Bilateral visual failure with optic tract involvement in sellar-suprasellar lymphoma: illustrative case

Abstract

BACKGROUND

Visual dysfunction related to sellar lesions is usually attributed to chronic compression of the optic chiasm, typically following an insidious, progressive course. Abrupt or disproportionately rapid visual failure should therefore prompt careful evaluation for alternative or additional mechanisms.

OBSERVATIONS

A 69-year-old woman experienced progressive visual deterioration over 3 months, followed by near-complete bilateral visual loss within 1 week. Visual field testing showed severe bilateral generalized depression. Dedicated sellar enhanced MRI revealed a large sellar-suprasellar mass compressing the optic chiasm. In addition to mass effect, contrast-enhanced images demonstrated optic tract enhancement and avid leptomeningeal enhancement, raising concern for spread along the optic tracts and meningeal dissemination beyond the primary sellar-suprasellar region. Axial fluid-attenuated inversion recovery sequences further showed bilateral optic tract hyperintensity with signal abnormalities extending to the adjacent hypothalamic structures. The patient underwent endoscopic transsphenoidal tumor resection. Pathological examination confirmed aggressive B-cell lymphoma (CD20+, CD79a+, CD5+, Ki-67 index 90%).

LESSONS

This case emphasizes that, in sellar lesions presenting with rapidly progressive visual loss, radiological assessment should extend beyond the sellar mass and optic chiasm to include the optic tracts and adjacent postchiasmatic visual pathway.

https://thejns.org/doi/10.3171/CASE26610

Visual impairment is one of the most common manifestations of sellar-suprasellar lesions because of their close relationship to the optic apparatus. In most patients, visual dysfunction develops gradually and is interpreted within the framework of chronic anterior visual pathway compression, particularly at the level of the optic chiasm. This classic pattern typically results in bitemporal hemianopia due to compression of the crossing nasal retinal fibers within the chiasm. Neuro-ophthalmic assessment in sellar disease therefore focuses heavily on chiasmal dysfunction and related visual field patterns.¹ However, visual impairment caused by sellar-suprasellar lesions is not always limited to chiasmal compression. Extension into the postchiasmatic visual pathways, particularly the optic tracts, may produce atypical clinical presentations that differ from the classic pattern of gradual visual decline. Rapid or disproportionate bilateral visual deterioration should therefore prompt careful evaluation of alternative anatomical mechanisms beyond isolated optic chiasm involvement.

In routine clinical practice, sellar MRI is primarily designed to depict the sellar and parasellar region and is highly effective for evaluating the pituitary gland, cavernous sinus, parasellar structures, and optic chiasm. However, it does not necessarily emphasize systematic assessment of the downstream retrochiasmal visual pathway, a limitation that may become clinically important when visual loss is unusually rapid or disproportionately severe.² Optic tract signal abnormalities related to tumor-associated edema have been reported on conventional axial fluid-attenuated inversion recovery (FLAIR) MRI in pituitary region tumors.³

Lymphoma involving the optic pathway and the hypothalamic-pituitary axis is an especially uncommon entity that may mimic more prevalent sellar-suprasellar lesions on presentation and neuroimaging. Its infiltrative nature can extend beyond the sellar-suprasellar region to involve adjacent visual and hypothalamic structures, contributing to abrupt and severe visual deterioration that may be underappreciated on a pituitary-centered diagnostic assessment.^4–6 We report a case of aggressive B-cell lymphoma centered in the sellar-suprasellar region, presenting with fulminant bilateral visual failure. Brain MRI revealed bilateral optic tract hyperintensity and adjacent hypothalamic signal abnormalities on FLAIR, together with optic tract and meningeal enhancement on contrast-enhanced sequences.

Illustrative Case

A 69-year-old woman presented with a 3-month history of progressive visual deterioration. During the week before admission, her visual function worsened dramatically, ultimately leaving her with only light perception in both eyes.

Automated perimetry using the Humphrey 24-2 Swedish Interactive Threshold Algorithm (SITA) Fast program demonstrated profound bilateral visual field depression (Fig. 1). In the right eye, fixation losses were 0/0 and false-positive errors were 6%. The visual field index was 6%, the mean deviation was −28.15 dB, and the pattern standard deviation was 3.72 dB. In the left eye, fixation losses were 0/0 and false-positive errors were 3%. The visual field index was 1%, the mean deviation was −29.80 dB, and the pattern standard deviation was 1.80 dB. In both eyes, the pattern deviation plot was not displayed because the fields were severely depressed, and the total deviation maps showed near-global sensitivity loss. These findings indicated profound bilateral functional impairment at presentation. Neurological examination was otherwise unremarkable. Cranial nerve examination (III–XII), motor strength, sensation, and deep tendon reflexes were intact. Gait assessment was limited because of severe visual impairment.

