INVERTED PAPILLOMA OF NOSE & PARANASAL SINUSES:

 INVERTED PAPILLOMA OF NOSE &     PARANASAL   SINUSES:

                                   


 Dr.K.K.Desarda.  Prof. & Head otolaryngology KEM Hospital Pune.

                                                           
Abstract:



 Inverted papilloma (Schneiderian papilloma) is a primarily benign lesion that occurs in the nasal cavity and paranasal sinuses. Clinical problems include a tendency towards local destruction,recurrence and malignant transformation into squamous cell carcinoma. Hence, complete surgical removal is the therapy of choice and a meticulous follow-up is mandatory. The different histological types of nasal papilloma, their pathogenesis and the clinical and histopathological diagnosis., staging systems, therapeutic approaches, and surgical concepts are discussed.

The medical management is limited. Historically, radiotherapy was used in the management of inverted papillomas. Inverted papillomas have not shown to be radiosensitive. With radiotherapy in recent times, it has been used in patients with synchronous squamous cell carcinoma. Currently, medical management is used as an adjunct to specific complications, such as sinusitis. Surgical management is the mainstay of treatment of inverted papilloma. Selection of the surgical procedure is based on its extent, location, and the presence of concurrent malignancy.



Introduction:


Inverted Papilloma is a benign neoplasm originating from the Schneiderian membrane of the nose and paranasal sinus cavities. It has varied clinical and histological features involving the lateral nasal wall, septum, ethmoids, maxillary and sphenoid sinuses and at times involves the skull base. Three  cses interesting cases are reported below because of varied presentation of inverted papilloma.
Since Inverted Papilloma is associated with chronic sinusitis, patients always had nasal and sinus surgery. Most authorities will consider this a true neoplasm because of its transformation into carcinoma as transitional cell, papilloma or squamous papillary epithelioma. Early clinical diagnosis and thorough evaluation by biochemical tests, high resolution coronal CT scanning MRI studies and radical surgery is advocated. For its recurrence and malignant transformation few authorities advocate deep X–ray therapy following radical surgery. Fifty six  cases of proved inverted papilloma were treated  by different surgical procedures during 1980 to 2000 at KEM Hospital  Pune .
The advantages and disadvantages of various techniques  are discussed. No single technique gives better result so combinations were tried for better results.
The etiology of inverted papilloma is still unknown, but a number of nonspecific causes, as seen in the slide to the right, have been implicated. It is most often considered a true epithelial neoplasm as its intense proliferation of epithelium is its dominant histologic feature. Finally, a viral etiology has also been suggested. The support for viral etiology includes its multifocal origin and its high rate of recurrence, as well as the fact that it has been found to be responsible for other papillomas in other areas of the body. Human papilloma virus is most often associated with inverted papilloma. HPV DNA has been identified in 32 percent of inverted papillomas by in situ heparinization and PCR. Among inverted papillomas associated with carcinomas, HPV is present in 58 percent. Type 16 is the most prevalent in inverted papillomas,
 Because of its varied presentation inverting papillomas can be difficult to distinguish from other nasal tumors and they tend to recur after limited operation and also tend to transform into carcinoma. Hence, it is impossible to predict which inverting papillomas will become malignant.

.Sinonasal papillomas are characterized by being in general unilateral, although bilateral papillomas occur infrequently. Second, they have a destructive capacity with an ability to extend on into adjacent areas by spreading along a mucosa. Third, they have a tendency to recur, and they will recur even if completely excised. Finally, they have the potential for malignant degeneration.The clinical appearance of the nasal mass resembles an allergic polyp looking like a gray and red nasal mass.

The incidence of associated malignancy has been estimated to be approximately 10 % to 15%  Lawson and Allen in 2003 reported that 7 percent of patients have associated malignancy with synchronous carcinoma and 4 with metachronous carcinoma. These develop three to eight years after initial diagnosis. In their literature review of 26 series published between 1970 and 2001, consisting of over 1400 patients, 8.9% were found to have associated malignancy, 67% with synchronous, and 32% with metachronous











Grading of Inverted Papilloma



Grade I  Lesions involving nasal cavity only.
Grade II  Lesions involving nasal cavity + Paranasal sinuses.
Grade III
 Lesions involving  nasal cavity + Paranasal sinuses +Intracranial  extension
.




