Journal of Neuro-Ophthalmology, March 1991, Volume 11, Issue 1

Inverted squamous papilloma with neuro-ophthalmic features.

The schneiderian or inverted papilloma is an infrequent and usually benign tumor of the paranasal sinuses. It most commonly manifests as unilateral nasal obstruction. This is a case of an inverted papilloma in a 92-year-old woman who presented with severe proptosis, loss of vision in the left eye, and rhinorrhea. The mass was visible in her left nostril; neuroimaging studies showed that the lesion extended from the left paranasal sinus into the frontal sinuses. In addition, there was secondary spread into the cranial cavity and brain substance.

Journal of Clinical Neuro- ophtlullmology 11( 1): 35- 38, 1991. Inverted Squamous Papilloma with Neuro- Ophthalmic Features Ila M. Peterson, M. D., and Carissa Heim, M. S. Y. Ig 1991 Raven Press, Ltd., New York The schneiderian or inverted papilloma is an infrequent and usually benign tumor of the paranasal sinuses. It most commonly manifests as unilateral nasal obstruc­tion. This is a case of an inverted papilloma in a 92­year- old woman who presented with severe proptosis, loss of vision in the left eye, and rhinorrhea. The mass was visible in her left nostril; neuroimaging studies showed that the lesion extended from the left paranasal sinus into the frontal sinuses. In addition, there was secondary spread into the cranial cavity and brain sub­stance. Key Words: Schneiderian papilloma- Inverted papil­loma- Squamous papilloma. From the Department of Pathology, Truman Medical Center, Kansas City, Missouri, U. S. A. Address correspondence and reprint requests to Dr. Ila M. Peterson at Department of Pathology, Truman Medical Center, 2301 Holmes Street, Kansas City, MO 64108, U. S. A. 35 Inverted papillomas arising in the nasal cavity and paranasal sinuses, although usually histolog­ically benign, may behave in an aggressive fash­ion. Patients usually present with symptoms re­lated to nasal obstruction, but they rarely manifest eye signs. This is a report of an elderly woman with an inverted papilloma of the nasopharynx and neuro- ophthalmic symptoms. CASE REPORT This 92- year- old woman presented with left fa­cial protrusion, left proptosis, rhinorrhea, and a mass lesion filling the left nasal passage. One year prior to her admission she noted a loss of vision in her bulging left eye. On initial physical examina­tion, her left eye was proptotic with dry and in­jected conjunctivae. She had a loss of light percep­tion, pupillary response, and extraocular move­ments in all directions. There was a large pink mass filling the left nares. The rhinorrhea was glu­cose positive, indicating the presence of cerebro­spinal fluid. The patient was incontinent and higher cortical functions were severely impaired. Computerized tomography without contrast showed a large tumor in the soft tissues of the left nasal cavity extending into the left frontal, maxil­lary, and ethmoid sinuses. There was widespread bony destruction and involvement of the left pterygoid fossa and left middle cranial fossa with compression of the left internal carotid artery ( Fig. 1). On magnetic resonance imaging the tumor was seen to involve the left optic nerve, the posterior pharynx, and frontal lobe ( Fig. 2). Biopsy of the mass protruding from the nasal cavity and ethmoid sinus consisted of a 2 x 1 x 1 cm cauliflower- like, soft, pale pink- tan tissue ag- l. M. PETERSON AND C. HElM FIG. 1. Computerized tomography showing tumor's destruction of the medial wall of the orbit and involve­ment of the left ethmoid and sphenoid sinus. gregate. The transitional epithelial mucosa in­cluded both stratified squamous and columnar cells covering a delicate fibrovascular stalk and possessed uniformly rounded nuclei, pale pink cy­toplasm, occasional nucleoli, a few mitotic figures, scattered micromucous cysts, and focal necrosis. . " .~ , .... . - " •,.' '. ' . I : 1 .. J I , ,. FIG. 2. Magnetic resonance scan demonstrating blow out artifact and soft tissue mass involving the frontal sinus, nasal cavity, sphenoid sinus, and nasopharynx. The tumor is breaking through out the frontal bone, with extension into anterior frontal lobe ( arrow). The cysts were mucin positive. The tumor inva­ginated into the stroma with blunted tongues ( Fig. 3). The patient received no therapy and was dis- ". FIG. 3. Inverted papilloma lined by " transitional" respiratory epithelium with micro­cysts and delicate fibrovascular core ( hematoxylin and eosin, 100x). /-------""'" INVERTED PAPILLOMA- CNS FEATURES 37 charged to a nursing home where she died 9 months later. DISCUSSION The inverted papilloma or schneiderian tumor ( named for its hypothetical parent tissue of ecto­dermal respiratory epithelium of the nasal pas­sage), although usually benign, can be locally ag­gressive and erode or distort adjacent bony struc­tures ( 1- 6). These papillomas represent between 0.4 to 4.7% of all nasal tumors removed. They have a strong male predilection ( greater than 2: 