Lacrimal Sac Tumors

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General considerations

A variety of tumors can arise in the canaliculus, lacrimal sac, and tha nasolacrimal duct, with the mayority originating in the lacrimal sac.[1] Lacrimal sac tumors are usually primary and of epithelial origin. Overall, 55% of lacrimal sac tumors are malignant. Mortality rates for malignant sac tumors vary with stage and type, with a fatal outcome in approximately a third of patients in some series. [2][3]

The most common primary neoplasms of the lacrimal sac appear to be epithelial tumors, particularlly squamous papilloma and squamous cell carcinoma and Lymphoma (Non-Hogkins B cell)[4][3] Other tumors and pseudotumors that have been reported less often in the lacrimal drainage system include melanoma, oncocytoma, hemangiopericytoma, solitary fibrous tumor, peripheral nerve tumors, angiofibroma, granular cell tumor, cavernous hemangioma, pleomorphic adenoma, adenocarcinoma, and adenoid cystic carcinoma. Non-neoplastic conditions than can simulate lacrimal sac tumors include cyst, hematoma, pyogenic granuloma, and juvenile xanthogranuloma. However, because of their location, they usually have a different clinical presentation than those in conjuntiva or eyelid, and also are less visible.

Clinical features

Lacrimal sac tumors are rare compared to other orbital tumors. Early treatment of these tumors is important because they tend to be infiltrating tumors. Diagnosis of these tumors is often delayed because they are confused with dacryocystitis. Dacryocystitis is often associated with inflammatory signs, is soft and fluctuant, and often has a purulent discharge that can be expressed with pressure. Swelling typically does not extend above the medial canthal ligament. In contrast, a solid neoplasm generally appears in adults as a progressive, firm, subcutaneous mass, usually, but not always superior to the medial canthus. Secondary epiphora, sometimes tinged with blood.[1]

Bloody tears are common signs of various pathologies. Epistaxis, lacrimal gland tumors, vicarious menstruation, bleeding disorders such as hemophilia, trauma, orbital varix, canaliculitis and tumors of the lacrimal sac, and frequently, the malignant tumors have been reported as the causes of bloody tears. Benign lacrimal sac lesions with bloody tears are rare entities and detected incidentally during lacrimal sac surgery.[5]

Diagnostic approaches

tumors in the lacrimal sac are not readily visible and imaging studies, including computed tomography (ct), magnetic resonance imaging (mri), and dacryocystograms may provide more important information about the extent and nature of the lesion. most tumors are initially circumscribed, defined by walls of the lacrimal sac. later, the more agressive neoplasms lose their definition and become more diffuse or ill defined on imaging studies.

Pathology

lacrimal sac tumors may conveniently be grouped into epithelial and nonepithelial types: papillomas are the commonest benign epithelial tumours, while oncocytic adenomas and benign mixed tumours are rare. transitional cell papillomas consist of stratified columnar epithelium containing scattered goblet cells and cilia, squamous papillomas show acanthotic, stratified squamous epithelium (with occasional foci of dyskeratosis) resting on thickened basement membranelike material, and mixed cell papillomas show features common to both types; all can display an exophytic or endophytic growth pattern, the latter having a higher rate of recurrence or of malignant transformation. benign papillomas have a tendency to recur, especially where of inverted form with a 10–40% recurrence.[3]

lacrimal sac carcinomas are believed to arise de novo or, less commonly, from a preceding papilloma. 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.

squamous cell carcinomas can exhibit a wide range of differentiation atypical squamous cells (with abundant eosinophilic cytoplasm, pleomorphic nuclei, prominent nucleoli, and mitotic figures) showing invasion of deeper tissue layers.

transitional cell carcinoma resembles that of the urinary bladder, with pleomorphic cells and prominent nucleoli. adenoid cystic carcinoma can display a cribriform or basaloid pattern the former resembling ‘swiss cheese’, with sharply demarcated aggregates of small tightly packed malignant cells and rounded cystic foci.

mucoepidermoid carcinoma has both epidermoid and mucous-secreting cells with mucin-filled cystic spaces, while oncocytic adenocarcinoma shows oncocytic cells with nuclear atypia, arranged in an infiltrative pseudoglandular pattern.

Management

treatment of malignant tumors of the lacrimal sac depends for the most part on the histological type, the tumor extension, and the patient’s general health. either the diagnosis of tumor is defined preoperatively or it is discovered intraoperatively. in all cases, it is recommended to extemporaneously analyze the tumor biopsy.[3] the management of a lacrimal sac neoplasm varies with the clinical findings and suspected diagnosis. when possible, primary neoplasms are maneged initially by complete surgical removal by dacryocystectomy.

additional treatment may be required, depending on the final histopathologic diagnosis. more malignant epithelial tumors, lymphoma, and metastasis may require local irradiation or chemotherapy, depending on the specific diagnosis.

wide excision is important for papilloma, because lacrimal sac papilloma is invasive ans has high incidenceof recurrence. in advanced cases, orbital or sinusexenteration may be necessary. regardless of the diagnosis, subsecuent reconstruction of the lacrimal drainage system can be undertaken, provided there is no tumor recurrence within a few months. the prognosis varies with specific diagnosis.

Conclusion

in conclusion, it should be remembered that malignant tumors of the lacrimal sac are rare and often initially diagnosed as dacryocystitis. these tumors carry a vital risk and should be immediately managed with maximum care. treatment should be decided within a multidisciplinary team and close, long-term monitoring is indispensable.


References

  1. 1.0 1.1 Shields, Jerry A., and Carol L. Shields. "Tumors of the Lacrimal Drainage System." Eyelid, Conjunctival, and Orbital Tumors: Atlas and Textbook. Second ed. Philadelphia: Lippincott Wiliams & Wilkins, 2008. 232-41.
  2. Montalban, A., B. Liétin, C. Louvrier, M. Russier, J.-L. Kemeny, T. Mom, and L. Gilain. "Malignant Lacrimal Sac Tumors." European Annals of Otorhinolaryngology, Head and Neck Diseases 127.5 (2010): 165-72.
  3. 3.0 3.1 3.2 3.3 Parmar, D. N., and G. E. Rose. "Management of Lacrimal Sac Tumours."Eye 17.5 (2003): 599-606. Web.
  4. Dwivedi, Raghavc, Piyush Jani, Liam Masterson, Faruque Riffat, Alison Marker, Simona Woodruff, and Alistairw Hardy. "Inverted Papilloma of Lacrimal Sac Invading into the Orbit: Case Report and Review of Literature." J Can Res Ther Journal of Cancer Research and Therapeutics 11.1 (2015): 238.
  5. Demir, Helin Deniz, Erdinc Aydın, and Resit Doğan Koseoğlu. "A Lacrimal Sac Mass with Bloody Discharge." Orbit 31.3 (2012): 179-80.