BENIGN NEOPLASMS OF THE NOSE AND PNS.pptx

BENIGN NEOPLASMS OF THE NOSE AND PNS

1. BENIGN NEOPLASMS OF THE NOSE AND PNS ---Dr. sayan banerjee

2. A FEW CHARACTERISTICS OF NOSE & P.N.S TUMOUR Wide variety of tumors. Short clinical course of patients afflicted with these neoplasms. Rarity with which the tumors occur. Nasal tumours are difficult to separate from paranasal sinus tumour except in early stage. Most of them are malignant in nature and therefore poor prognosis for patients with nasal and paranasal sinus malignancy. This has led many investigators to focus their attention on it. Recent developments in fibre -optics, radiographic imaging, and a growing consensus on staging and nomenclature. A vast amount of clinical information is now coming into focus and becoming available.

3. BENIGN INTERMEDIATE MALIGNANT Squamous papilloma Encephalocele* Glioma* Dermoid* Neurofibroma Schwannoma Angiofibroma* Osteoma Chondroma Ossifying fibroma Cementoma Fibrous dysplasia* Odontogenic tumour* Inverted papilloma Meningioma Hemangioma Hemangiopericytoma Ameloblastoma Plasmacytoma Squamous cell carcinoma Adenocarcinoma Adenoid cystic carcinoma Malignant melanoma Olfactory neuroblastoma Lymphoma Osteogenic sarcoma Chondrosarcoma Fibrosarcoma Rhabdomyosarcoma* N.B. These tumours are more common in children

4. THE DISTRIBUTION OF VARIOUS SITES FOR PRIMARY TUMORS OF THE NASAL CAVITY AND PARANASAL SINUSES THE DISTRIBUTION OF MALIGNANT EPITHELIAL TUMORS OF THE NASAL CAVITY AND PARANASAL SINUSES SNUC, SINONASAL UNDIFFERENTIATED CARCINOMA

5. SQUAMOUS PAPILLOMA This verrucous growth is similar to skin warts. It can arise from the vestibule and lower part of nasal septum. These papillomas may be single/multiple and pedunculated/sessile. Treatment: They need local excision. The cauterization of the base prevents recurrence. They can be managed even with cryosurgery or laser.

6. INVERTED PAPILLOMA/ RINGERTZ TUMOR: These are histologically a squamous or transitional cell epithelium surrounding fibrovascular stroma with endophytic growth. The neoplastic epithelium of this variety of papilloma grows towards the underlying stroma rather than on the surface and hence they are called INVERTED PAPILLOMA . It is thought to be caused by human papilloma virus(HPV). They arise from the lateral nasal wall and have been reported to be associated with squamous cell carcinoma in 5 to 15% of patients. These are the most common benign tumour of nose . The inverted papillomas are very much known for recurrence. CLINICAL FEATURES – These are mostly seen in males of 40 -70 years of age. Unilateral nasal obstruction and nasal discharge . They arise from the lateral nasal wall at or near middle meatus. They present as gray or reddish polypoid mass. It can invade the paranasal sinuses and orbit. May also present with proptosis if lamina papyracea is breached

7. Coronal T1-weighted magnetic resonance image (MRI) demonstrating a tumor ( black arrows ) with intermediate intensity in the right maxillary sinus Coronal T2-weighted MRI demonstrating a tumor with intermediate intensity and surrounding secretions with high signal intensity ( white arrows ) CT and MRI are complementary and both are needed to plan the appropriate surgical approach. CT is used to define the extent of the disease and to identify areas of bony erosion. Up to 75% of patients with IPs have evidence of various degrees of bone destruction, which may appear as cortical thinning, remodeling, erosion, or sclerotic changes. Areas of bony thickening may represent the site of attachment of the tumor. CT scans used alone frequently overestimate the extent of disease due to the lack of differentiation of tumors from adjacent inflammatory mucosa or retained secretions. CT alone has a sensitivity of 69% and a specificity of 20%. T2-weighted images and contrast-enhanced T1- weighted MRIs are used in conjunction to further evaluate the surrounding soft tissue and to define the extent of orbital or intracranial involvement. MRI allows for excellent delineation of the tumor from the surrounding soft tissues, inflammation, and retained secretions. Tumors will be of intermediate intensity on T2-weighted images, whereas the inflammatory component will be of high signal intensity. Additionally, the tumor will have intermediate intensity on T1 images with IV gadolinium enhancement; retained secretions will not enhance but will be rimmed by a bright signal from the inflamed mucosa. INVERTED PAPILLOMA:

8. INVERTED PAPILLOMA: STAGING: TYPICAL ENDOSCOPIC APPEARANCE OF AN INVERTED PAPILLOMA. A POLYPOID LESION WITH A PALE, PAPILLARY SURFACE PROTRUDES FROM THE MIDDLE MEATUS AND EXTENSIVELY FILLS THE LEFT NASAL CAVITY

9. Medial maxillectomy is the treatment of choice. It can be performed by lateral rhinotomy or sublabial degloving approach. Although endoscopic approach is being more preferred nowadays. Up to 20% of inverted papillomas may show surface keratinization, and anywhere from 5% to 10% may show varying degrees of dysplasia. These two latter features, although not necessarily indicative of malignancy, should reinforce the need for thorough histologic evaluation of the lesion. Recurrence does not correlate with the subsequent development of malignancy. When malignancy does occur, it most frequently manifests in the form of squamous cell carcinoma; however, verrucous carcinoma, mucoepidermoid carcinoma, spindle cell carcinoma, and clear cell carcinoma also may be seen. Wide surgical resection is the mainstay of treatment for inverted papillomas and can be performed endoscopically or by an open approach based on the extent and location of the tumor INVERTED PAPILLOMA: TREATMENT:

10. EXOPHYTIC PAPILLOMA/ TRANSITIONAL CELL PAPILLOMA/ FUNGIFORM PAPILLOMA/ SEPTAL PAPILLOMA: These are the papillomas arising from the Schneiderian membrane with rich papillary fronds and fibrovascular cores covered by layers of epithelium . These tumours are more commonly seen in men (2–10 times) between 20–50 years of age. In contrast to inverted papilloma------ These variants are localised more on the lower anterior nasal septum with no significant lateralisation . They are infrequent on the lateral nasal wall. Malignant change in exophytic papilloma is rare.

11. ONCOCYTIC/ CYLINDRICAL CELL PAPILLOMAS: These tumours also arise from the lateral nasal wall and like inverted papillomas . They are in association with malignant disease. Histological picture shows that the epithelial lining contains multiple layers of eosinophilic cells, goblet cells, and microcysts full of mucin. The high content of cytochrome C oxidase and mitochondria establishes this oncocytic appearance. In contrast to inverted papilloma, there is equal distribution among the sexes and HPV has not been isolated from these variants.

12. INTRANASAL MENINGOENCEPHALOCELE: Meningoencephalocele is usually seen in infants and young children. It is herniation of brain tissues and meninges through foramen cecum or cribriform plate. Clinical: A smooth polyp like mass in the upper part of nose between the septum and middle turbinate can be seen, which increases in size on crying or straining. D/D: It can be easily misdiagnosed as a polyp, which if avulsed results in CSF rhinorrhea and meningitis. Biopsy: It is contraindicated. CT scan: It demonstrates the defect in the base of skull. Treatment: Frontal craniotomy with severing the stalk from the brain is done along with the repair of dural and bony defect. After sealing the cranial defect, intranasal mass is removed. FURSTENBERG'S TEST- compression of the ipsilateral internal jugular vein usually causes an encephalocele to enlarge. So, this test is positive in encephalocele.

13. EXTRANASAL MENINGOENCEPHALOCELE: Herniation of brain tissue with meninges occurs through a congenital bony defect, which may be intranasal or extranasal . Clinical features: An extranasal meningoencephalocele presents with pulsatile swelling. This cystic swelling is reducible and shows cough impulse. Swelling may be present in the following sites: Root of nose ( naso -frontal variety). Side of nose ( naso -ethmoidal variety). Anteromedial aspect of the orbit ( naso -orbital variety). Treatment: It needs neurosurgery, which includes severing the stalk from the brain and repairing the bony defect .

14. GLIOMA : Most of the gliomas, which are nipped off portions of encephalocele (during embryonic development), are extranasal (60%). Other types include intranasal (30%) and both intra and extranasal (10%). EXTRANASAL GLIOMAS: Clinical feature: Firm encapsulated subcutaneous swellings may be seen on nasal bridge, side of nose or near the inner canthus. Treatment: Glioma is removed by external nasal approach. INTRANASAL GLIOMAS: Patients are infants and children. It presents as a firm polyp and may be seen protruding at the anterior nares. FURSTENBERG'S TEST- compression of the ipsilateral internal jugular vein usually causes an encephalocele to enlarge, but not a glioma. So, this test is negative in nasal glioma.

