three squamous papilloma, two lymphoma, one leukemia) [3]; in 31 of 377 specimens (8.2%) in Anderson and coworkers’ series (eight sarcoidosis, seven lymphoma, four papilloma, four lymphoplasmacytic infiltrate, two transitional cell carcinoma, one oncocytoma, one granular cell tumor, one adenocarcinoma, one poorly differentiated carcinoma, one plasmacytoma, one leukemia) [1]; and in 3 of 193 specimens (1.6%) in Merkonidis et al.’s series (two sarcoidosis, one transitional cell papilloma) [15]. No specific pathologies could be observed in 44 biopsy specimens by Mauriello et al. [13] and in 202 specimens by Lee-Wing and Ashenhurst [11].

In summary, the risk of overlooking significant pathologies in selective lacrimal sac biopsy can be minimized by detailed medical history, comprehensive clinical examination, and intraoperative inspection of the lacrimal sac during external DCR.

6.5Definitive Treatment and Prognosis

of Lesions A ecting the Lacrimal Drainage System

Depending on the histopathologic diagnosis of the lacrimal sac biopsy, the anatomic extent of the lesion, and the outcome of clinical staging, further definitive treatment is individual and often multidisciplinary, including orbital exenteration, lateral rhinotomy, chemotherapy, radiotherapy, immunotherapy (e.g., interferon alpha), or systemic immunosuppression (Table 6.4). The prognosis for patients with lacrimal sac lesions depends on the pathologic characteristics of the process, the stage at which

diagnosis is made, and the effectiveness of the treatment. However, long-term prognosis remains uncertain due to the paucity of reports with long-term follow-up. Therefore, no evidence-based clinical practice guidelines exist on the therapy of lesions affecting the lacrimal drainage system.

6.5.1Case A

A 39-year-old female presented with epiphora and a right-sided firm lacrimal sac mass of 6-month duration (Fig. 6.3a). Detailed inspection of the lacrimal sac during external DCR revealed abnormal swelling. Incisional biopsy with surgical debulking demonstrated a primary MALT lymphoma of the lacrimal sac (Fig. 6.3b). The patient was treated successfully with radiotherapy (43 Gy), with no sign of local recurrence or systemic disease at 6-year follow-up.

6.5.2Case B

Following an episode of dacryocystitis, a 56-year-old female with 8 months of epiphora was found to have a firm, incompressible medial canthal mass (Fig. 6.4a). External DCR with incisional biopsy disclosed an extensive squamous cell carcinoma (Fig. 6.4b). Further therapy included dacryocystectomy, excision of periosteum and nasolacrimal duct using lateral rhinotomy, radio- (59Gy) and chemotherapy (5-fluoruracil and cisplatin). Within 1 year after surgery, no local recurrence or metastatic disease could be observed.

A 68-year-old female presented with a 10-month history of right-sided epiphora, bloody tears, and medial canthal mass (Fig. 6.5a). CT revealed a soft tissue mass of the right lacrimal sac with widening of the bony nasolacrimal canal (Fig. 6.5b). A transcutaneous incisional biopsy confirmed the diagnosis of malignant melanoma (Figs. 6.5c and d). After staging, further therapy included orbital exenteration, lateral rhinotomy with en bloc resection of lacrimal drainage apparatus and adjuvant radioimmunotherapy. One year after surgery, no evidence of local recurrence or metastatic disease could be detected [9].

A 67-year-old female attended with an 8-month history of right-sided epiphora and recurrent dacryocystitis and having noticed a mass inferior to the medial canthus (Fig. 6.6a). Nasal space was unremarkable. Coronal CT scan revealed a circumscribed mass limited to the lacrimal sac and upper portions of the nasolacrimal duct (Fig. 6.6b). Lacrimal sac biopsy disclosed a benign oncocytoma (Figs. 6.6c and d). Excision of the whole mass was attempted using dacryocystectomy combined with canaliculorhinostomy and silicone tube intubation. No local recurrence could be seen within a follow-up of 5 years (including nasal endoscopy) [10].

6.5.5Case E

Six months after endonasal DCR, a 63-year-old female was referred due to persistent epiphora and recurrent dacryocystitis (Fig. 6.7a). External DCR demonstrated a prominent mass of the lacrimal sac. Excisional biopsy revealed a pyogenic granuloma (Fig. 6.7b). Seven years after surgery, the patient reported complete resolution of the preoperative symptoms with a patent lacrimal drainage system on clinical irrigation.

6.5.6Case F

A 70-year-old male with the history of Wegener granulomatosis presented with bilateral epiphora and recurrent dacryocystitis for 8 months (Fig. 6.8a). External DCR with incisional biopsy of the lacrimal sac and nasal mucosa showed necrotizing vasculitis with granuloma-

tous inflammation (Fig. 6.8b). After bilateral external DCR with silicone tube intubation and control of the systemic disease with endoxane and cyclosporine, the patient was free of symptoms and local recurrence within a follow-up of 32 months.

6.5.7Case G

Following bilateral endonasal DCR for epiphora and dacryocystitis, 12 months later a 65-year-old female with the history of sarcoidosis was referred with recurrent bilateral dacryocystitis (Fig. 6.9a). The patient underwent bilateral external DCR with silicone tube intubation and—due to granulomatous inflammation compatible with active sarcoidosis in the incisional biopsy specimens from the lacrimal sac and nasal mucosa (Fig. 6.9b)—immunosuppressive treatment. The patient remained recurrence free at 1 year of follow-up.