin was injected into the artery (Fig. 3b). A lateral rhinotomy approach was utilized to gain access to the tumor, followed by dissection along the medial wall of the orbit and around the tumor (Fig. 3c).
Microscopic analysis revealed an undifferentiated tumor consisting of a solid sheet of small, round blue cells (a 10 x 2, b 10 x 40). c Immunohistochemically, tumor cells showed strongly positive staining with CD99.
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F4
A microscopic analysis revealed an undifferentiated tumor consisting of a solid sheet of small, round blue cells (Fig. 4a, b). Immunohistochemically, the tumor cells were strongly positive for CD99 (Fig. 4c).
Molecular studies using PCR analysis confirmed the chromosomal translocation of FLI1 (exon 6).
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F5
Molecular studies using PCR confirmed the chromosomal translocation of FLI1 (exon 6) (Fig. 5).
Adjuvant chemotherapy consisting of vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide (7 cycles total) was commenced. He also received radiation therapy for local control (total dose of 50.4 Gy). The patient is alive without evidence of recurrence or metastasis.
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F6
Radiation therapy was also administered for local control (total dose: 50.4 Gy) (Fig. 6).