Management:

 Key to diagnosis is a detailed history, of course. Patients may have a history of unilateral symptoms or history of multiple surgical procedures for nasal polyps. Second, a thorough physical exam where a unilateral mass may be seen or endoscopic exam may reveal multiple polypoid masses with multiple digitations located laterally to the middle turbinate. Three, biopsy is key. Given the similar appearance and possible presence of concurrent polyps, histologic examination is critical. Multiple biopsies may be necessary, as seen in our case, due to inadequate sampling or sampling of concurrent polyp or inflammatory tissue or error in diagnosis.
Finally, radiographic evaluation is critical. CT scan is considered to be the study of choice. The most common CT profile is a unilateral mass with a lobulant surface occupying the middle meatus and extending into one or more of the adjacent sinuses. Opacification, mucosal thickening, of the paranasal sinuses may be seen, as well as bony thinning remodeling or erosion caused by inverted papilloma growth. Disadvantages of the CT scan are primarily due to its difficulty to differentiate inspissated mucus, polyps, or mucoperiostial thickening from inverted papilloma. MRI may also be used in the diagnosis of inverted papilloma. It is superior to CT scan for distinguishing papillomas from underlying inflammation and provides better delineation of lesions in contrast to surrounding soft tissue.
The medical manageent is limited. Historically, radiotherapy was used in the management of inverted papillomas. Inverted papillomas have not shown to be radiosensitive. With radiotherapy in recent times, it has been used in patients with synchronous squamous cell carcinoma. Currently, medical management is used as an adjunct to specific complications, such as sinusitis. Surgical management is the mainstay of treatment of inverted papilloma. Selection of the surgical procedure is based on its extent, location, and the presence of concurrent malignancy.
Three procedures that have been used to treat inverted papillomas are lateral rhinotomy and medial maxillectomy. Currently, the gold standard for the treatment of inverted papilloma is midfacial degloving and endoscopic sinus surgery. Lateral rhinotomy medial maxillectomy is particularly useful for inverted papillomas that are perilacrimal, nasofrontal, supraorbital, ethmoidal, or in the orbit. A curvilinear incision beginning just below the medial aspect of the eyebrow is made inferiorly half-way between the medial canthus and the nasion. The incision is extended inferiorly along the lateral aspect of the nose around the ala. The incision includes a full thickness skin down to periosteum. The periosteum is then elevated as far lateral as the lateral aspect of the maxillary antrum, as far superior as the orbital rim exposing and preserving the infraorbital nerve, and along the nasal bone in the ascending process of the maxilla. The periorbit is then undermined off the lamina papyracea, dislocating the lacrimal sac out of the lacrimal fossa and transacting the lacrimal duct as far distal as possible. The periorbit is then further undermined off the medial floor of the orbit. The anterior and posterior ethmoid artery is identified. These are the most constant landmarks for the frontoethmoid suture line. Staying below the suture line is critical to avoid entrance into the anterior cranial fossa. An opening is made through the anterior wall of the maxillary antrum, and the entire front wall of the maxillary antrum is removed up to the orbital rim. Then osteotomies are made, first along the floor of the nose through the bone between the antrum and the nasal cavity; second, through the frontoethmoid suture line below the level of the anterior ethmoid artery; and finally, along the medial floor of the orbit to the posterior wall of the antrum. The lateral nasal wall is then removed by cutting through the middle inferior turbinate attachments and then all mucosa is removed from the maxillary antrum. Then the sphenoid sinus is opened and its mucosa also removed. Dacryocystorhinostomy is then performed to avoid epiphora, a common postoperative complication of this procedure. It can be accomplished in two ways—either by catheterization of the lacrimal duct using an indwelling silicone or incising the lacrimal sac along it long axis and then suturing the edges in place to adjacent tissues. The main advantages of this approach include a radical excision, access to all lateral sinuses, the skull base, nasopharynx, and orbit. Disadvantages include, obviously, a surgical scar, possible CSF leak, epiphora, injury to orbit, and mucocele formation.
The contraindications to a purely endoscopic resection of inverted papillomas include the concomitant presence of squamous cell carcinoma, massive skull base erosion, intradural or intraorbital extension, and extensive involvement of the frontal sinus.
Midfacial degloving is particularly useful for inverted papillomas that are bilateral nasal in origin. Four types of incisions are required in this approach. The first is bilateral intercartilaginous incisions, bilateral septocolumellar incisions, a complete transfixion incision, and then a gingivobuccal incision. This is made from one maxillary tubercule to another. The soft tissue is then incised around the piriform aperture and nasal floor is undermined as high as the orbital rims. These incisions facilitate the exposure of a piriform aperture in the lateral nasal wall. A medial maxillectomy is then performed . and this can be combined with the frontal sinus osteoplastic flap for access to the frontal sinus. Its main advantages are that there is no external scar, invisibility, and bilateral access. Its disadvantages include insufficient access to more distant areas, such as vestibular, orbital, ethmoid cells; and complications include vestibular stenosis, oral antral fistula, epistaxis, and nasal congestion.
Endoscopic sinus surgery is particularly useful for inverted papillomas in the lateral nasal and nasal cavity, middle meatus, maxillary sinus, and the anterior and posterior ethmoid cells. The procedure largely consists of tumor debulking through a microdebridder until the origin is identified, and then lesions are usually excised en bloc from the identified tumor attachment sites with a wide cup of normal mucosa. Frozen sections are obtained, negative margins confirmed, and bone may be removed from underlying sites of attachment Although traditionally endoscopic surgery is used more for small lesions, bulky lesions have made endoscopic surgery difficult.  However, a new technique has been described for treatment of massive tumors with attachments within the maxillary sinus. It is called SSES (Sequential Segmental Endoscopic sinus Surgery). Basically, this involves sequential excision of larger tumors into massive segments, four segments usually. First, the nasal cavity; second, the middle meatus, including portions of the ostiomeatal complex; third, the maxillary sinus ostium and antrum along with the maxillary sinus medial wall if an endoscopic medial maxillectomy is performed; and fourth the frontal or sphenoid sinus .Advancements in diagnosis and treatment of sinus disease have led to successful use of endoscopic techniques in the treatment of inverted papilloma .

