Lacrimal sac malignancy is a potentially life-threatening condition that mostly affects the elderly. It is rare and therefore there is limited data in the literature regarding its management and prognosis.1–3 We describe a case of lacrimal sac squamous cell carcinoma presenting in a 6-year-old boy. To the best of our knowledge, lacrimal sac squamous cell carcinoma has not been reported before at such a young age.
A 6-year-old boy presented with painless swelling around left medial canthus for 1 month. The patient also complained of watering from the same eye. He was diagnosed as having acute dacryocystitis and was prescribed systemic antibiotics earlier. However, the swelling did not respond to treatment and the patient was referred to our tertiary center for further treatment.
There was no history of discharge or bleeding from the nose or eye and no other systemic illnesses. On examination, the patient's best corrected visual acuity was 6/6 in the right eye and 6/9 in the left eye. Anterior and posterior segments were within normal limits. A 4 × 3.5 cm firm, immobile, non-tender, and non-fluctuant mass was seen medial to the medial canthus. The overlying skin was normal (Figure 1A). There was no regurgitation from the lacrimal puncta on pressing the mass. The ipsilateral postauricular lymph node was enlarged. A contrast-enhanced computed tomographic (CT) scan of the orbit and paranasal sinuses was performed and revealed a well-defined heterogeneous, hyperdense enhancing mass centered on the left lacrimal fossa (Figure 2A).
A 6-year-old boy with (A) left side swelling over the lacrimal sac area; (B) large areas of necrosis, hemorrhage, and islands of poorly differentiated squamous cell carcinoma (hematoxylin–eosin stain, original magnification ×100); and (C) higher magnification of the same (hematoxylin–eosin stain, original magnification ×400).
(A) Axial computed tomography (CT) image shows necrotic soft tissue mass at the medial canthus of the left orbit centered at the lacrimal fossa. Underlying bone was normal. (B) Follow-up CT (pre-chemotherapy) shows marked progressive enlargement of the mass with destruction of underlying lamina papyracea and its extension into the adjacent anterior ethmoid air cells. The globe shows lateral dystopia. (C) Follow-up CT (9 weeks after starting neoadjuvant chemotherapy) shows excellent response, indicated by significant size reduction.
Because malignancy was suspected, a fine needle aspiration biopsy was performed and it revealed a poorly differentiated tumor. The incisional biopsy performed showed large areas of necrosis, hemorrhage, and islands of poorly differentiated squamous cell carcinoma on histopathological examination (Figures 1B–1C). A diagnosis of lacrimal sac squamous cell carcinoma was made. However, after biopsy, there was a rapid increase in tumor size. A repeat contrast-enhanced CT scan of the orbit and paranasal sinuses showed a remarkable increase in the tumor's dimensions, with extension into the posterior orbit and adjacent anterior ethmoid air cells (Figure 2B).
In view of the extensive disease, neoadjuvant chemotherapy was initiated with paclitaxel (200 mg/m2) and carboplatin (using Calvert's formula) for three cycles. A good clinical response was noted initially, with an 80% reduction in tumor size and resolution of cervical lymphadenopathy (Figure 2C). However, the response was not sustained and the disease progressed after three treatment cycles. The patient's therapy was changed to ifosfamide (1.8 mg/m2 days 1 to 5) for two cycles. However, due to continued disease progression, his therapy was changed again to a four-drug oral metronomic regimen that included daily celecoxib and thalidomide alternating with etoposide and cyclophosphamide, and local radiotherapy was advised with palliative intent. However, the child died.
With approximately 775 cases of lacrimal sac tumors reported in the literature to date, 60% to 94% are primary and epithelial in origin. Approximately 40% to 90% of reported cases are malignant and have a mortality rate of 13.6% to 37.5% and a recurrence rate of 23% to 41%.1–8 In one case series, lymph node metastasis was reported in 27% and lung and esophagus metastasis in 9% of cases.6 Fatality in lacrimal sac malignancies correlates to the tumor extent and type. Tumors extending beyond the sac are associated with a higher mortality rate. Papillary carcinoma has a good survival prognosis, whereas transitional cell carcinoma is associated with the worst survival prognosis.3
Lacrimal sac tumors typically present in the fifth decade of life, with benign tumors presenting a decade earlier. In a clinicopathological study involving 115 lacrimal sac neoplasms, the authors reported an age range of 14 to 74 years for malignant lacrimal sac, with only 4 cases presenting before the age of 20 years.7 In another study involving 82 cases, malignant epithelial tumor of the lacrimal sac presented at a mean age of 58 years. The youngest patient in that study was 16 years old.6 Two additional cases of patients with lacrimal sac lymphoma younger than 18 years have been documented in the literature.9,10 To the best of our knowledge, our case is the youngest patient with lacrimal sac malignancy reported.