FIG. 1.

Automated visual field testing. Humphrey 24-2 SITA Fast total deviation plots of the right eye (A) and left eye (B). The grayscale and numeric total deviation maps demonstrate severe generalized visual field depression in the right eye and near-total sensitivity loss in the left eye. The visual field index (VFI) and mean deviation (MD) were 6% and −28.15 dB in the right eye and 1% and −29.80 dB in the left eye, respectively. OD = oculus dexter; OS = oculus sinister.

Contrast-enhanced sellar MRI demonstrated a large sellar-­suprasellar mass with marked superior extension and compression of the optic chiasm (Fig. 2). On coronal T1-weighted images, the lesion was isointense and extended superiorly toward the third ventricle. Coronal T2-weighted images showed mildly increased signal intensity within the tumor, with associated hypothalamic involvement and edema-like hyperintensity along the course of the optic tracts. Contrast-enhanced coronal and sagittal images revealed avid enhancement of the mass, accompanied by leptomeningeal enhancement, raising suspicion for meningeal involvement. Notably, abnormal enhancement was also identified along the optic tracts, more pronounced on the left. Sagittal images further showed superior posterior displacement and compression of the mammillary bodies by the mass. FLAIR imaging showed hyperintensity involving the chiasmatic region and extending along the bilateral optic tracts (Fig. 3). Additional signal abnormalities were also present in adjacent hypothalamic and mesencephalic structures, including the tuber cinereum, mammillary bodies, hypothalamus, ventral cerebral peduncles, and periaqueductal gray matter. Anatomically, these findings are better interpreted as two related but distinct patterns of involvement: 1) edema along the anterior visual pathway, extending from the optic chiasm into the bilateral optic tracts, and 2) edema involving adjacent hypothalamic and mesencephalic structures.

FIG. 2.

Sellar-suprasellar mass with optic chiasm compression. A: Coronal T1-weighted image demonstrating an isointense lesion extending toward the third ventricle. B: Coronal T2-weighted image showing mildly increased intratumoral signal intensity with associated hypothalamic involvement and edema-like hyperintensity along the course of the bilateral optic tracts (arrows). C and D:Postcontrast coronal (C) and sagittal (D) images revealing avid enhancement of the mass, leptomeningeal enhancement, and abnormal enhancement along the optic tracts (thin arrow) and basal ganglia (thick arrow), more pronounced on the left. The sagittal image also demonstrates superior posterior displacement and compression of the mammillary bodies (star).

FIG. 3.

FLAIR and diffusion kurtosis imaging (DKI) findings of the optic tracts and hypothalamus. A: Axial FLAIR images demonstrating hyperintensity along the optic tracts (arrow), extending into the hypothalamus (star) and midbrain. B: DKI-based fiber tractograms delineating the bilateral optic tracts.

The patient underwent conventional endoscopic transsphenoidal surgery. Intraoperatively, the tumor capsule was partially intact. The neoplasm appeared whitish with a moderately firm consistency. Initial intratumoral decompression was performed, during which CSF leakage was encountered. The adhesion between the tumor and the optic nerve was carefully dissected along the tumor border, identifying the arachnoid plane between the tumor capsule and the surrounding structures. The deep posterior aspect of the tumor was densely adherent to the hypothalamus, making dissection with a curette difficult; therefore, a combination of microforceps and ring curette was used for separation. A pedicled nasoseptal flap was used to repair the CSF leak. The procedure achieved a near-total resection. Histopathological examination of the sellar lesion confirmed aggressive B-cell lymphoma. Immunohistochemistical analysis showed tumor cells positive for CD20, CD79a, and CD5, with CD3 negativity and a Ki-67 labeling index of 90%.

During the 1st postoperative week, the patient remained in a state of persistent high fever and delirium, findings suggestive of postoperative hypothalamic dysfunction. Bedside visual assessment after surgery suggested slight improvement in light perception, indicating only limited visual recovery after decompression; however, formal visual field testing was not feasible because of her postoperative clinical condition. Follow-up contrast-enhanced MRI 3 weeks after resection revealed slight residual enhancement in the surgical bed (Fig. 4A–D).

FIG. 4.

Contrast-enhanced MR images obtained 3 weeks (A–D) and 9 months (E–H) after resection. A: Coronal T1-weighted image. B: Coronal T2-weighted image. C:Contrast-enhanced coronal T1-weighted image. D: Contrast-enhanced sagittal T1-weighted image. E: Contrast-enhanced coronal T1-weighted image. F:Contrast-enhanced sagittal T1-weighted image. G:Axial T2-weighted image showing diffuse white matter changes, ventricular enlargement, and hydrocephalic changes. H:Diffusion-weighted image showing no obvious restricted diffusion within the surgical bed.

