|Year : 2015 | Volume
| Issue : 1 | Page : 238-240
Inverted papilloma of lacrimal sac invading into the orbit: Case report and review of literature
Alistair W Hardy1, Raghav C Dwivedi1, Liam Masterson1, Faruque Riffat1, Alison Marker2, Simon A Woodruff3, Piyush Jani1
1 Department of Otolaryngology, Head and Neck Surgery, Addenbrookes Hospital, Cambridge, United Kingdom
2 Department of Histopathology, Addenbrookes Hospital, Cambridge, United Kingdom
3 Department of Ophthalmology, Addenbrookes Hospital, Cambridge, United Kingdom
|Date of Web Publication||16-Apr-2015|
Raghav C Dwivedi
Department of Otolaryngology, Head and Neck Surgery, Addenbrooke's Hospital, Cambridge University Teaching Hospitals NHS Foundation Trust, Cambridge, CB2 0QQ
Source of Support: None, Conflict of Interest: None
Clinical trial registration PDCH/12/PU11-1173
Inverted papilloma (IP) is a sinonasal tumor of benign etiology with local invasion and malignant potential. IP arising in lacrimal sac invading the orbit is extremely rare with only one case reported so far. The presented case appears to be the second such case reported in the literature. A 60-year-old Caucasian male presented with a medial canthal mass and epiphora. Incisional biopsy confirmed a transitional neoplasm. The lesion was completely excised enbloc with clear margins by using a Weber-Ferguson incision; orbital clearance and nasolacrimalfossa clearance was achieved via a medial maxillectomy. Enbloc resection of orbital and nasolacrimal parts of the tumor with clear margins is recommended.
Keywords: Inverted papilloma, lacrimal sac, lacrimal duct, nasal cavity, orbit, paranasal sinus
|How to cite this article:|
Hardy AW, Dwivedi RC, Masterson L, Riffat F, Marker A, Woodruff SA, Jani P. Inverted papilloma of lacrimal sac invading into the orbit: Case report and review of literature. J Can Res Ther 2015;11:238-40
|How to cite this URL:|
Hardy AW, Dwivedi RC, Masterson L, Riffat F, Marker A, Woodruff SA, Jani P. Inverted papilloma of lacrimal sac invading into the orbit: Case report and review of literature. J Can Res Ther [serial online] 2015 [cited 2022 Jun 29];11:238-40. Available from: https://www.cancerjournal.net/text.asp?2015/11/1/238/155111
Alistair W. Hardy and Raghav C. Dwivedi contributed equally for this work
| > Introduction|| |
Inverted papillomas (IPs) are rare, locally aggressive benign neoplastic lesions with malignant potential. They are known to arise from the sinonasal respiratory epithelium or the Schneiderian epithelium. Embryologically, this epitheliumis derived from the ectoderm of the invaginating olfactory placode.  They are notorious for local invasion and high recurrence rates post-surgery secondary to rapid endophytic proliferation/invagination of the cells located along the basement membrane of the epithelium into the stroma with an intact basement membrane. IPs constitute 0.5-4% of all primary sinonasal neoplasm.  The most common primary site of origin is the lateral nasal wall, followed by the maxillary and the ethmoid sinuses. Rarely, IP can occur in the frontal sinus, sphenoid sinus, nasopharynx, oropharynx, and the middle ear. 
Theoretically nasolacrimal system can be secondarily involved in the disease process by local extension along the nasolacrimal bony canal or by erosion of the bone and extension directly into nasolacrimal system.  Lacrimal sac as a primary site of origin for IPs is rather very unusual with only a few cases reported in the literature [Table 1]. Moreover, IP originating primarily in the lacrimal sac and invading the orbit is an extreme rarity with just one such case reported to date. We present the second such case to be reported in the literature.
|Table 1: Reported cases of inverted papilloma arising primarily in lacrimal sac|
Click here to view
| > Case report|| |
A 60-year-old man presented to the clinic after noticing a swelling on the inner side of his right eye of 4 weeks' duration. He also complained of discomfort in the inner side of his eye and epiphora. There was no history of direct trauma to the eye; however, interestingly he gave a history of sustaining a fall at work which drew his attention to the swelling. There was no history of diplopia, opthalmoplegia, visual disturbances, nasal obstruction, epistaxis, rhinorrhea, recurrent sinonasal infection, or headaches. Patient is a nonsmoker and drinks only socially; there is no history of working with the wood dust.
Past medical history was significant for right tonsillar squamous cell carcinoma (SCC) stage T4N2aM0, diagnosed 8 years ago and treated by radical neck dissection and chemoradiotherapy. Patient was deemed disease free after active post treatment surveillance for 5 years. In addition, the patient was diagnosed to have plasmablastic lymphoma of the left mandible 4 years ago and treated with chemotherapy (CHOP regimen). The lymphoma is in remission.
