Schneiderian Inverted Papilloma: Report of a Case Beulah D. Jones, MD Bronx, New York
A case of Schneiderian inverted papilloma is presented. Etiology, gross and microscopic pathology, and clinical course are discussed along with a plan of treatment. The inverted or Schneiderian papilloma is a rare but identifiable pathologic entity. It was first cited by Billrothl2 in 1855, who termed it a "vilIous cancer." Since that time, there have been approximately 500 case reports. The problem has been mainly one of terminology in describing the lesion.2 The proposed nomenclature was either not descriptive of the pathology or not a clear definition of the clinical behavior of the disease. One author3 grouped it with all transitional cell tumors. Schneiderian papilloma delimits the location accurately as well as the demonstrated pathology. There has been one case, however, of a lesion originating in the oropharynx.1 The case presented here is typical in that vague nonspecific symptoms, mimicking nasal polyps, clouded the true diagnosis.
Case Report A 38-year-old male presented with a vague history of six years duration of difficulty in breathing. The symptoms were progressive over the previous six months to complete left-sided nasal obstruction. Epistaxis had become prominent in the preceding four to five months, particularly with manual manipulation. The past medical history systems review was unremarkable. Physical examination revealed complete block of the left side of the nose by polypoid lesions. The patient was admitted to Westchester Square Hospital in June 1976, with a presumptive diagnosis of nasal polyps. Plain films of the
From the Department of Surgery, Westchester Square Hospital, Bronx, New York. Requests for reprints should be addressed to Dr. Beulah D. Jones, 353 Hawthorne Avenue, Yonkers, NY 10705.
paranasal sinuses revealed complete opacification of the left alae nasae (Figure 1). There was clouding of the left maxillary, frontal, and ethmoid sinuses. The spenoid sinuses were clear. No air-fluid levels were appreciated. The patient was then scheduled for surgical exploration. At the time of surgery, polyps of such bulk were noted that a frozen section was requested. The pathologist's report was negative for malignancy with a working diagnosis of nasal polyps. A complete excision of all polyps was done. Amputation of the middle turbinate and exenteration of the left ethmoid labyrinth, by the intranasal route, was performed. A left Caldwell-Luc and intranasal antrostomy revealed a grossly thickened mucosa without polypoid degeneration. The patient had an unremarkable postoperative course and was discharged on the eighth postoperative day. Receipt of the final pathology report and review of the microscopic pathology confirmed the diagnosis of Schneiderian inverted papilloma. The patient was readmitted with a plan for wide local excision by lateral rhinotomy. Polytomography of the sinuses exhibited no bony destruction or distortion of the architecture of the sinuses (Figure 2). The patient decided against further operative intervention and became lost to contact.
Discussion Schneiderian inverted papilloma represents 2.5 to 3 percent ofall primary tumors of the upper respiratory tract. Seen primarily in males in the older age group, cases have been described from the second to the eighth decade of life. The youngest reported case was an eight-year-old male.4 The etiology of this lesion has been thoroughly investigated.2'5 Review of several series noted chronic infection but definitely ruled
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 70, NO. 11, 1978
out an inflammatory process as a possible cause. Previous multifocal sites of involvement have been proposed but not proven.2'6 The theory of a viral etiology has not yet been ruled out. Several authors have implicated the papova virus group: electron microscopy has demonstrated intranuclear inclusion bodies. To date, the precise etiology remains unknown. The primary site has been identified as the ethmoid recess, but there have been investigators who feel the anterior nasal septum is the site of origin in some of smaller lesions.7 Symptoms are not pathognomonic. Usually, the presenting complaint is unilateral nasal obstruction. Pain, anosmia, epitaxis, and frontal headaches were the four most common complaints in one series.2 The prevalence of unilaterality is well documented,1-12 as well as the distinct rarity of allergic history. Schneiderian inverted papilloma in association with nasal polyps is not an uncommon finding. The changes are due to mechanical obstruction of normal lymphatic and venous drainage by the bulky lesion. Pathologically, the lesion is endophytic (Figure 3) although gross examination may reveal a large multilobulated mass. The epithelial layer is hyperplastic, up to 40 layers thick.9 Subepithelial crypts and knobs (which maintain connection with the surface epithelium at all times) are present. Basal layer proliferation with cords in the underlying connective tissue is a prominent feature. Areas interrupted from surface continuity may resemble isolated nests, hence the term pseudoinvasiveness. The earmark is that there is no actual disruption of surface continuity (Figures 3 and 4). The basement membrane is not invaded in this lesion. Dyskeratosis, although present in 10 percent of cases, does not necessarily indicate malignant
degeneration.57 The underlying stroma is characteristically loose and vascular in nature with varying numbers of plasmacytes and lymphocytes. Polymorphonuclear leukocytes are usually sparse. The appearance of 801
''11.