Diagnosis is often delayed due to its rarity, non-specific symptoms, and low index of suspicion. A dacryocystitis-like clinical presentation results in misdiagnosis and further delay in appropriate management. Also, the treatment is largely based on retrospective case series, and the most appropriate management strategy remains controversial. The treatment advocated for lacrimal sac malignancy is radical surgery followed by external beam radiotherapy. In cases with malignancy limited to the sac, dacryocystectomy with lateral rhinotomy for possible spread to the nasolacrimal duct is recommended. However, tumors with extension beyond the sac are treated with more aggressive surgeries including exenteration with lateral rhinotomy and sinus resection in cases with paranasal sinus involvement. In some cases, concurrent chemotherapy and radiotherapy are also performed. There are infrequent reports in the literature on the outcome of treating lacrimal sac malignancies with chemotherapy.4,8 One case series reported the successful use of neoadjuvant chemotherapy in tumor debulking in one case.8 In the current study, neoadjuvant chemotherapy was used in view of extensive involvement of the disease. It is notable that the tumor exhibited a remarkable but unsustained response to chemotherapy.
The current study highlights the fact that, although lacrimal sac malignancy is a neoplasm of old age, it can manifest in the pediatric age group. Therefore, ophthalmologists should be aware of the signs that may help diagnose this rare malignancy early. The clinical signs that indicate lacrimal sac tumors include a mass in the medial canthal area extending above the medial canthal tendon, bloodstained regurgitant fluid from the sac, epiphora, and nasal mass. Clinical features of sac inflammation (dacryocystitis) and/or secondary acquired nasolacrimal duct obstruction may be present even in lacrimal malignancies. A high index of suspicion and knowledge of clinical signs of lacrimal sac tumors is essential for all ophthalmologists treating childhood epiphora to help detect this rare malignancy early.
- Shields JA, Bakewell B, Augsburger JJ, Flanagan JC. Classification and incidence of space-occupying lesions of the orbit: a survey of 645 biopsies. Arch Ophthalmol. 1984;102:1606–1611. doi:10.1001/archopht.1984.01040031296011 [CrossRef]
- Heindl LM, Jünemann AGM, Kruse FE, Holbach LM. Tumors of the lacrimal drainage system. Orbit. 2010;29:298–306. doi:10.3109/01676830.2010.492887 [CrossRef]
- Krishna Y, Coupland SE. Lacrimal sac tumors: a review. Asia Pac J Ophthalmol (Phila). 2017;6:173–178. doi:10.22608/APO.201713 [CrossRef]
- Skinner HD, Garden AS, Rosenthal DI, et al. Outcomes of malignant tumors of the lacrimal apparatus: the University of Texas MD Anderson Cancer Center experience. Cancer. 2011;117:2801–28110. doi:10.1002/cncr.25813 [CrossRef]
- Montalban A, Liétin B, Louvrier C, et al. Malignant lacrimal sac tumors. Eur Ann Otorhinolaryngol Head Neck Dis. 2010;127:165–172. doi:10.1016/j.anorl.2010.09.001 [CrossRef]
- Ni C, D'Amico DJ, Fan CQ, Kuo PK. Tumors of the lacrimal sac: a clinicopathological analysis of 82 cases. Int Ophthalmol Clin. 1982;22:121–140. doi:10.1097/00004397-198202210-00010 [CrossRef]
- Stefanyszyn MA, Hidayat AA, Pe'er JJ, Flanagan JC. Lacrimal sac tumors. Ophthalmic Plast Reconstr Surg. 1994;10:169–184. doi:10.1097/00002341-199409000-00005 [CrossRef]
- El-Sawy T, Frank SJ, Hanna E, et al. Multidisciplinary management of lacrimal sac/nasolacrimal duct carcinomas. Ophthalmic Plast Reconstr Surg. 2013;29:454–457. doi:10.1097/IOP.0b013e31829f3a73 [CrossRef]
- Schefler AC, Shields CL, Shields JA, Demirci H, Maus M, Eagle RC Jr, . Lacrimal sac lymphoma in a child. Arch Ophthalmol. 2003;121:1330–1333. doi:10.1001/archopht.121.9.1330 [CrossRef]
- Carlin R, Henderson JW. Malignant lymphoma of the nasolacrimal sac. Am J Ophthalmol. 1974;78:511–513. doi:10.1016/0002-9394(74)90241-4 [CrossRef]