The 3 most common cancers in children in the United States include leukemia(33%), brain tumors (20%), and non-Hodgkin lymphoma (10%).1 Non-Hodgkin lymphoma in children most commonly manifests in the abdomen (35%), anterior mediastinum (26%), and peripheral lymph nodes (19%). Less than 5% of such cases occur initially in the orbit.2 In a review of 250 pediatric orbital tumors, Shields and coworkers3 found 6 cases (2.4%) of orbital lymphoma, none of which were in the lacrimal sac. Using a MEDLINE search of the English language literature from 1970 to 2002, we identified 21 reported cases of primary non-Hodgkin lymphoma of the lacrimal sac, 4- 13 and only 1 patient was younger than 18 years.4 We report a case of a primary non-Hodgkin lymphoma of the lacrimal sac in a 10-year-old child.
A 10-year-old otherwise healthy Hispanic male had a 3-week history of painful swelling and epiphora in the left medial canthus (Figure 1). He recalled blunt trauma to the right side of his forehead 3 months earlier. On examination, his visual acuity was 20/25 OD and 20/30 OS. The only ocular abnormality was a firm, tender mass in the left lacrimal sac region straddling the medial canthal ligament and fixed to the orbital rim. There were no secretions from the lacrimal punctum, and epiphora without blood was noted. Lacrimal sac ultrasonography disclosed an acoustically hollow subcutaneous mass measuring 11 mm in base and 18 mm in height, consistent with a cystic mass (Figure 2). Orbital computed tomography revealed a solid mass involving the left lacrimal sac region, with associated effacement of the lacrimal sac fossa and bone remodeling(Figure 3). Based on these findings, the lesion was suspected to be a lacrimal sac cyst obstructing the nasolacrimal duct. Surgical repair revealed a firm, distended lacrimal sac. The opened sac disclosed a pink, solid mass conforming to the sac wall, and complete dacrocystectomy was performed.
External photograph demonstrates an inferomedial lacrimal sac fullness (arrow) causing lateral displacement of the left globe.
B-scan ultrasonography demonstrates a subcutaneous, nearly dumbbell-shaped sonolucent area with a dense wall (arrows at margins), consistent with a solid mass in the lacrimal sac region of the left eye.
Computed tomography of the left orbit demonstrates the solid, noncalcified lacrimal sac mass (arrow) with associated effacement of the lacrimal sac fossa and bone remodeling.
Histopathologic examination showed an intense diffuse infiltrate of atypical lymphocytes, consistent with extranodal marginal B-cell lymphoma of the mucosa-associated lymphoid tissue type in the stroma of the lacrimal sac (Figure 4). The infiltrate lacked germinal centers and contained prominent foci of monocytoid lymphocytes (Figure 5). The malignant cells expressed B-cell–specific antigens (CD20, CD79A) and coexpressed T-cell marker CD43 but lacked other T-cell–specific antigens (CD3, CD5). Polymerase chain reaction performed on paraffin-embedded tissue disclosed a clonal rearrangement of immunoglobulin heavy-chain genes.
Diffuse infiltrate of lymphoid cell compresses the lumen of the lacrimal sac (hematoxylin-eosin, original magnification ×25).
Monomorphic population of lymphocytes comprising infiltrate has a monocytoid appearance consistent with mucosa-associated lymphoid tissue lymphoma (hematoxylin-eosin, original magnification ×250).
Systemic evaluation revealed no findings of lymphoma. The patient was then treated with 2 cycles of chemotherapy, consisting of vincristine, cyclophosphamide, doxorubicin, and prednisone. Thirty months after diagnosis, he remains alive with no evidence of disease clinically or radiographically. He has no treatment-related complications and no persistent epiphora.
Nasolacrimal disorders are common in young children. Primary congenital nasolacrimal duct obstruction is the most frequent disorder of the lacrimal system, occurring in approximately 5% of all newborns. In infants, the obstruction is usually secondary to incomplete canalization of the valve of Hasner in the lower nasolacrimal duct.14 Secondary acquired nasolacrimal duct obstruction can also occur in children owing to infectious, inflammatory, traumatic, or mechanical causes.6 However, secondary infections or inflammatory causes most frequently accompany congenital nonpatent nasolacrimal systems. Nontraumatic spontaneous nasolacrimal duct obstruction in older children, as in our 10-year-old patient, is distinctly unusual.