Figure 1. Plain film of the paranasal sinuses showing complete opacification of the left alae nasae.
-X; ,,., ...... ..~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~..... ^
.itf
Figure 3. Photomicrograph of the lesion. Note its endophytic pattern and many cell layers.
_ plsicmcsai calze prssr
-
s
!
-_~~~~*~1
oniuusaes.o
atohvf h
kl
Figure 2. Polytomography of the sinuses. Note an absence of bony destruction.
withuexens.Plion fintof the prnasoparyn mucous-secreting glands is present resulting from invagination of the surface
epithelium
producing pseudogland
formation. Individual cells are uniform in size with intracellular bridges, demonstrated particularly in the basal layers. Occasional mitoses are present, but are always normal in configuration. The clinical course is dominated by local unilateral recurrences and pressure atrophy of surrounding structures. Twenty-five to 50 percent will recur at least once.-5 Marked atypia, short of carcinoma, can be expected in ten percent of cases; there are no criteria determining which lesions will undergo malignant degeneration. Multicentric foci are not common but are usually extensions or transformation of meta-
802
Figure 4. Photomicrograph showing an absence of basement membrane disruption.
and sinuses is the usual course of the disease. The sphenoid sinuses are usually uninvolved. Principles of management must follow along those of wide local excision.11'12 The optimal approach is through the lateral rhinotomy. Subtotal or complete maxillectomy may be necessary in selected cases. Literature Cited 1. Nonsachuk JS: Oropharyngeal papilloma. Arch Otolaryngol 100(1):71-73, 1974 2. Vrabec DP: The inverted Schneiderian papilloma: A clinical and pathological study. Laryngoscope 85(1):186-220, 1975 3. Robbins S: Pathology, ed 3. Philadelphia, WB Saunders, 1969, p 768 4. Sinha SN, Srivastava RD: Transitional
cell papilloma of nose and paianasal sinuses. Ear Nose Throat 51(6):220-223, 1971 5. Batsakis JG: Tumors of the head and neck: Clinical and Pathological Considerations. Baltimore, Williams and Wilkins, 1974, pp 76-82 6. Skolnik E, cited by Nonsachuk JS: Orapharyngeal papilloma. Arch Otolaryngol 100(1):71-73, 1974 7. The maxillary sinus. Otolaryngol Clin North Am 9:34.40, 1976 8. Paparella M, Shumrick D: Otolaryngology, vol 3: Head and Neck, Philadelphia, WB Saunders, 1973, pp 140-141 9. Armed Forces Institute of Pathology: Atlas of Tumor Pathology, section IV, fascicle 12. Washington, DC, American Registry of Pathology AFIP, 1964, pp 32-33 10. Billroth T, cited by Vrabec DP: The inverted Schneiderian papilloma: A clinical and pathological study. Laryngoscope 85(1):186220, 1975 11. The nasal cavities. Otolaryngol Clin North Am 6:803, 804, 811-812, 1973 12. Surgery of the paranasal sinuses. Otolaryngol Clin North Am 4:154-156, 1971
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 70, NO. 11, 1978
A case of Schneiderian inverted papilloma is presented. Etiology, gross and microscopic pathology, and clinical course are discussed along with a plan of treatment. The inverted or Schneiderian papilloma is a rare but identifiable pathologic entity. It was first cited by Billrothl2 in 1855, who termed it a "vilIous cancer." Since that time, there have been approximately 500 case reports. The problem has been mainly one of terminology in describing the lesion.2 The proposed nomenclature was either not descriptive of the pathology or not a clear definition of the clinical behavior of the disease. One author3 grouped it with all transitional cell tumors. Schneiderian papilloma delimits the location accurately as well as the demonstrated pathology. There has been one case, however, of a lesion originating in the oropharynx.1 The case presented here is typical in that vague nonspecific symptoms, mimicking nasal polyps, clouded the true diagnosis.
