Orbital lymphoma: masquerading as involutional ptosis

Unilateral ptosis can be the first symptom related to other causes that are traumatic, myogenic, neurogenic, or mechanical in nature.

Orbital lymphoma has presented a new face that physicians should recognize, according to Jane S. Kim, MD, from the Department of Ophthalmology and Visual Sciences, Kellogg Eye Center, University of Michigan, Ann Arbor.

Ptosis does not operate in a vacuum and can be associated with numerous other conditions, some of which can be life-threatening. In most cases, involutional ptosis is associated with levator dehiscence, but unilateral ptosis can be the first symptom related to other causes that are traumatic, myogenic, neurogenic, or mechanical in nature.

In the case under discussion, the patient was a 65-year-old woman whose history included hyperlipidemia, hypothyroidism, and chronic migraines.

She presented with a 9-month duration of left-sided ptosis, which was more marked at the end of the day and with fatigue. She did not experience pain, diplopia, muscle weakness, dysarthria, dysphagia, difficulty breathing, or other focal neurologic deficits, according to Kim.

Evaluations
The physical examination revealed no palpable adnexal masses, orbital rim deformities, or relative proptosis.

There was no prolapsed lacrimal gland or conjunctival mass visible on lid eversion and no palpable preauricular or cervically lymphadenopathy.

However, there was mildly increased resistance to retropulsion noted on the left compared to the right eye and mild fullness of the left superior sulcus.

The visual acuity was 20/20 bilaterally; intraocular pressures were in the normal range; pupils showed no afferent defect; ocular motility was normal, as was the fundus, cornea, and anterior chamber appearance.

The clinical examination also showed that margin-to-reflex distance 1 was 3.5 mm on the right and 0.5 mm in the left affected eye, and the levator function of that eye was 11 mm in contrast to greater than 14 mm in the fellow eye.

Magnetic resonance imaging (MRI) showed a well-demarcated tumor that involved the markedly enlarged left lacrimal gland with molding to adjacent ocular structures without bony erosion.

There was inferior displacement of the left globe with a mass effect on the superior rectus-levator complex, Kim described.

A biopsy of the left lacrimal gland showed mantle cell lymphoma. Other testing showed no systemic involvement.

The patient underwent 24 Gy of radiation to the left lacrimal gland, and 3 months after completion another MRI showed a markedly reduced tumor, with near uniform enhancement comparable to the right eye.

A 6-month positron emission tomography-computed tomography scan showed resolution of tracer uptake in the left lacrimal gland.

The investigators pointed out that mantle cell lymphoma is a rare form of non-Hodgkin’s lymphoma characterized by widespread disease on presentation, an aggressive clinical course, frequent recurrences, and poor survival.

They emphasized the importance of long-term follow-up because the 5-year survival for patients with mantle cell lymphoma can be as low as 38% despite the good survival rates associated with most of the ocular adnexal lymphomas that have a 5-year survival of 81%.

“This case underscores the importance of performing a thorough examination (e.g., palpation of periocular soft tissues, resistance to retropulsion) followed by an appropriate work-up. Careful attention to the clinical examination can lead to early detection and treatment of life-threatening conditions,” Kim concluded.

This article is adapted from Kim’s presentation at the Women In Ophthalmology 2021 Summer Symposium, August 26-29, Amelia Island, FL.

She has no financial interest in this subject matter.