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Br J Ophthalmol 97:106 doi:10.1136/bjophthalmol-2012-302454
  • Education

Lacrimal sac mucocele

  1. Raman Malhotra3
  1. 1Corneo Plastic Unit, Queen Victoria Hospital, East Grinstead, West Sussex, UK
  2. 2Department of Pathology, UCL Institute of Ophthalmology, London, UK
  3. 3Corneo-Plastic Unit, Queen Victoria Hospital NHS Trust, East Grinstead, UK
  1. Correspondence to Raman Malhotra, Corneo Plastic Unit, Queen Victoria Hospital, East Grinstead, West Sussex, RH19 3DZ, UK; raman{at}ramanmalhotra.com

Case

An 84-year-old Caucasian lady was referred to the clinic with a 4-month history of a non-tender right medial canthal swelling (figure 1). This was associated with initial soreness and discharge only. There was no associated epiphora. Her systemic medications included aspirin and calcium supplements. Visual acuity was 6/7.5 in each eye. Clinical examination was suggestive of right chronic dacryocystitis with a non-regurgitating mucocele. Probing demonstrated a complete obstruction at the level of common canaliculus on the right side with regurgitation of saline from upper punctum on syringing. The left lacrimal drainage system was patent on syringing. Endoscopic endonasal examination was normal. Dacryocystography or lacrimal scinitigram was not performed. A diagnosis of encysted mucocele secondary to chronic dacryocystitis was made. She was advised to undergo dacryocystectomy (DCT) under local anaesthesia as she did not have symptoms of epiphora. The excised lacrimal sac was sent for histological examination (figure 2).

Figure 1

Swelling in the medial canthal area (arrow showing lacrimal sac mass).

Figure 2

Lacrimal sac wall with a dense stromal lymphoid infiltrate (H&E, ×4 objective). Inset: High power view demonstrating centrocyte-like morphology (H&E, ×40 objective).

Questions

  1. What does the histological appearance show (figure 2)?

  2. What clinical features are suggestive of a lacrimal sac tumour?

  3. What investigations can be performed when suspecting a lacrimal sac tumour?

  4. What are the management options?

For answers see 113

Answers

For questions see 106

What does the histological appearance show?

The lacrimal sac wall has a dense nodular lymphoid infiltrate with no distinct germinal centres (figure 2). The infiltrating cells are small and centrocyte-like. They were immunoreactive for CD20, CD79a, Bcl-2, Bcl-6, IgM and IgD, but not for CD3, CD5, CD10 or cyclin D1. CD23 was expressed in neoplastic cells and highlighted disrupted follicular dendritic cell networks. Ki-67 demonstrated a proliferation fraction of 10%. The immunophenotype was not typical for a specific lymphoma, and both follicular and extranodal marginal zone lymphoma were considered. The cytomorphology, lack of follicular architecture and lack of CD10 expression favoured a diagnosis of extranodal marginal zone lymphoma.

What features are suggestive of a lacrimal sac tumour?

The most common presenting symptoms and signs associated with lacrimal sac neoplasms include epiphora, recurrent dacryocystitis and lacrimal sac mass.1 A painless, non-reducible and firm mass, especially if it extends above the medial canthal tendon, is most suspicious of a tumour.2 Pain and bleeding from puncta or nose is rare. An irreducible firm swelling in the medial canthal area is likely to be a lacrimal sac tumour; however, an encysted mucocele due to impacted valve of Rosenmuller can have a similar presentation. As the common presenting features are non-specific and mimic dacryocystitis, further investigations are required to reach a diagnosis in patients with a suspicion of lacrimal sac tumour.

What investigations can be performed when suspecting a lacrimal sac tumour?