Case Report A 38-year-old male presented with a vague history of six years duration of difficulty in breathing. The symptoms were progressive over the previous six months to complete left-sided nasal obstruction. Epistaxis had become prominent in the preceding four to five months, particularly with manual manipulation. The past medical history systems review was unremarkable. Physical examination revealed complete block of the left side of the nose by polypoid lesions. The patient was admitted to Westchester Square Hospital in June 1976, with a presumptive diagnosis of nasal polyps. Plain films of the
From the Department of Surgery, Westchester Square Hospital, Bronx, New York. Requests for reprints should be addressed to Dr. Beulah D. Jones, 353 Hawthorne Avenue, Yonkers, NY 10705.
paranasal sinuses revealed complete opacification of the left alae nasae (Figure 1). There was clouding of the left maxillary, frontal, and ethmoid sinuses. The spenoid sinuses were clear. No air-fluid levels were appreciated. The patient was then scheduled for surgical exploration. At the time of surgery, polyps of such bulk were noted that a frozen section was requested. The pathologist's report was negative for malignancy with a working diagnosis of nasal polyps. A complete excision of all polyps was done. Amputation of the middle turbinate and exenteration of the left ethmoid labyrinth, by the intranasal route, was performed. A left Caldwell-Luc and intranasal antrostomy revealed a grossly thickened mucosa without polypoid degeneration. The patient had an unremarkable postoperative course and was discharged on the eighth postoperative day. Receipt of the final pathology report and review of the microscopic pathology confirmed the diagnosis of Schneiderian inverted papilloma. The patient was readmitted with a plan for wide local excision by lateral rhinotomy. Polytomography of the sinuses exhibited no bony destruction or distortion of the architecture of the sinuses (Figure 2). The patient decided against further operative intervention and became lost to contact.
Discussion Schneiderian inverted papilloma represents 2.5 to 3 percent ofall primary tumors of the upper respiratory tract. Seen primarily in males in the older age group, cases have been described from the second to the eighth decade of life. The youngest reported case was an eight-year-old male.4 The etiology of this lesion has been thoroughly investigated.2'5 Review of several series noted chronic infection but definitely ruled
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 70, NO. 11, 1978
out an inflammatory process as a possible cause. Previous multifocal sites of involvement have been proposed but not proven.2'6 The theory of a viral etiology has not yet been ruled out. Several authors have implicated the papova virus group: electron microscopy has demonstrated intranuclear inclusion bodies. To date, the precise etiology remains unknown. The primary site has been identified as the ethmoid recess, but there have been investigators who feel the anterior nasal septum is the site of origin in some of smaller lesions.7 Symptoms are not pathognomonic. Usually, the presenting complaint is unilateral nasal obstruction. Pain, anosmia, epitaxis, and frontal headaches were the four most common complaints in one series.2 The prevalence of unilaterality is well documented,1-12 as well as the distinct rarity of allergic history. Schneiderian inverted papilloma in association with nasal polyps is not an uncommon finding. The changes are due to mechanical obstruction of normal lymphatic and venous drainage by the bulky lesion. Pathologically, the lesion is endophytic (Figure 3) although gross examination may reveal a large multilobulated mass. The epithelial layer is hyperplastic, up to 40 layers thick.9 Subepithelial crypts and knobs (which maintain connection with the surface epithelium at all times) are present. Basal layer proliferation with cords in the underlying connective tissue is a prominent feature. Areas interrupted from surface continuity may resemble isolated nests, hence the term pseudoinvasiveness. The earmark is that there is no actual disruption of surface continuity (Figures 3 and 4). The basement membrane is not invaded in this lesion. Dyskeratosis, although present in 10 percent of cases, does not necessarily indicate malignant
degeneration.57 The underlying stroma is characteristically loose and vascular in nature with varying numbers of plasmacytes and lymphocytes. Polymorphonuclear leukocytes are usually sparse. The appearance of 801
''11.
