Stine Dahl Vest, Lauge Hjorth Mikkelsen, Frederik Holm, Geeta K. Vemuganti, Bradley A. Thuro, Steffen Heegaard
To characterize the clinical features of subtype-specific lacrimal gland lymphoma and their effect on patient survival.
Multicenter retrospective interventional case series.
Patient data were collected from 6 international eye cancer centers from January 1, 1980, through December 31, 2017. All patients with histologically verified primary or secondary lymphoma of the lacrimal gland were included. Primary endpoints were overall survival (OS) and disease-specific survival (DSS).
A total of 260 patients with lacrimal gland lymphoma were identified. The median age was 58 years and 52% of patients were men. Non-Hodgkin B-cell lymphomas constituted 99% (n = 258) and T-cell lymphomas constituted 1% (n = 2). The most frequent lymphoma subtypes were extranodal marginal zone B-cell lymphoma (EMZL) (n = 177, 68%), follicular lymphoma (FL) (n = 26, 10%), diffuse large B-cell lymphoma (DLBCL) (n = 25, 10%), and mantle cell lymphoma (MCL) (n = 17, 7%). Low-grade lymphomas (EMZL and FL) were most commonly treated with external beam radiotherapy (EBRT), whereas high-grade lymphomas (DLBCL and MCL) were treated with chemotherapy in combination with rituximab and/or EBRT. The prognosis was relatively good with a 5-year OS and DSS of 73.8% and 87.5%, respectively. Lymphoma subtype was a statistically significant predictor for DSS, with EMZL (5-year DSS: 93.4%) having the best prognosis and DLBCL (5-year DSS: 52.6%) having the poorest.
This is the largest reported collection of data of subtype-specific lacrimal gland lymphoma. The subtype distribution of lacrimal gland lymphoma resembles that of the ocular adnexa. Prognosis is good and the histologic subtype is a significant predictor for disease-specific survival.
Sabundayo, Maria Suzanne; Takahashi, Yasuhiro; Kakizaki, Hirohiko
A 69-year-old Japanese male presented with a 3-month history of bilateral ptosis with lacrimal gland bulge. He came from western Japan, an area endemic for human T-lymphotropic virus Type 1 infection. Physical examination and imaging revealed bilaterally enlarged lacrimal glands. Hematologic testing, biopsy with histopathology, and immunohistochemistry were all consistent with adult T-cell leukemia/lymphoma.
Daniele Lorenzano, MD, Geoffrey E. Rose, DSc, FRCOphth
Lacrimal gland carcinoma can form a triangle of tissue back to the orbital apex, intraconal spread apparently being prevented by the intermuscular septum. The “wedge sign” frequency is assessed in lacrimal carcinoma, lacrimal lymphoma, or dacryoadenitis.
Retrospective masked review of images from patients with biopsy-proven lacrimal gland pathology.
For each patient, the presence of a triangle of tissue between the lateral rectus and lateral orbital wall and the superior rectus and the orbital roof was assessed by masked review of computed tomography or magnetic resonance imaging. For the lateral compartment, the wedge was classified as “grade 1” if it just reached the sphenoidal trigone and “grade 2” if it was a complete triangle reaching the orbital apex. Comparison of proportions was made using the Fisher exact test, using an α risk of 0.05 as clinically significant.
Imaging for 116 patients was reviewed: 39 with lacrimal gland carcinoma, 37 with lymphoma, and 40 with dacryoadenitis. The lateral wedge (grade 1 or 2) was most common in patients with carcinoma (16/39; 41%), was present in 11 of 37 patients (30%) with lymphoma, and was rarest in patients with dacryadenitis (6/40; 15%) (P = 0.033). The proportion in patients with carcinoma (41%) was similar to that in patients with lymphoma (30%) (P = 0.345); the proportion in patients with lymphoma (30%) was similar to that in patients with dacryoadenitis (15%) (P = 0.170), but carcinoma (41%) was significantly different from dacryoadenitis (15%) (P = 0.013). The proportions for malignant lesions (carcinoma and lymphoma; 27/76, 36%) and benign dacryoadenitis (15%) were significantly different (P = 0.029). Likewise, a superior wedge, of any extent, was present in 10 of 39 carcinomas (26%), 2 of 40 dacryoadenitis (5%), and 6 of 37 lymphomas (16%) (P = 0.033).
The “wedge sign” is most common in lacrimal gland carcinoma, but can occur in patients with severe forms of dacryoadenitis or lymphoma and generally indicates life-threatening lacrimal gland pathology that requires urgent biopsy. It is significantly more common in lacrimal gland carcinoma compared with dacryoadenitis and in malignancy (lymphoma and carcinoma) compared with inflammation.