Following discharge, the patient was transferred to a rehabilitation facility for ongoing care. She received glucocorticoid therapy and subsequently underwent whole-brain radiotherapy using helical tomotherapy at an outside institution. Treatment was discontinued after eight radiotherapy sessions because of persistent nausea, vomiting, and poor overall tolerance. Standard high-dose methotrexate-based chemotherapy was not administered because of her poor general condition and limited tolerance of further treatment. At the latest follow-up, her Glasgow Coma Scale score was 13, accompanied by significant cognitive impairment, including impaired recognition. A recent enhanced brain MRI study, approximately 9 months postoperatively, revealed lesional regression compared to previous imaging. Additionally, brain MRI revealed cerebral degenerative changes consistent with leukoaraiosis and interstitial edema, accompanied by bilateral hippocampal atrophy (medial temporal lobe atrophy grade 3) (Fig. 4E–H).

Endocrine evaluation before surgery demonstrated pituitary dysfunction, including decreased morning cortisol (2.50 μg/dL) with low-normal adrenocorticotropic hormone (ACTH), mild hyperprolactinemia (131.27 ng/mL), and reduced luteinizing hormone (LH) (0.27 mIU/mL), suggesting hypothalamic-pituitary axis involvement. Postoperatively, adrenal insufficiency persisted, with decreased ACTH and morning cortisol levels requiring continued glucocorticoid therapy (Table 1).

TABLE 1.

Preoperative and postoperative endocrine findings indicating hypothalamic-pituitary dysfunction

Hormonal Parameter	Reference Range	Preop	POD1
Morning cortisol (8 am), μg/dL	6.7–22.6	2.50↓	15.81*
ACTH, pg/mL	7.0–65.0	9.59	6.34↓
Free T3, pmol/L	2.43–6.01	3.56	2.44
Free T4, pmol/L	9.01–19.04	9.99	8.37↓
TSH, μIU/mL	0.35–4.94	0.137↓	0.3778
GH, ng/mL	0.010–3.607 (female)	1.101	3.557
Prolactin, ng/mL	3.46–19.40	131.27↑	43.89↑
LH, mIU/mL†	5.16–61.99	0.27↓	0.20
FSH, mIU/mL†	26.7–133.41	5.72↓	4.96↓

GH = growth hormone; FSH = follicle-stimulating hormone; POD1 = postoperative day 1; TSH = thyroid-stimulating hormone; ↓ = below; ↑ = above.

* Patient received glucocorticoid therapy after surgery.

† Reference ranges for LH and follicle-stimulating hormone were based on values for postmenopausal females. Values outside the reference ranges are indicated by the arrows.

Informed Consent

The necessary informed consent was obtained in this study.

Discussion

Observations

This case illustrates an imaging-based anatomical explanation for unusually rapid visual collapse in sellar-suprasellar lymphoma, in which lymphoma involvement extends along the optic tract rather than remaining confined to isolated compression of the optic chiasm. In routine practice, however, structured assessment of the chiasmatic and postchiasmatic visual pathway is not yet consistently integrated into clinical image interpretation.⁷ Consequently, once attention is drawn to a large enhancing sellar mass, subtle but diagnostically relevant abnormalities along the visual pathway may not receive adequate attention.

Visual loss from sellar-suprasellar tumors is usually discussed as compressive chiasmopathy, with progressive acuity decline and characteristic chiasmal field defects rather than collapse to near-functional blindness over a few days. From an imaging perspective, acute or rapidly progressive visual failure in a sellar-suprasellar mass should first prompt consideration of pituitary apoplexy, one of the most important differential diagnoses. Pituitary apoplexy typically occurs in a pituitary adenoma and presents with severe abrupt headache and visual acuity and visual field defects. MRI may demonstrate intratumoral hemorrhage or infarction, with hemorrhagic signal intensity depending on the stage of blood products.⁸ Craniopharyngioma is another important differential diagnosis, particularly when a cystic or calcified component is present. Acute visual defects may occur from rapid cyst enlargement or intratumoral hemorrhage.⁹ Internal carotid artery aneurysms can mimic pituitary adenoma or other sellar masses and may cause visual impairment or cranial nerve palsies through compression of the optic pathway or cavernous sinus structures.¹⁰ Recognizing this vascular mimic is clinically critical because misdiagnosis before transsphenoidal surgery may be catastrophic.