Figure 1. Plain film of the paranasal sinuses showing complete opacification of the left alae nasae.
-X; ,,., ...... ..~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~..... ^
.itf
Figure 3. Photomicrograph of the lesion. Note its endophytic pattern and many cell layers.
_ plsicmcsai calze prssr
-
s
!
-_~~~~*~1
oniuusaes.o
atohvf h
kl
Figure 2. Polytomography of the sinuses. Note an absence of bony destruction.
withuexens.Plion fintof the prnasoparyn mucous-secreting glands is present resulting from invagination of the surface
epithelium
producing pseudogland
formation. Individual cells are uniform in size with intracellular bridges, demonstrated particularly in the basal layers. Occasional mitoses are present, but are always normal in configuration. The clinical course is dominated by local unilateral recurrences and pressure atrophy of surrounding structures. Twenty-five to 50 percent will recur at least once.-5 Marked atypia, short of carcinoma, can be expected in ten percent of cases; there are no criteria determining which lesions will undergo malignant degeneration. Multicentric foci are not common but are usually extensions or transformation of meta-
802
Figure 4. Photomicrograph showing an absence of basement membrane disruption.
and sinuses is the usual course of the disease. The sphenoid sinuses are usually uninvolved. Principles of management must follow along those of wide local excision.11'12 The optimal approach is through the lateral rhinotomy. Subtotal or complete maxillectomy may be necessary in selected cases. Literature Cited 1. Nonsachuk JS: Oropharyngeal papilloma. Arch Otolaryngol 100(1):71-73, 1974 2. Vrabec DP: The inverted Schneiderian papilloma: A clinical and pathological study. Laryngoscope 85(1):186-220, 1975 3. Robbins S: Pathology, ed 3. Philadelphia, WB Saunders, 1969, p 768 4. Sinha SN, Srivastava RD: Transitional
cell papilloma of nose and paianasal sinuses. Ear Nose Throat 51(6):220-223, 1971 5. Batsakis JG: Tumors of the head and neck: Clinical and Pathological Considerations. Baltimore, Williams and Wilkins, 1974, pp 76-82 6. Skolnik E, cited by Nonsachuk JS: Orapharyngeal papilloma. Arch Otolaryngol 100(1):71-73, 1974 7. The maxillary sinus. Otolaryngol Clin North Am 9:34.40, 1976 8. Paparella M, Shumrick D: Otolaryngology, vol 3: Head and Neck, Philadelphia, WB Saunders, 1973, pp 140-141 9. Armed Forces Institute of Pathology: Atlas of Tumor Pathology, section IV, fascicle 12. Washington, DC, American Registry of Pathology AFIP, 1964, pp 32-33 10. Billroth T, cited by Vrabec DP: The inverted Schneiderian papilloma: A clinical and pathological study. Laryngoscope 85(1):186220, 1975 11. The nasal cavities. Otolaryngol Clin North Am 6:803, 804, 811-812, 1973 12. Surgery of the paranasal sinuses. Otolaryngol Clin North Am 4:154-156, 1971
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 70, NO. 11, 1978
