Sinonasal Chondrosarcoma Presenting With Isolated Severe Vision Loss

A 24-year-old man presented with a 2-month history of progressive, painless vision loss in the right eye, with no history of headache, nasal congestion, rhinorrhea, or epistaxis.

Photo and Video Essay Section Editors: Melissa W. Ko, MD Dean M. Cestari, MD Peter Quiros, MD Sinonasal Chondrosarcoma Presenting With Isolated Severe Vision Loss Michael T. B. Nguyen, MD, Armin Farahvash, BSc, Brendan C. Dickson, MD, John M. Lee, MD, Michael D. Cusimano, MD, PhD, Derek S. Tsang, MD, MSc, Jonathan A. Micieli, MD, CM FIG. 1. Optic nerve photographs at presentation (A) demonstrating mild temporal pallor in the right optic nerve. Automated 24-2 SITA-Fast visual field testing at presentation (B) showing severe generalized depression with a mean deviation of 234.54 dB. Optic nerve photographs at 6-month follow-up demonstrating increased temporal pallor of the right optic nerve (C). Automated 24-2 SITA-Fast visual field at 6-month follow-up showing a central scotoma with mean deviation of 26.90 dB (D). Abstract: A 24-year-old man presented with a 2-month history of progressive, painless vision loss in the right eye, with no history of headache, nasal congestion, rhinorrhea, or epistaxis. Department of Ophthalmology and Vision Sciences (MTBN, JAM), University of Toronto, Toronto, Ontario, Canada; Faculty of Medicine (AF), University of Toronto, Toronto, Ontario, Canada; Department of Pathology and Laboratory Medicine (BCD), Mount Sinai Hospital; Department of Laboratory Medicine and Pathobiology (BCD), University of Toronto, Toronto, Ontario, Canada; Department of OtolaryngologyHead and Neck Surgery (JML), St. Michael’s Hospital, Toronto, Ontario, Canada; Department of Otolaryngology-Head and Neck Surgery (JML), University of Toronto, Toronto, Ontario, Canada; Division of Neurosurgery (MDC), Department of Surgery, St. Michael’s Hospital, Toronto, Ontario, Canada; Department of Surgery (MDC), University of Toronto, Toronto, Ontario, Canada; Radiation Medicine Program (DST), Princess Margaret Cancer Centre, University Health Network; Department of Radiation Oncology (DST), University of Toronto, Toronto, Ontario, Canada; Division of Neurology (JAM), Department of Medicine, University of Toronto, Toronto, Ontario, Canada; and Department of Ophthalmology (JAM), St. Michael’s Hospital, Toronto, Ontario, Canada. The authors report no conflicts of interest. Address correspondence to Jonathan A. Micieli, MD, CM, Kensington Vision and Research Centre, 340 College Street, Suite 501, Toronto, ON, Canada, M5T 3A9; E-mail: [e-mail] e752 His visual acuity was counting fingers at 1 ft in the right eye and 20 of 20 in the left eye with a right relative afferent pupillary defect and mild temporal optic disc pallor. MRI of the brain and orbits showed a mass involving bilateral ethmoid and sphenoid sinuses and right nasal cavity. He underwent urgent extended endoscopic endonasal transsphenoidal approach for resection of the sinonasal skull base tumor and photon radiation therapy. Pathology revealed a well-differentiated cartilaginous neoplasm with focal areas of entrapped native bone, consistent with a chondrosarcoma WHO grade I/III. At 6-month follow-up after surgery, he had a visual acuity of 20/40 in the right eye and 20/20 in the left eye. Malignant tumors from the sinonasal area should be kept in the differential diagnosis for compressive optic neuropathies and may present with vision loss even in the absence of nasal or sinus symptoms. Journal of Neuro-Ophthalmology 2021;41:e752–e755 doi: 10.1097/WNO.0000000000001130 © 2020 by North American Neuro-Ophthalmology Society A 24-year-old man presented with a 2-month history of progressive, painless vision loss in his right eye. He denied headache, nasal congestion, rhinorrhea, or epistaxis. Nguyen et al: J Neuro-Ophthalmol 2021; 41: e752-e755 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo and Video Essay FIG. 2. MRI of the orbits demonstrating a T1-hypointense (A), T2-mildly hyperintense (B) mass with heterogeneous enhancement involving bilateral ethmoid and sphenoid sinuses and right nasal cavity (C). There was severe narrowing of the optic canal with compression of the canalicular portion of the right optic nerve (D). He saw multiple eye-care providers who noted reduced visual acuity in his right eye, but no other abnormalities. He was seen in neuro-ophthalmology consultation and had a visual acuity of counting fingers at 1 ft in the right eye and 20/20 in the left eye There was a greater than 1.2 log unit right relative afferent pupillary defect, and dilated fundus examination demonstrated mild temporal pallor in the right optic nerve (Fig. 1A). Optical coherence tomography of the retinal nerve fiber layer showed a normal average thickness in both eyes (97 mm right eye and 89 mm left eye), and automated visual field testing showed severe generalized depression in the right eye (Fig. 1B). Due to a concern of compressive optic neuropathy, he underwent MRI of the orbits and brain with contrast. This showed a lobulated T1-hypointense, T2-hyperintense mass with heterogeneous enhancement involving bilateral ethmoid and sphenoid sinuses and right nasal cavity (Fig. 2). There was expansion of the sinuses including the overlying planum sphenoidale eccentric to the right causing severe narrowing of the optic canal with compression of the canalicular portion of the right optic nerve. Computed tomography (CT) of the chest, abdomen, and pelvis were normal. He underwent urgent extended endoscopic endonasal transsphenoidal approach for resection of the large sinonasal skull base