Cancer of the Lacrimal Sac

Abstract

Tumors of the lacrimal sac are rare, and are discussed primarily in the ophthalmological literature. The otolaryngologist, however, often performs the definitive surgery and must, therefore, be familiar with this disease. The lacrimal apparatus secretes and then drains lubricating fluid from the eye. This report focuses on the drainage mechanism which is anatomically and functionally a single structure composed of the canaliculi, the lacrimal sac, and the nasolacrimal duct. By 1963, 184 lacrimal sac tumors had been reported in the world literature. We have collected an additional 21 patients from the literature. This brings the total malignant tumors to 125, of which 74 were of epithelial origin. One sarcoma and four poorly differentiated epidermoid tumors treated in our department are presented in detail. The diagnosis is often evasive though the history of mass and epiphora is typical. Conservative treatment for dacryocystitis only temporizes. Work-up should include external and slitlamp examination, complete rhinological evaluation, sinus x-rays, tomograms of the bony lacrimal sac area, and dacryocystograms. Biopsy gives pathological confirmation. The largest group is epidermoid carcinoma, mostly of the poorly differentiated nonkeratinizing type. The treatment for benign lesions is local excision. Preoperative irradiation is indicated for epidermoid carcinoma, followed by wide local excision. Radical maxillectomy may be reserved for recurrences, and neck dissection for palpable nodes may be helpful. Mesenchymal tumors respond best to radiotherapy. Death in lacrimal sac cancer results from metastases, most often to the neck and lung. Five year survival rates appear to be slightly greater than 50%.

Management of lacrimal sac tumours

“…3,8 In our series of 15 patients, 11 (73%) were female and the median age at presentation was 59 years (range 22-94 years).…”

“…1,3 Dacryocystography in the presence of an intrinsic tumour often shows a filling defect of the sac lumen (Figure 2a) or a distended sac with uneven or mottled contrast media. Orbital and sinus CT is imperative where tumour is suspected and will provide evidence of expansion or erosion of the lacrimal sac fossa, or invasion into neighbouring structures (Figures 2b and 3b).…”

“…2,4 Squamous and transitional cell carcinomas are the most common, but other types include adenocarcinoma, oncocytic adenocarcinoma, mucoepidermoid, poorly differentiated, and adenoid cystic carcinoma; carcinomas have a quoted recurrence rate of 50% (mortality increasing with recurrence), and 37.5% mortality has been reported after wide surgical excision and radiotherapy. 1,3 Squamous cell carcinomas (cases B and F) can exhibit a wide range of differentiationFatypical squamous cells (with abundant eosinophilic cytoplasm, pleomorphic nuclei, prominent nucleoli, and mitotic figures) showing invasion of deeper tissue layers ( Figure 1b). Transitional cell carcinoma (cases C and H) resembles that of the urinary bladder, with pleomorphic cells and prominent nucleoli (Figure 2c).…”

“…[1][2][3][4][5][6] Mesenchymal primary tumours, such as fibrous histiocytoma, fibroma, haemangioma, haemangiopericytoma, angiosarcoma, or lipoma, are less common (14%) and the rarer tumours include lymphomas (8%), malignant melanomas (4%), and neural tumours (1%). 6,7 Secondary tumours may originate either from adjacent structures, such as the paranasal sinuses and orbit, or as metastasesFalthough the latter are rarely confined to the lacrimal sac alone.…”

“…6,7 Secondary tumours may originate either from adjacent structures, such as the paranasal sinuses and orbit, or as metastasesFalthough the latter are rarely confined to the lacrimal sac alone. 3 Inflammatory lesions, such as nonspecific chronic inflammation ('pseudotumour') or granulomatous disease, are not neoplasms but may present as lacrimal sac masses.…”