1) and occur at a mean age of 50 years. Whites are more commonly affected than blacks ( 1,3,5). The most common presenting complaints have included uni­lateral nasal obstruction or stuffiness, epistaxis, nasal discharge, and pain. Rarely diplopia or pro­ptosis has occurred. The tumor is almost exclu­sively unilateral, and the majority arise in the lat­eral nasal wall, ethmoid and maxillary sinuses, or nasal septum ( 5). Intracranial extension has been reported ( 4) ( See Table 1). There is no increased prevalence of allergies, smoking, or other medical conditions in patients with inverted papillomas. Five percent of these tu­mors have been associated with carcinomas; some of these patients have occupational risk factors such as exposure to wood dust and tanning chem­icals ( 5). Recently, the human papillomavirus se­rotype 11 has been identified by DNA hybridiza­tion technique in this lesion ( 2). The differential diagnosis of a nasal inverted papilloma includes squamous papilloma, inflam­matory polyp, mucocele, and papillary adenocar­cinoma. On gross examination the tumor is either endophytic or exophytic, the latter being its vari­ant counterpart, the fungiform papilloma. Histo­logic examination reveals a bland squamoid, co­lumnar, or " transitional" epithelium of the in­verted papilloma invaginating the underlying stroma. The squamous papilloma resembles the common cutaneous wart. Inflammatory polyps have an edematous inflamed stroma covered with a respiratory or metaplastic mucosa and arise in patients with a history of allergies. Mucoceles contain large pockets of mucus surrounded by glandular or squamoid mucosa. Papillary adeno­carcinomas possess frankly malignant neoplastic cellular features. Malignant transformation of an inverted papilloma occurs but is unusual « 2% of cases) ( 7). An invasive squamous- cell carcinoma arising in the same site as an inverted papilloma is more frequent and reported in 13% of Hyams's Se­ries ( 8). Because of the limited nature of the biopsy in this case, a coexisting squamous- cell carcinoma could not be ruled out. Primary treatment is surgical extirpation. A few patients with benign inverted papillomas that have undergone malignant transformation have been ir­radiated with good responses ( 5). A high recur­rence rate of 75% with simple polypectomies has occurred. Lateral rhinotomy provides better expo­sure for excision with a lower recurrence rate of 27% ( 3). TABLE 1. Cases of inverted nasal papilloma with CNS symptomatology Reference Lawton et al. ( 1) van Olphen et al. ( 4) Peterson et al. Presentation 58- year- old woman with proptosis, upward displacement, and decreased vision in right eye. Compertized axial tomography ( CAT) scan: tumor in right maxillary sinus destroying bone and invading inferolateral orbit. 32- year- old woman with headache over frontal sinuses, swelling, nasal obstruction. CAT scan: tumor in anterior cranial fossa, nasal cavity, frontal and ethmoid sinuses. 92- year- old woman with proptosis, facial protrusion, rhinorrhea, and tumor filling left nasal passage. CAT scan: tumor of left nasal cavity and extending into left frontal, maxillary, and ethmoid sinuses; widespread bony destruction with tumor involvement of left pterygoid and middle cranial fossa, left optic nerve, and frontal lobe. Treatment Rhinotomy Lateral rhinotomy and craniotomy Biopsy diagnosis; no therapy. Outcome Patient died of unrelated causes 9 days later; no autopsy Patient died 9 months after biopsy; no autopsy. /~--------'., I Clin Neuro- ophlhalmol, Vol. 11, No. 1, 1991 38 I. M. PETERSON AND C. HElM Acknowledgment: The authors would like to thank Dr. William Schaetzel for his microscopic photographs and Dr. Randall James for his assistance in interpretation of the radiographic scans. REFERENCES 1. Lawton AW, Karesh JW, Gray WC Proptosis from maxil­lary sinus inverted papilloma with malignant transforma­tion. Arch Ophthalmol 1986; 104: 874- 7. 2. Respler DS, Pater A, Jahn A, Pater MM. HPY isolation and characterization of papillomavirus DNA from nasal invert­ing ( schneiderian) papillomas. Ann 0101 Rhinal Laryngol 1987; 96: 170- 3. ' Clin Neuro- ophthalmol, Vol. 11, No. L 1991 3. Smith 0, Gullare PJ. Inverting papilloma of the nose: anal­ysis of 48 patients. , OtoI1987; 16: 154- 6. 4. van Olphen AF, Lubsen H, van ' Tverlaat JW. An inverted papilloma with intracranial extension. Laryngol 0101 1988; 102: 534- 7. 5. Weissler eM, Montgomery WW, Montgomery SK, Turner PA, Joseph MP. Inverted papilloma. Ann 0101 Rhinol1.. Jlryn­gal 1986; 95: 215-- 21. 6. Hyams YJ, Batsakis JG, Michaels L. Tumors of Ihe upper res­piratory tract and ear. Washington, D. C: Armed Forces In­stitute of Pathology Fascicle 25, 1986: 34- 44. 7. Batsakis JG. Squamous cell " papillomas" of oral cavity, sinonasal tract and larynx. In: Tumors of Ihe head and neck. Baltimore, MD: Williams and Wilkins, 1974: 135. 8. Hyams VJ. Papillomas of the nasal cavity and paranasal sinuses. Ann 0101 Rhinal 1.. 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