15. NASAL DERMOID: There occurs widening of upper part of nasal septum with splaying of nasal bones and hypertelorism. Types: Simple dermoid: It presents as a midline swelling over the nasal bones and does not have any external opening. Dermoid cyst with sinus: External pit: Infants and children present with a pit or a sinus over the dorsum of nose. Hair may be protruding out from the sinus. Intracranial connection: The sinus track communicate intracranially. Dermoid cyst lies between nasal bones and upper part of septum. Treatment: Surgery consists of splitting of the nasal bones and removal of cyst along with its extension in the upper part of the nasal septum. Intracranial extension needs associated neurosurgical approach. Simple dermoid lies superficial to nasal bones Dermoid with sinus. Dermoid situated deep to nasal bones and sinus tract has an external pit in the midline of dorsum of nose Dermoid with intracranial connection

16. OSTEOMA vs CHONDROMA Osteoma: Osteoma is formed of mature lamellar bone. The most common site is frontal sinus followed by ethmoid and maxillary. They usually remain asymptomatic and are seen incidentally on X-rays. The obstruction to the sinus ostium can lead to mucocele and pressure symptoms in the orbit, nose and cranium. Treatment: Symptomatic osteoma needs surgical removal. Chondroma: The various types include mixed, and fibro, osteo and angio- chondromas. Histological differentiation between benign and malignant tumors is not completely defined. The common sites of origin are ethmoid, nasal cavity and nasal septum. They present as smooth, firm and lobulated growth. Treatment is wide excision because of their tendency to malignant transformation.

17. FRONTAL SINUS OSTEOMA ALAR CARTILAGE CHONDROMA NASAL CAVITY CHONDROMA

18. MONOSTOTIC FIBROUS DYSPLASIA Site: The most common site is maxilla followed by ethmoid and frontal sinuses. Clinical features: Patient develops disfigurement of the face, nasal obstruction and displacement of the eye. Treatment: Wide removal (surgical resculpturing ) provides good cosmetic and functional results. FIBROUS DYSPLASIA This benign non-odontogenic jaw neoplasm is a hamartomatous lesion. Normal bone is replaced by fibrous tissue, which calcifies in an abnormal pattern. It involves jaw bones, more commonly in mandible than maxilla occasionally arising within the maxilla. They may be polyostotic and monostotic forms, with monostotic being more common.

19. The plain x-ray frontal view showing the CLASSICAL GROUND GLASS APPEARANCE. The 3D CT scan showing the increased density of the left maxilla impinging on the nasal bone and the orbit. Giant fibrous dysplasia of the left maxilla impinging up on the orbit, nose and distortion of the facial features The oblique right lateral view showing the complete obstruction of the left nostril and the encroachment of the oral cavity by the swelling

20. SCHWANNOMA: It is a tumor arising from Schwann cells. Between 25% and 45% of such tumors have been observed in the head and neck region, where the vestibular nerve is the most frequent site of origin. Only 4% of head and neck schwannomas originate from the sinonasal tract. Ethmoid > Maxillary Sinus > Nasal Fossa > Sphenoid Sinus; Involvement of more than one sinus is a not uncommon finding. Age: 6-78yrs (25-55yrs). The lesion is almost always solitary, but instances of multiple schwannomas have been documented, particularly in Von Recklinghausen’s disease. Sympathetic nerves to the septal blood vessels, parasympathetic nerves to septal mucous glands, and sensory nerves to the nasal septum (nasopalatine nerve and the anterior and posterior ethmoidal nerves) have been suggested as structures of origin for a schwannoma of this region. CLINICAL FEATURES: Unilateral nasal obstruction; Epistaxis; Mucopurulent rhinorrhea ; Anosmia; Facial swelling; Proptosis and pain when the maxillary sinus is involved; Extension into the sphenoid sinus can lead to diplopia (secondary to deficits of III, IV and VI cranial nerves), deep retro-orbital pain and occipital, frontal and bitemporal headache.

21. CT clearly depicts the relationship of the lesion to the surrounding bony structures; erosion is more common with large schwannomas. The lesions usually have a mottled central lucency with peripheral intensification on contrast-enhanced CT scans. The heterogeneous appearance is related to areas of increased vascularity with adjacent non-enhancing cystic or necrotic regions. MR I is superior to CT in differentiating a tumor from inflammatory changes and normal tissues; furthermore, intracranial extension can be better delineated. Schwannomas have an intermediate signal intensity on T1-weighted images, whereas a T2-weighted signal varies from intermediate intensity (highly cellular lesion) to non-homogeneously high intensity (cystic and stromal lesions).