Primary non Hodgkin's lymphoma of the lacrimal sac
World Journal of Surgical Oncology
Primary non Hodgkin's lymphoma of the lacrimal sac Ramachandran Venkitaraman1 and Mathew K George*2
0 Division of Medical Oncology, Royal Darwin Hospital , Casuarina , Australia
1 Division of Clinical Oncology, Royal Marsden Hospital , Sutton, Surrey, SM2 5PT , UK
Background: Primary Non Hodgkin's Lymphoma (NHL) of the lacrimal sac is rare. Methods: The clinical features of a 78 year old female who presented with epiphora and swelling of the left lacrimal sac are described. Results: Computerised tomography showed a mass involving the left lacrimal sac. Histopathological examination revealed a diffuse large B cell NHL. Immunohistological examination demonstrated B cell origin. Chemotherapy could not be administered due to co morbid conditions. The patient was treated with radiotherapy to a dose of 45 Gy in 25 fractions. Patient is disease free and on follow up after 36 months. Conclusion: Primary radiotherapy is a treatment option with curative potential for localized NHL of the lacrimal sac and may be considered in patients who cannot tolerate appropriate chemotherapy.
Background
Tumors involving the nasolacrimal drainage system are
rare and more than 90% of these tumours are of epithelial
origin [
1
]. Majority of lymphomas involving the lacrimal
sac are secondary to systemic lymphoreticular malignancy
[
1
]. Primary non Hodgkin's lymphoma (NHL) of the
lacrimal sac is a rare neoplasm [
2-6
]. Therefore, the
clinicopathological features and optimal management of
primary lacrimal sac NHL are not well characterized.
In this report, we present the clinical features of a patient
with primary NHL of the lacrimal sac treated successfully
with radiotherapy.
Case presentation
A 78 year old female patient presented with epiphora of
the left eye of 6 months duration and a painless swelling
of the left lacrimal sac area of 2 months duration. Physical
examination showed a firm, non-tender mass measuring
3 × 4 cm in the right lacrimal fossa. Irrigation test revealed
complete obstruction of the lacrimal drainage system.
Rest of the eye and adnexae were normal. Computerised
tomography (CT) scan of the orbit showed a
heterogeneous soft tissue mass arising from the lacrimal sac,
infiltrating and pushing the globe laterally and forward, causing
exophthalmos with no bony erosion (figure 1). There was
homogenous and intense enhancement with contrast.
An incision biopsy was performed from the mass and
pathological examination of revealed diffuse large cell
lymphoma. Immunohistochemistry demonstrated
malignant clonal cells of B cell origin (CD20 positive), lacking
T cell antigens. Physical examination, biochemical tests,
CT scan of the chest and abdomen, and bone marrow
sFCaiocgmuturpmeuto1eurrisewditThocmonotgrraaspthenohf atnhceeomrebnitt showing the lacrimal
Computerised Tomograph of the orbit showing the lacrimal
sac tumour with contrast enhancement.
biopsy did not show evidence of lymphomatous
involvement elsewhere. The patient had diffuse large B cell Non
Hodgkin's lymphoma of the right lacrimal sac, stage IE by
the Ann Arbor staging system and IPI score of 2 (low
intermediate).
The patient was treated with radiotherapy to a dose of 45
Gy in 25 fractions over 5 weeks by three dimensional
conformal lens sparing technique. Systemic chemotherapy
was not proposed in view of the advanced age of the
patient, poor performance status and concurrent co
morbidities. The patient continues to be on follow up after 36
months with no evidence of local or systemic recurrence
and preservation of visual acuity.
Discussion
Primary lymphoma of the lacrimal sac is rare, with most
reported cases representing secondary involvement from
systemic lymphoproliferative malignancy [
1
]. Only 16 of
212 lacrimal sac tumors described by Flanagan and Stokes
were lymphoreticular tumors [
1
]. In largest cumulative
series of nonepithelial tumors of the lacrimal sac, Pe'er at
al found that 8 of 35 cases were primary lymphoma [
2
].
The typical presentation of a lacrimal sac tumour is as a
medial canthal swelling with nasolacrimal duct
obstruction. The differentials for a lacrimal sac mass include
nongranulomatous inflammation and granulomatous
infections, dermoid cyst, mucocele, lipoma,
lymphangioma and tumors of the lacrimal duct like
adenocarcinoma, squamous cell carcinoma, adenoid cystic
carcinoma, hemangiomas, sarcomas, melanoma,
lymphoma and secondaries.
Nasolacrimal sac tumors may arise within the sac or from
structures surrounding the sac or duct. In previous reports
of primary NHL of the lacrimal sac, Jordan and Nerad [
7
]
observed a lacrimal sac mass extending into the
nasolacrimal duct, Saccogna et al [
8
] demonstrated CT findings of
a large mass involving the nasolacrimal duct area and the
adjacent paranasal sinuses and Erickson et al [
9
] treated
several patients with lymphomatous infiltration of the
nasolacrimal canal.
The vast majority of NHLs of the orbit and ocular adnexa
ar (...truncated)