Neoplasms of the lacrimal sac are uncommon, especially in children. They include epithelial (75%) and nonepithelial (25%) tumors, such as mesenchymal tumors (12%), melanoma (5%), and malignant lymphomas (<6%).10 The lacrimal sac may also be involved secondarily in patients with leukemia, particularly older patients with chronic lymphocytic leukemia.15 In a review by the Armed Forces Institute of Pathology, of 35 nonepithelial tumors of the lacrimal sac, 8 patients (23%) had malignant lymphoma of the lacrimal sac.10 The median age of these patients was 64 years, and the youngest patient was 39 years old.
Lymphoma in the periocular region generally originates in the conjunctiva or orbit.16,17 Lymphoma of the lacrimal sac, however, is unusual. Twenty-one cases of primary lymphoma of the lacrimal sac have been reported during the past 30 years (Table 1).4- 13 The median age at onset was 51 years, and only 1 case occurred in a child, similar to our case.4 The most common initial manifestations in these cases were epiphora and painless swelling of the lacrimal sac area(Table 1). The mean duration of epiphora prior to diagnosis was 9 months, and the mean duration of swelling was 2 months. Our case differed in that our patient had acute signs and symptoms, including pain, of 3 weeks' duration.
Imaging studies, such as computed tomography or magnetic resonance imaging can be helpful in the diagnosis of lacrimal sac lymphoma. In 9 reported cases, imaging was performed, and an isodense homogeneous mass was found. Six patients(66%) demonstrated effacement of the lacrimal sac fossa and/or erosion of the medial orbital wall (Table 1). In our case, computed tomography showed a solid mass, and ultrasonography misinterpreted it as a hollow mass, owing to the homogeneous density of the compact lymphoma. Erickson et al9 found magnetic resonance imaging to be superior to computed tomography for imaging tumors of the lacrimal sac, as magnetic resonance imaging provided better tumor definition and determination of the cystic or solid nature of the mass.
The management of lacrimal sac lymphoma remains controversial. In the 22 reported cases, all patients underwent incisional biopsies, and the most common treatment was external beam radiation, either alone (5 patients) or in combination with systemic chemotherapy (4 patients) or excisional biopsy(1 patient). Two patients (cases 5 and 20), and our patient (case 22), received excisional biopsy and systemic chemotherapy, with no external beam radiation treatment. Overall, the patients' prognoses were favorable, with 11 (85%) of 13 patients free of local recurrence and systemic disease at a mean of 24 months' follow-up. Because the clinical course of lymphoma is lengthy and indolent, our patient will require long-term follow-up. Four of the 9 patients who received external beam radiation developed postirradiation stenosis of the nasolacrimal duct, and 2 required dacrocystorhinostomy.4,8,9 One advantage of systemic chemotherapy alone may be avoidance of this complication, as demonstrated in our case.
In summary, we report an unusual case of a child with a lacrimal sac tumor that proved histopathologically to represent a non-Hodgkin B-cell lymphoma. We advise that older children with nontraumatic nasolacrimal duct obstruction undergo imaging studies of the lacrimal sac to rule out a solid tumor. Patients with this diagnosis appear to have a favorable prognosis with a variety of treatment approaches. Systemic chemotherapy rather than radiotherapy may avoid potential persistent epiphora due to radiation-induced lacrimal duct stenosis.
This research was supported by the Paul Kaiser International Award of Merit in Retina Research (Dr J. Shields), the Rosenthal Award of the Macula Society (Dr C. Shields), the Noel T. and Sara L. Simmonds Endowment for Ophthalmic Pathology, Wills Eye Hospital, Philadelphia, Pa (Dr Eagle), and the Eye Tumor Research Foundation, Philadelphia (Dr C. Shields).
The authors have no relevant financial interest in this article.
Corresponding author and reprints: Carol L. Shields, MD, Ocular Oncology Service, Wills Eye Hospital, 840 Walnut St, Philadelphia, PA 19107.
Schefler AC, Shields CL, Shields JA, Demirci H, Maus M, Eagle RC. Lacrimal Sac Lymphoma in a Child. Arch Ophthalmol. 2003;121(9):1330-1333. doi:10.1001/archopht.121.9.1330