Examination revealed a 2 cm × 2 cm, firm, fixed mass at the right medial canthus abutting the lower and medial orbital rim. There was no bony erosion of the orbital rim and the overlying skin was freely mobile. Occular moments were normal in all gazes without any diplopia. Flexible nasendoscopic examination confirmed patent nasal cavities without any mucosal abnormality or mass lesion. Detailed endoscopic examination of nasopharynx, oropharynx, larynx, and hypopharynx was unremarkable.
There were no other palpable masses or any lymphadenopathy in the head and neck region. Systemic examination did not reveal and lymphadenopathy, icterus, or organomegaly.
An incision biopsy was undertaken and histology confirmed a transitional neoplasm. Microscopy showed conjunctival epithelium continuous with a papillary neoplasm composed of stratified transitional epithelium [Figure 1]. Computerized tomography (CT) revealed a soft tissue mass in the right medial can thus measuring 24 × 19 mm. There was no bony erosion, but did show tumor invasion into the orbit with upward and lateral displacement of the globe [Figure 2]. Staging CT neck and chest were performed in view of his two previous malignancies and they were negative. Thus, a diagnosis of IP was made and complete excision was recommended.
|Figure 1: Hematoxylin and eosin stained sections of the tumor showing hyperplastic ribbons of epithelium with endophytic growth pattern ((a) low magnification, ×4) and multilayered epithelium of transitional type ((b) high magnification, ×20)|
Click here to view
|Figure 2: Computed tomography showing the mass of the tumor within the orbit with minimal extension in the nasolacrimal apparatus. (a) Axial view and (b) coronal view|
Click here to view
The lesion was completely excised enbloc by using a Weber-Ferguson incision; orbital clearance and nasolacrimal fossa clearance was achieved via a medial maxilllectomy [Figure 3]. The periorbita was intact with no invasion of the globe. The medial canthal ligament was reattached and postop recovery was uneventful. Postop histopathology confirmed complete excision of the tumor with clear margins. The patient is in active surveillance and is disease-free until at13-month follow-up.
|Figure 3: Weber-Ferguson incision and medial maxillectomy (a) and complete enblocspecimen (b)|
Click here to view
| > Discussion|| |
Papillomas are defined as neoplasms of surface epithelium with finger-like projections. Sinonasal papillomas are classified into three separate types; fungiform (exophytic), cylindrical cell, and inverted (transitional cell) papilloma; IP being the commonest (70%).  The Schneiderian epithelium which gives rise to IPsis a specialized ectodermal respiratory epithelium lining the lateral nasal wall and paranasal sinuses.  The exact etiology of IP is unknown; however, transitional metaplasia occurs in the basal layer of epithelium, followed by a proliferative phase which leads to the epithelium expanding outwards, inwards, or both to accommodate the increasing surface area.  This transformation may be in some way driven by an inflammatory reaction, but this is not yet proven. 
Athorough literature search was conducted in PubMed, Medline, Embase, Cumulative Index to Nursing and Allied Health Literature (CINAHL), and Google Scholar for primary lacrimal sac IP. Full length articles of relevant papers were obtained and reviewed by first two authors (AWH and RCD) independently. Further cross referencing was done to complete the literature search. Finally, six representative cases were included for this review. The presented case was also included in for the purpose of analysis [Table 1].
The median age (inter quartile range) of presentation of lacrimal sac IP is 34.5 (28.5-55.0) years; with slight female preponderance, male: female (M: F) =1:1.2 [Table 1]. Medial canthal mass is the commonest presenting symptom, followed by epiphora which is seen in 92% patients. Diplopia and visual impairment is rarely seen in these patients and indicates slow growth of the tumor; allowing adequate compensation of function. Invasion of the orbit by growing tumor is very rare, being present in two cases only. Both these cases were males, with relatively older age at the initial presentation. Orbital invasion may indicate more aggressive behavior of IPs with advancing age and male gender. Because of predominant ophthalmic symptoms, not surprisingly patients usually present to ophthalmologists before being referred to an ear, nose, and throat specialist (ENT). The initial workup requires adequate imaging and histological confirmation.
CT is the imaging modality of choice for lacrimal sac IPs and confirms the extent and invasion of the tumor. Bony erosions of the lateral nasal wall are present in half of the reported cases and occur when the lesion arises from the inferior and medial aspect of the lacrimal sac. Those lesions arising from the superiorand lateral aspect of the lacrimal sac will mainly tend to invade the orbit rather than erode the maxilla bone as in the presented case. [Figure 2] shows that the bulk of tumor is present within the orbit with minimal extension in the nasolacrimal canal. There is only one other reported case  of orbital invasion; however in this case reported by Golub et al.,  the tumor bulk was in the nasolacrimal duct with minimal involvement of the orbit.
Surgical excision with clear margins remains the mainstay of treatment for IPs of lacrimal sac, and the majority of cases can be managed by transnasal endoscopicexcision. Those invading the orbit necessitate a combined resection involving removal of both orbital parts of the tumor as well as enbloc excision of the nasolacrimal canal. Open approach is preferred as it provides adequate exposure for the orbital extension of the tumorand the periorbita, as well as allowing enbloc excision of the nasolacrimal canal via the medial maxillectomy. In view of high rate of recurrence (10-34%) and possibility of malignant transformation (5-11.5%),  complete resection of tumor with clear margins is mandatory.