CT scan and MRI are most helpful in evaluating the lacrimal sac and, perhaps less of value, dacryocystography in the presence of an intrinsic tumour often shows a filling defect of the sac lumen or a distended sac with uneven or mottled contrast media.3 CT findings suggestive of a tumour include soft tissue mass of the lacrimal sac or adjacent paranasal sinuses. It allows evaluation of bone, demonstrating bony erosion, nasolacrimal duct or canal enlargement as well as extension of the mass into surrounding tissues. However, a diffuse involvement of sac will be seen as a soft tissue swelling seen in any cause of chronic dacryocystitis. Systemic examination (including regional lymphadenopathy, endonasal examination), chest x-ray (or CT) and haematological tests (full blood count, liver function tests) should be undertaken when a lacrimal sac tumour is suspected.4 A biopsy of the lacrimal sac is the most important diagnostic tool in these cases.

What are the management options?

In cases with high clinical suspicion of a lacrimal sac tumour, an incisional biopsy (even under frozen section) followed by DCT and subsequent definitive therapy including radical resection, radiotherapy or chemotherapy is advocated.5 ,6 In cases with low clinical suspicion of a tumour, an open dacryocystorhinostomy (DCR) with biopsy of any abnormal tissue can be performed. Locally invasive tumours, such as papillomas, may be readily monitored for recurrence by nasal endoscopy, while aggressive tumours may be treated with radical surgery or radiotherapy.3

There was no clinical suspicion of tumour in our patient and a DCT was performed as she did not have symptoms of epiphora. DCT is a relatively quick procedure, easily performed under local anaesthesia and less invasive when compared with a DCR. It eliminates the lacrimal sac swelling and is an ideal option for elderly patients with discharge but no epiphora, or those with significantly dry eyes. In cases with low suspicion for malignancy, external DCR with biopsy of any abnormal tissue can be performed.

Our patient was referred to the haematologist for further management. Systemic examination and a CT scan of chest and abdomen did not detect any abnormality. Bone marrow biopsy was not considered due to her advanced age. As there was no residual swelling palpable at the medial canthal area following DCT, neither radiotherapy nor chemotherapy was recommended and she remains asymptomatic under review.

Discussion

Tumours of the lacrimal sac are rare and often initially diagnosed as dacryocystitis. Primary lacrimal sac tumours can be broadly divided into epithelial, lymphoid, mesenchymal and melanoma of which epithelial tumours are by far the commonest1 (box 1).

Box 1 Tumours of the lacrimal sac (histopathological classification)

Epithelial tumours

  • Benign

  • Papilloma: squamous, transitional

  • Oncocytoma

  • Benign mixed tumours

  • Malignant

  • Squamous carcinoma

  • Transitional carcinoma

  • Adenocarcinoma

  • Oncocytic adenocarcinoma

  • Mucoepidermoid

  • Adenoid cystic carcinoma

  • Papilloma with carcinoma

Non-epithelial tumours

  • Mesenchymal

  • Fibrous histiocytoma

  • Haemangiopericytoma

  • Haemangioma

  • Lipoma

  • Neurofibroma

  • Haemopoietic tumours

  • Lymphoma

  • Granulocytic tumours

  • Melanoma

  • Granulocytic sarcoma

Primary lymphoma of the lacrimal sac is rare,5 with most reported cases representing secondary involvement from systemic lymphoproliferative malignancy. Lacrimal sac involvement can be a primary site or a metastatic lesion of a systemic lymphoma owing to the presence of rich plexus of lymphatics and blood vessels surrounding the lacrimal sac.

In Europe, extranodal marginal zone B cell lymphomas of the mucosa-associated lymphoid tissue (MALT lymphoma) and diffuse large B cell lymphoma (DLBCL) are major lymphomas of the lacrimal sac.5 MALT lymphomas are low grade lymphomas whereas DLBCL are high grade tumours that run an aggressive course. Localised disease can be treated successfully with surgery, radiation therapy and chemotherapy.5

Our patient had a MALT lymphoma with no evidence of systemic spread. As DCT had removed the primary tumour load, no further resection was carried out and she remains asymptomatic under review.

Footnotes

  • Contributors The authors contribution included acquisition of data, drafting the article and final revision for publication.

  • Competing interests None.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

References

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