The Many Facets of Dacryoadenitis
Lymphoproliferative tumors account for 7% of the biopsies for orbital and lacrimal gland inflammation. Lacrimal gland lymphoma represents 24% of the lacrimal gland tumors in patients aged 60 year or older, with increasing incidence over the last decades. It concerns most frequently reactive lymphoid hyperplasia, MALT and follicular lymphoma. Japan has a higher incidence of ocular adnexal MALT lymphoma, of which 10% is IgG4 producing.
Inflammatory symptoms such as eyelid erythema are present in 15%, and pain or irritation in 26% of the cases. Dry eye is not reported. Bilateral lacrimal gland lymphoma occurs in the region of 24% of the cases, and is associated with systemic disease. Imaging studies show a smoothly enlarged and enhanced lacrimal gland, with surrounding inflamed soft tissue (Fig. 4).
(Enlarge Image)
Figure 4.
Mucosa-associated lymphoid tissue lymphoma of the lacrimal gland. (Top left) This 49-year-old man had a recent history of painless right upper eyelid swelling and redness, along with pain in all joints. On examination, the eyelid was diffusely swollen without palpable mass. There was no dry eye. (Top right) His CT scan showed a diffusely enlarged lacrimal gland and eyelid infiltration. (Bottom left) Biopsy specimen of the lacrimal gland showed a dense B-cell-rich infiltrate, extending into the epithelium, with a few T cells. There was loss of acini and dilation of the ducts. The B cells were monomorphous, with abundant cytoplasm and round, hyperchromatic nuclei. PCR analysis detected a t(11;18) translocation and monoclonal rearrangement of the immunoglobulin heavy-chain (IgH) (hematoxylin and eosin staining, original magnification ×100). These findings were conclusive evidence of MALT lymphoma. He was staged IV based on additional bone marrow invasion. He responded well to a regimen with chemotherapy and rituximab. He developed a mild dry eye. (Bottom right) CT scan obtained 9 months later, demonstrating a discretely enlarged lacrimal gland. Repeat biopsies of the lacrimal gland mass showed dense fibrosis with normal glandular parenchyma, and those of the periglandular orbital fat showed mild fibrosis and inflammation. Ten years from onset, he is still in continuous complete remission. CT, computed tomography; MALT, mucosa-associated lymphoid tissue.
Treatment implies radiotherapy or tumor removal in localized unilateral disease, although, in the case of reactive lymphoid hyperplasia, observation after the biopsy may be sufficient. Localized bilateral lacrimal gland disease, more aggressive histologic subtypes, and systemic disease is treated in accord with the chemotherapy, immunotherapy, and radiotherapy protocols.
Lacrimal Gland Lymphoproliferative Tumor
Lymphoproliferative tumors account for 7% of the biopsies for orbital and lacrimal gland inflammation. Lacrimal gland lymphoma represents 24% of the lacrimal gland tumors in patients aged 60 year or older, with increasing incidence over the last decades. It concerns most frequently reactive lymphoid hyperplasia, MALT and follicular lymphoma. Japan has a higher incidence of ocular adnexal MALT lymphoma, of which 10% is IgG4 producing.
Inflammatory symptoms such as eyelid erythema are present in 15%, and pain or irritation in 26% of the cases. Dry eye is not reported. Bilateral lacrimal gland lymphoma occurs in the region of 24% of the cases, and is associated with systemic disease. Imaging studies show a smoothly enlarged and enhanced lacrimal gland, with surrounding inflamed soft tissue (Fig. 4).
(Enlarge Image)
Figure 4.
Mucosa-associated lymphoid tissue lymphoma of the lacrimal gland. (Top left) This 49-year-old man had a recent history of painless right upper eyelid swelling and redness, along with pain in all joints. On examination, the eyelid was diffusely swollen without palpable mass. There was no dry eye. (Top right) His CT scan showed a diffusely enlarged lacrimal gland and eyelid infiltration. (Bottom left) Biopsy specimen of the lacrimal gland showed a dense B-cell-rich infiltrate, extending into the epithelium, with a few T cells. There was loss of acini and dilation of the ducts. The B cells were monomorphous, with abundant cytoplasm and round, hyperchromatic nuclei. PCR analysis detected a t(11;18) translocation and monoclonal rearrangement of the immunoglobulin heavy-chain (IgH) (hematoxylin and eosin staining, original magnification ×100). These findings were conclusive evidence of MALT lymphoma. He was staged IV based on additional bone marrow invasion. He responded well to a regimen with chemotherapy and rituximab. He developed a mild dry eye. (Bottom right) CT scan obtained 9 months later, demonstrating a discretely enlarged lacrimal gland. Repeat biopsies of the lacrimal gland mass showed dense fibrosis with normal glandular parenchyma, and those of the periglandular orbital fat showed mild fibrosis and inflammation. Ten years from onset, he is still in continuous complete remission. CT, computed tomography; MALT, mucosa-associated lymphoid tissue.
Treatment implies radiotherapy or tumor removal in localized unilateral disease, although, in the case of reactive lymphoid hyperplasia, observation after the biopsy may be sufficient. Localized bilateral lacrimal gland disease, more aggressive histologic subtypes, and systemic disease is treated in accord with the chemotherapy, immunotherapy, and radiotherapy protocols.