Case presentations:

Case No. 1
Mr. D. K. aged 68 had complaints of left nasal obstruction, headache, epiphora and bleeding from nostrils (epistaxis). He had nasal polypectomy and intranasal antrostomy done seven years ago. An ENT examination revealed a large greyish mass occupying the left nasal cavity pushing the septum to right side. A probe could not be passed around the nasal mass since it was coming from the lateral nasal wall. The nasal vault was tender and there was a evidence of nasolacrimal duct obstruction. Left maxilla was tender and mass did not bleed on touch. A provisional diagnosis of recurrence of nasal polyposis was made with the view of malignancy in mind.

The routine bio–chemical tests were within normal limits, X–ray paranasal sinuses revealed opacity and left maxillary sinuses with medial wall destruction. A CT scan also revealed a lesion of maxillary sinus with erosion of medial wall and a soft tissue mass in the left nasal cavity extending to the nasopharynx.
In view of the recurrence and erosion of the medial wall, the patient was subjected to lateral rhinotomy with medial maxillectomy procedure. The mass was removed with maxillary clearance. Histopathology confirmed the diagnosis of inverted papilloma with no evidence of malignancy. Post operative recovery was uneventful. The patient was reviewed in a follow up clinic and had no recurrence for the last one and a half year.

Case No. 2
Mrs. M. L. aged 48 had a bilateral nasal obstruction, mouth breathing and headache. She was non–diabetic, non–hypertensive and had nasal polypectomy two years ago. A routine clinical examination revealed bilateral polypoidal masses occupying both the nostrils. The color of the mass was grayish and firm in consistency and did not bleed on touch. Both maxillary sinuses were tender. Postnasal space did not show any soft tissue mass..
Clinical diagnosis of bilateral nasal polyposis was made (Recurrence) with associated maxillary sinusitis and no extension to nasopharynx. CT PNS revealed extensive opacity of maxillary and ,ethmoid sinuses without any erosion of orbital cavity. Endoscopic  sinus surgery  was planned under GA .  Post operative recovery was uneventful. Histopathology of the biopsy revealed inverted papilloma. A review after 18 months was satisfactory without recurrence.
Case No. 3
Mr. A. K. aged 28 came to the ENT Clinic with the chief complaints of nasal obstruction, bleeding from left nostril, headache, and proptosis of left eye for over six months. A clinical diagnosis of the left nasal polyposis was made after a thorough clinical examination. A routine hemogram was performed and this including biochemical tests were within normal limits. A CT scan revealed a lesion involving the entire left ethmoid, nasal cavity, maxillary sinus, nasopharynx and anterior wall of sphenoid sinus.A planned endoscopic ethmoidectomy and transantral maxillary clearance was done. The post operative period was uneventful. and  the patient’s left proptosis decreased . Histopathology report revealed inverted papilloma. Post–operative review after six months was quite satisfactory and without recurrence.
Discussion:
The search for an ideal surgical approach to removing inverted papillomas has been fairly controversial, with proponents of radical surgery vying with those who support endoscopic endonasal procedures. The external approaches include medial maxillectomy, frontoethmoidectomy, mid facial degloving and Caldwell-Luc surgeries. Recurrence rates following these procedures have been reported to range from 4 to 35%.  The short-term complications of these approaches include epiphora, dacryocystitis, diplopia, transient blepharitis, lid edema, and cerebrospinal fluid leak. Late complications include persistent crusting, pain, nasocutaneous fistula, frontal sinus mucocele, vestibular stenosis, unacceptable scarring, and nasal collapse

