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Lacrimal gland lymphoma


Key points

  • Definition: Malignant lymphoma of the lacrimal gland is relatively rare and is more prevalent in elderly women.

  • Synonym: Primary lacrimal gland lymphoma.

  • Classic clue: Patient presents with painless proptosis, showing nearly symmetrical bilateral lacrimal gland enlargement with smooth, homogeneous, diffuse enhancement and restricted diffusion.

  • Despite being rare, lymphomas comprise 37% of lacrimal gland malignancies.

Imaging

Computed tomography features

  • Bilateral lacrimal gland enlargement is isodense or slightly hyperdense to extraocular muscles (EOMs) (see Figure 40-1 , A ).

    FIGURE 40-1 ■, A, Axial nonenhanced computed tomography (NECT) shows significant lacrimal gland enlargement OU, L>R, isodense to EOMs. B and C, Sagittal postcontrast CT shows innumerable well-circumscribed multilevel lymph nodes in patient with known NHL. It is important to look for extraorbital clues.

  • Mild to moderate postcontrast enhancement similar to EOMs (see Figure 40-1 , B ).

  • Proptosis with medial displacement of globes (see Figure 40-1 , B ).

  • Absence of adjacent bony orbital invasion and destruction are pertinent negative findings.

  • The normal lacrimal gland thickness is about 4 to 5 mm.

  • Although studies have shown that increased lacrimal gland thickness correlates well with increased lacrimal gland volume, visual assessment of gland size is sufficient for most diagnoses.

  • Figure 40-1 , A and B, comfortably confirms gland thickness far above “normal” values.

Magnetic resonance imaging features

  • T1 isointense to hypointense to EOM.

  • T2 isointense to hyperintense to EOM.

  • T1 gadolinium exhibits homogeneous enhancement.

  • • Lacrimal gland lymphoma (LGL)

    reveals restricted diffusion with increased diffusion weighted imaging (DWI), decreased apparent diffusion coefficient (ADC) signal intensity.

  • Dedicated orbital imaging thin section, fat saturation, gadolinium-enhanced axial and coronal sequences are essential for excellent evaluation. ,

Clinical issues

Presentation

  • Painless proptosis most frequent symptom.

  • May demonstrate downward displacement of globes.

Treatment

Radiation therapy

  • Radiation therapy has an important role in management.

  • Recommended for stage I EA patients.

  • Mucosa-associated lymphoid tissue (MALT) lymphoma responds well to low-dose radiation therapy.

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