Inverted Papilloma Lacrimal Sac
Inverted papilloma of the lacrimal sac (IP) is a benign sinonasal tumor with potential for malignant transformation. IP is a rare tumor comprising 0.5-4% of all sinonasal neoplasms.
IP has a median age of onset of 34.5 years with a slight female predisposition (M:F 1:1.2). It has a predilection for the lateral nasal wall followed by the maxillary sinus, and ethmoid sinus. While rare, IP can also occur in the frontal and sphenoid sinus, nasopharynx, oropharynx and middle ear.
IP is not a primary lacrimal drainage system tumor but a primary tumor of the tissue surrounding the lacrimal drainage system and spreads to the lacrimal sac via secondary extension. It is exceedingly rare for IP to originate from the lacrimal sac, having been reported only 6 times in the past 30 years. It is a benign lesion with potential for malignant transformation (squamous cell carcinoma, transitional cell carcinoma) in 5-11.5% of cases. Malignant transformation is usually preceded by atypia on histology. IP arises from the sinonasal respiratory epithelium, also known as the Schneiderian epithelium, lining the lateral nasal wall and paranasal sinus which develops from the olfactory placode as an invagination of ectoderm.
Sinonasal papillomas grow via 3 patterns:
- Exophytic – fungiform growth
- Inverted – downward extension into mucosa in an inverted fashion
Human Papilloma Virus (HPV) subtypes 6 and 11 and Epstein Barr Virus (EBV) have been identified in different cases. When present, cases with HPV tend to have a higher likelihood of malignant potential. Polymerase chain reaction (PCR) and In situ hybridization (ISH) can be used to detect the presence of HPV in biopsy specimens.
Patients usually present with epiphora or dacryocystitis. On examination, patients will often have a mass below the medial canthal tendon and may have ulceration or telangiectatic vessels at the medial canthus. Irrigation of the lacrimal system may cause bloody reflux. Rarely will patients present with diplopia or visual impairment. In some cases, IP may be detected incidentally during dacryocystorhinostomy for nasolacrimal duct obstruction, or a papilloma may present from the punctum.
CT is the imaging modality of choice, especially given the tendency for bony erosions of the nasal and maxilla bone. CT will often show a unilateral, polypoid mass in the nasal cavity extending into the maxillary and ethmoid sinus. Tissue biopsy is necessary to make a final diagnosis.
The differential diagnosis includes:
- Secondary lacrimal extension from any benign or malignant sinonasal tumor
- Squamous cell carcinoma and basal cell carcinoma of the eyelid skin with extension
- Adenoid cystic carcinoma
- Epidermoid carcinoma
- Lymphoma (Non-Hodgkins B cell)
- Solitary fibrous tumor
- Other metastatic tumors
- Lacrimal sac cyst
- Lacrimal sac hematoma
- Pyogenic granuloma
- Juvenile Xanthogranuloma
Treatment is primarily surgical with a goal of total resection with clear margins as this tumor has a high recurrence rate (reported as high as 71%) post-surgery. The inverted growth pattern into the lacrimal sac wall makes excision difficult and often incomplete. Historically this was achieved with a medial maxillectomy with midface degloving or a craniofacial approach. With isolated lacrimal disease a dacryocystectomy may be sufficient. Recently with advances of endoscopic surgery, a multidisciplinary approach between Otolaryngology and Ophthalmology where endoscopic sinus surgery, argon laser treatment and orbitotomy with excision is sufficient for select lesions with orbital involvement. Orbital exenteration with radiotherapy has been reported for lesions that have achieved malignant transformation. Additional therapies that have been reported include interferon alpha-2b and irrigation of the lacrimal system with mitomycin C.
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