Inverted papillomas are relatively uncommon tumors of the nasal cavity comprising approximately 0.5% to 4% of all primary nasal tumors. Its incidence ranges from 0.75 to 1.5 cases per 100,000 per year. They occur approximately 15 th as often as inflammatory polyps. Age ranges widely from 6 to 89 years, and most are usually diagnosed in the 5 th to 7 th decade. Average age of diagnosis is 53 years. There is a male predominance 3:1, and it affects primarily Caucasians.
Sinonasal papillomas have been categorized in to three distinct subtypes based on histologic appearance. Inverted papillomas (70%),cylindrical cell papilloma (19%), and fungiform  papillomas  (11%),although all these are histologically benign in nature but inverted and cylindrical  papillomas  may be associated with malignancy which ranges from 4% to 17 % for inverted papillomas and 9% to 13% for cylindrical papillomas..
Symptoms are nonspecific often mimicking sinusitis. The most common is unilateral nasal obstruction seen in over 60% of patients. Nasal discharge, headache, facial pressure and pain, epistaxis, and anosmia may also be seen. Signs may include a polypoidal mass filling the nasal cavity extending from the vestibule to the nasopharynx. The nasal septum is often bowed to the contralateral side due to slow expansile growth. Proptosis and facial swelling  is also seen in these patient  at late stages.
Sinonasal papillomas are characterized by being in general unilateral, although bilateral papillomas occur infrequently. Second, they have a destructive capacity with an ability to extend on into adjacent areas by spreading along a mucosa. Third, they have a tendency to recur, and they will recur even if completely excised. Finally, they have the potential for malignant degeneration Although most of the lesions arise from the lateral wall, middle meatus and ethmoid complex, they may sometimes arise from the septum and underlying perichondrium, the cartilage, lateral nasopharyngeal wall, maxillary sinus, sphenoid sinus and may involve the base skull. The distribution is: lateral wall 68%, ethmoid complex 57%, septum 28% intracranium 4%. The highest recurrence rate  is 70%.
 Advantages and Disadvantages of endoscopic surgery.
It is a less invasive procedure and you have a multiangle visualization and absence of facial scarring. In terms of disadvantages - orbit injury, CSF leak, and periorbital ecchymosis. It is especially difficult for the larger tumors that fill the entire nasal cavity. This is increasingly circumvented by the ability to first debulk the tumor and then perform excision in segments. The main controversy in the treatment of inverted papilloma lies in the final advantage and disadvantage: seemingly contradictions, comparable versus greater recurrence rate. This inherent contradiction is reflected in the debate that still exists regarding whether less extensive procedures result in incomplete excision of the inverted papilloma and, thus, more recurrence



Acknowledgement

I am thankful to  Dr. B.J. Coyaji Chief Medical officer,KEM Hospital Pune for permitting me to publish this paper.I extend sincere thanks to  Nursing staff and staff ENT Dept for their kind co-operation in  the preparation of this manuscript.


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Contributed by Dr. K. K. Desarda,