Anchora Sella

A now 75-year-old man was evaluated in October 2018 for fatigue, decreased libido, and decreased vision in the left eye. Visual fields showed bitemporal hemianopia, worse in the left eye. Brain imaging showed a large (3.6 cm) sellar mass which was resected in October 2019 and was histopathologically confirmed to be meningioma (WHO Grade 1, Ki-67 1%). Visual function improved. He defaulted on follow up in the context of the coronavirus pandemic. In June 2023 he experienced double vision, along with headache and epistaxis. Brain MRI on June 14, 2023 showed a 2.5 x 3.2 x 4.1 cm sellar mass. Eye examination on June 17, 2023, showed visual acuities of 20/25 in the right eye and 20/30 in the left eye; there was a complete aBduction defect of the left eye. Initial neuro-ophthalmology examination on June 28, 2023 was remarkable for visual acuities of 20/25 in the right eye and 20/200 in the left eye, a left relative afferent pupillary defect and aBduction defect of the left eye. Automated perimetry showed mild superior temporal defect in the right eye and dense depression in the left eye. Repeat brain MRI on July 28, 2023 showed increased size of sellar mass, now 4.0 x 3.1 x 5.4 cm.

“Anchora Sella” Adeniyi Fisayo 1, Vanessa Veloso 2 1 Yale University, 2 Yale New Haven Hospital History & Exam: A now 75-year-old man was evaluated in October 2018 for fatigue, decreased libido, and decreased vision in the left eye. Visual fields showed bitemporal hemianopia, worse in the left eye. Brain imaging showed a large (3.6 cm) sellar mass which was resected in October 2019 and was histopathologically confirmed to be meningioma (WHO Grade 1, Ki-67 1%). Visual function improved. He defaulted on follow up in the context of the coronavirus pandemic. In June 2023 he experienced double vision, along with headache and epistaxis. Brain MRI on June 14, 2023 showed a 2.5 x 3.2 x 4.1 cm sellar mass. Eye examination on June 17, 2023, showed visual acuities of 20/25 in the right eye and 20/30 in the left eye; there was a complete aBduction defect of the left eye. Initial neuro-ophthalmology examination on June 28, 2023 was remarkable for visual acuities of 20/25 in the right eye and 20/200 in the left eye, a left relative afferent pupillary defect and aBduction defect of the left eye. Automated perimetry showed mild superior temporal defect in the right eye and dense depression in the left eye. Repeat brain MRI on July 28, 2023 showed increased size of sellar mass, now 4.0 x 3.1 x 5.4 cm. Financial Disclosures: The authors had no disclosures. Grant Support: None. 30 | North American Neuro-Ophthalmology Society “Anchora Sella” Answer Final Diagnosis: Locally advanced poorly differentiated sinonasal cancer, SMARCB1 deficient. Summary of Case: On August 3, 2023, endoscopic endonasal resection of the sellar mass revealed a soft hemorrhagic mass within the sphenoid sinus, sellar and suprasellar spaces. Gross appearance of the mass was atypical for meningioma while frozen section revealed malignant, metastatic appearance. Final pathology revealed a densely cellular lesion and composed of poorly differentiated/undifferentiated pleomorphic cells with high mitotic activity and complete loss of SMARCB1(INI1) immunohistochemical expression. Neuro-ophthalmology examination on August 22, 2023 showed visual acuity of 20/60 in the right eye and no light perception in the left eye. Pupils were sluggishly reactive, with a dense left relative afferent pupillary defect. There was aBduction defect of both eyes and pallor of both optic discs, worse in the left eye. Automated perimetry showed inferior altitudinal and superior temporal defect in the right eye with sparing of the superior nasal quadrant. Treatment plan is for induction chemotherapy with paclitaxel and carboplatin followed by radiotherapy for consolidation. SMARCB1-deficient carcinoma is a rare subset of sinonasal carcinomas that harbors inactivating alterations of the SMARCB1 tumor suppressor gene. SMARCB1 is located at 22q11.2 and is a component of the chromatin remodeling complex SWI/SNF, but its function is largely unknown (1). In a large case-series study, 39 patients with ages ranging from 19 to 89 (median 52) years old presented with locally advanced disease (T3 and T4 stages) often showing extensive involvement of the paranasal sinuses, nasal cavity and skull base (2). Grossly, these tumors exhibit infiltrative borders and a variable exophytic papillary surface component. SMARCB1-deficient sinonasal carcinomas have an aggressive clinical course. The prognosis is heterogeneous with more than half of the patients dying of their disease within two years. Platinum-based chemotherapy which has shown good response for SMARCA4-deficient non–small cell lung carcinoma has also been suggested for SMARCB1-deficient sinonasal carcinomas. (3) Struggle/Dilemma of the Clinical Presentation Description: It was incorrectly assumed that re-growth of a sellar mass, at the exact same location of a previously resected meningioma, represented a recurrence of this tumor. This demonstrates premature closure bias (dropping the anchor too early!); As the patient was lost to follow-up during the coronavirus pandemic, it is unclear how tumor progression and symptom development occurred during this time. Timing of surgical intervention was accelerated by the rapid decline in afferent visual function. Keywords: Skull base tumors, 6th nerve palsy, Intracranial tumors References: (1) Yoshida, K., Fujiwara, Y., Goto, Y. et al. The first case of SMARCB1 (INI1) - deficient squamous cell carcinoma of the pleura: a case report. BMC Cancer 18, 398 (2018). https://doi.org/10.1186/s12885-018-4321-x (2) Agaimy A, Hartmann A, Antonescu CR, Chiosea SI, El-Mofty SK, Geddert H, Iro H, Lewis JS Jr, Märkl B, Mills SE, Riener MO, Robertson T, Sandison A, Semrau S, Simpson RH, Stelow E, Westra WH, Bishop JA. SMARCB1 (INI-1)-deficient Sinonasal Carcinoma: A Series of 39 Cases Expanding the Morphologic and Clinicopathologic Spectrum of a Recently Described Entity. Am J Surg Pathol. 2017 Apr;41(4):458-471. doi: 10.1097/PAS.0000000000000797. PMID: 28291122; PMCID: PMC5354087. (3) Tra Truong, Bayardo Perez-Ordoñez. Selected epithelial sinonasal neoplasms: an update. Diagnostic Histopathology. Volume 25, Issue 7, 2019, Pages 281-288. ISSN 1756-2317. https://doi.org/10.1016/j.mpdhp.2019.04.009. Contact Information: None provided. 2024 Annual Meeting Syllabus | 31