Of the seven lacrimal sac IP cases reviewed in this report, surgical management was sufficient in four cases. In two cases where the surgical margins were involved, postoperative radiotherapy was used. , Mitomycin C irrigation of the nasolacrimal system was used as an adjunct in one case.  Use of interferon alpha-2b  has also been reported as an adjunctive treatment; however, its role is still under investigation.
Long-term prognosis of IPs where complete surgical excision with clear margins is performed is excellent. Overall rate of recurrence in this series of patients was 14%, with one patient  requiring revision surgery. This compares favorably with nasal and paranasal sinus IPs where the recurrence rates are reported to be 10-34%. 
| > Conclusion|| |
Primary IPs of the lacrimal sac is a rare disease with only a handful of reported cases; orbital extension is extremely rare. We recommend enbloc resection of orbital and nasolacrimal part parts of the tumor with clear margins to avoid the need of adjuvant therapy.
| > References|| |
Raemdonck TY, Van den Broecke CM, Claerhout I, Decock CE. Inverted papilloma arising primarily from the lacrimal sac. Orbit 2009;28:181-4.
Elner VM, Burnstine MA, Goodman ML, Dortzbach RK. Inverted papillomas that invade the orbit. Arch Ophthalmol 1995;113:1178-83.
Golub JS, Parikh SL, Budnick SD, Bernardino CR, DelGaudio JM. Inverted papilloma of the nasolacrimal system invading the orbit. Ophthal Plast Reconstr Surg 2007;23:151-3.
Chaudhry IA, Taiba K, Al-Sadhan Y, Riley FC. Inverted papilloma invading the orbit through the nasolacrimal duct: A case report. Orbit 2005;24:135-9.
Fechner RE, Sessions RB. Inverted papilloma of the lacrimal sac, the paranasal sinuses and the cervical region. Cancer 1977;40:2303-8.
Anderson KK, Lessner AM, Hood I, Mendenhall W, Stringer S, Warren R. Invasive transitional cell carcinoma of the lacrimal sac arising in an inverted papilloma. Arch Ophthalmol 1994;112:306-7.
Parmer DN, Rose GE. Management of lacrimal sac tunours. Eye (Lond) 2003;17:599-606.
Woodcock M, Mollan SP, Harrison D, Taylor D, Lecuona K. Mitomycin C in the treatment of a Schneiderian (inverted) papilloma of the lacrimal sac. Int Ophthalmol 2010;30:303-5.
Heathcote JG. Transitional neoplasm of the naso-lacrimal system: A review of the histopathology and histogenesis. Saudi J Opthalmol 2012;26:125-31.
Roh HJ, Procop GW, Batra PS, Citardi MJ, Lanza DC. Inflammation and the pathogenesis of inverted papilloma. Am J Rhinol 2004;18:65-74.
Parulekar MV, Khooshabeh R, Graham C. Topical and intralesional interferon therapy for recurrent lacrimal papilloma. Eye (Lond) 2002;16:649-51.
[Figure 1], [Figure 2], [Figure 3]
|This article has been cited by|
||Inverted papilloma originating from the lacrimal sac and the nasolacrimal duct with marked FDG accumulation
| ||Shuichi Ito, Makoto Hosono, Kazuna Kawabata, Hitomi Kawamura, Masaaki Ishikawa, Mitsunori Kanagaki |
| ||Radiology Case Reports. 2021; 16(11): 3577 |
|[Pubmed] | [DOI]|
||Inverted Papilloma of the Orbit and Nasolacrimal System
| ||Jessie Wang, Joshua Ford, Bita Esmaeli, Paul Langer, Neda Esmaili, Gregory J. Griepentrog, Steven M. Couch, John Nguyen, Katherine G. Gold, Kathleen Duerksen, Cat N. Burkat, Morris E. Hartstein, Parag Gandhi, Rachel K. Sobel, Jee-Young Moon, Anne Barmettler |
| ||Ophthalmic Plastic & Reconstructive Surgery. 2021; 37(2): 161 |
|[Pubmed] | [DOI]|
||Inverted Papilloma of the Epiglottis: A Rare Case
| ||Tae Seong Eo, Eun Kyung Kim, Ji Hoon Kim, Hyang Ae Shin |
| ||Ear, Nose & Throat Journal. 2020; : 0145561320 |
|[Pubmed] | [DOI]|
||Epiphora as a sign of unexpected underlying squamous cell carcinoma within sinonasal inverted papilloma
| ||Di Maria Alessandra, Confalonieri Filippo, Piscopo Raffaele, Balia Laura, Malvezzi Luca |
| ||Archives of Case Reports. 2020; 4(1): 038 |
|[Pubmed] | [DOI]|