tumor and decompression of the right optic nerve. This was a collaborative effort between the otolaryngology head and neck surgery and neurosurgery teams. Residual tissue was left intraoperatively because of close proximity to the internal carotid artery. Pathology showed a well-differentiated cartilaginous neoplasm with focal areas of entrapped native bone, consistent with a chondrosarcoma (WHO grade I; Fig. 3). He was treated with photon radiation therapy given the residual tumor and underwent (70 Gy in 35 fractions using a volumetric modulated arc therapy technique; Fig. 4) without complications. There was a brainstem maximum dose of 66.6 Gy and an optic apparatus maximum of 59.8 Gy. He was seen in neuro-ophthalmology follow-up 6 months after surgery and had a visual acuity of 20/40 in the right eye and 20/20 in the left eye and increased temporal pallor in the right eye (Fig. 1C). A central scotoma was evident on automated visual field testing at that time (Fig. 1D). Repeat MRI of the brain 6 months after surgical resection demonstrated diffuse changes within the paranasal sinuses consistent with postoperative changes. There were no signs of tumor recurrence. FIG. 3. Representative hematoxylin and eosin (H&E) stained sections of tumor. Low magnification demonstrating curetted fragments of chondroid tissue subtending respiratory mucosa (A). Intermediate magnification showing permeation of tumor around preexisting bone trabeculae (B). Intermediate magnification highlighting area of mildly increased cellularity (C). High magnification revealing mild nuclear pleomorphism and occasional binucleation (D). Nguyen et al: J Neuro-Ophthalmol 2021; 41: e752-e755 e753 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo and Video Essay FIG. 4. Radiation dose distribution for this patient, with axial, sagittal,, and coronal views. The total prescription was 70 Gy. Colorwash areas received the doses shown in the legend in the top-right corner. For example, volumes receiving at least 95% of the prescription dose (66.5 Gy) are shown in red, whereas volumes receiving at least 50% of the prescription dose (35 Gy) are shown in purple (D). Chondrosarcomas are the third most common primary bone neoplasm after myeloma and osteosarcoma. They are differentiated from chondroblastic osteosarcoma by the absence of osteoid matrix production (1). Chondrosarcomas most often occur in the pelvis, extremities, and ribs. The head and neck region comprises 1%–12% of cases (2–4). A National Cancer Database report on 400 chondrosarcomas of the head and neck found that 12% originated in the sinonasal area, which is more common in women and in patients younger than 50 years (5). Surgical excision is the primary treatment modality with neck dissection typically not performed because of the low incidence of regional metastases (6). Radiation treatment is a commonly used adjuvant treatment and is rarely used alone, whereas chemotherapy is reserved for high-grade cases (5,6). Postoperative radiotherapy is used in cases of large tumors, involvement of the skull base and vital neurovascular structures and subtotal resection as in our patient. The survival rate of chondrosarcoma has been reported to range from 44% to 87% with the most important prognostic factor being resectability as most deaths occur because of locally e754 invasive disease (7). Patients undergoing radiotherapy have been shown to have a lower proportion of local recurrence and distant metastasis compared with surgery alone in a systematic review of the literature (29.4% vs 32.8%); however, the small sample size because of the rarity of the condition and lack of standardized patient data make it difficult to draw strong conclusions (7). Vision loss as the presenting symptom of sinonasal chondrosarcoma is rare, with few cases reported in the literature (8–13). Of the 7 cases identified, we were able to retrieve one similar to our case in having isolated visual symptoms in an adult (8). Waga et al (8) presented the case of a 27-year-old woman who had 3 months of left eye blurred vision and was found to have a mass centered in the ethmoid sinus extending into the anterior cranial fossa with compression of the left optic nerve. Surgical resection was performed without adjuvant therapy, but visual outcome was not reported. Sinonasal chondrosarcomas frequently manifest with associated symptoms such as headache, nasal congestion, rhinorrhea, or epistaxis, and patients with vision loss and these symptoms should have Nguyen et al: J Neuro-Ophthalmol 2021; 41: e752-e755 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo and Video Essay a sinonasal mass considered in the differential diagnosis (9– 13). In a systematic review of the literature, nasal obstruction was by far the most frequently reported presenting symptom as most originate from the nasal septum (7). Chondrosarcomas outside the sinonasal area are more common and may also be associated with vision loss when originating from the orbit (14) and skull base (15). Metastasis to the choroid have also been reported (16). In summary, compressive optic neuropathies are characterized by slowly progressive vision loss. Malignant tumors from the sinonasal area should be kept in the differential diagnosis and may present with vision loss from optic nerve compression even in the absence of nasal or sinus symptoms. Although intracranial chondrosarcomas can manifest with neuro-ophthalmic symptoms, this is also the case for sinonasal chondrosarcomas. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: M. T. B. Nguyen, A. Farahvash, and J. 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