The crucial imaging clue was that the abnormality was not confined to the sellar mass or compressed chiasm, but extended posteriorly along the optic tract, favoring infiltrative tumor involvement rather than a typical hemorrhagic, cystic, or vascular mimic. Although malignant B-cell lymphoma involving the anterior visual pathway has been previously reported, our case showed a different spatial pattern. Rather than presenting as a primary anterior visual pathway lesion, the tumor was centered in the sellar-suprasellar region, initially mimicking a common pituitary tumor and compressing the optic chiasm from below. The posterior extension of optic tract enhancement and FLAIR hyperintensity, together with hypothalamic and mesencephalic abnormalities, suggested lymphomatous extension along the visual pathway with associated blood-brain barrier disruption, rather than edema caused solely by mechanical compression.

Lessons

Recent morphometric studies have shown that the severity of visual impairment correlates not only with suprasellar extension but also with the degree and configuration of optic pathway deformation, implying that the clinically relevant burden is exerted on the visual pathway rather than at a single point of chiasmal contact.¹¹ Ju et al. further reported that preoperative optic tract edema was associated with worse visual function and less favorable visual outcome in patients with sellar and suprasellar tumors, suggesting that this finding may reflect substantial injury along the visual pathway.³ These imaging findings also have prognostic implications. Previous studies have shown that surgical decompression may relieve mass effect but does not necessarily reverse established optic pathway injury, particularly when the optic tract is already compromised.¹² Contemporary prognostic evidence indicates that worse baseline visual function, longer symptom duration, and adverse MRI-based optic chiasm metrics are associated with a lower likelihood of meaningful recovery.^13,14 Accordingly, edema-like signal change and optic tract enhancement along the chiasmatic-optic tract axis should be interpreted as a marker of reduced functional reserve rather than merely descriptive radiological findings. In this context, surgery remains essential for tissue diagnosis and decompression, but expectations for postoperative visual recovery should be guarded.

In addition to providing prognostic information, recognition of optic pathway involvement may also influence the initial management strategy. For sellar-suprasellar lesions presenting with rapid or unexplained visual deterioration, comprehensive imaging evaluation, including dedicated sellar MRI and thin-slice brain sequences and careful assessment of the optic pathways, is essential before surgical decision-making whenever feasible. When lymphoma is suspected or confirmed, tissue diagnosis followed by appropriate systemic therapy should be prioritized rather than extensive tumor resection. However, in cases with severe and progressive visual dysfunction caused by direct compromise of the optic apparatus, decompressive surgery may still be considered with the goal of preserving visual function. Previous studies of pituitary apoplexy have demonstrated that early decompression can achieve favorable visual outcomes in patients with severe visual deficits. ^15,16 Intraoperative frozen-section analysis identified a malignant neoplasm but was insufficient to confirm lymphoma. Given the indeterminate diagnosis, aggressive radiographic appearance, and concern for rapid progression during the turnaround time for final pathological results, gross-total resection was pursued to alleviate optic tract compression. Ultimately, this case suggests that in sellar-suprasellar tumors with unusually rapid visual collapse, radiologists should carefully evaluate the entire visual pathway, not only the sellar mass and degree of chiasmal compression.

This report is limited by the absence of long-term ophthalmological and oncological follow-up after discharge. Nevertheless, the imaging findings provide a plausible anatomical explanation for the unusually rapid bilateral visual failure.

This case highlights a rare presentation of sellar-suprasellar aggressive B-cell lymphoma with rapidly progressive bilateral visual failure. Critically, it demonstrates that dedicated sellar MRI alone may be insufficient when visual decline is abrupt or disproportionately severe. Conventional brain MRI with axial sequences may reveal optic tract, retrochiasmal, and hypothalamic abnormalities that help explain the clinical presentation. Therefore, such sequences should be considered in the imaging evaluation of patients with rapidly progressive visual loss associated with sellar lesions. Moreover, this case underscores the challenge of intraoperative diagnosis; a negative frozen-section finding does not exclude lymphoma. In cases with strong clinical suspicion for lymphoma, a biopsy-first strategy is recommended to avoid the morbidity of aggressive resection and to expedite chemosensitivity-specific treatment, particularly when the hypothalamus is extensively involved.

Disclosures

The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

Author Contributions

Conception and design: both authors. Acquisition of data: both authors. Analysis and interpretation of data: both authors. Drafting the article: both authors. Critically revising the article: Cheng. Reviewed submitted version of manuscript: both authors. Approved the final version of the manuscript on behalf of both authors: Cheng. Administrative/technical/material support: Guo. Study supervision: Cheng.

Correspondence

Yijun Cheng: Center of Pituitary Tumor, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. cyj12574@rjh.com.cn.