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C. Meco and H. Basak
The Antrochoanal Polyp
Antrochoanal polyps (ACPs) are benign, unilateral large polyps originating generally within maxillary sinus and rarely from sphenoid sinus extending through the natural or accessory ostium to the nasal cavity and then choana. They are frequently seen in the paediatric population and young adults with unknown aetiology and pathogenesis with a ten­dency to recur. According to one widely accepted theory, ACPs arise from maxillary antrum as a cyst due to mucus gland obstruction or ostium obstruc­tion (as a result of an allergic or infectious process). Histologically they show more inammatory and less eosinophilic cell inltration.
They clinically present with nasal obstruction, but there are some reports with epistaxis, dyspha­gia and obstructive sleep apnoea as a presenting symptom. Nasal endoscopic examination reveals a smooth-surfaced polypoid tissue from the mid­dle meatus to the nasal cavity and choana, which can also be demonstrated as soft tissue opacity on imaging studies. Nevertheless, neither the site of origin nor the differential diagnosis from other unilateral sinonasal disease cannot be determined solely by radiological assessment. For proper diagnosis nasal endoscopy with a CT scan of sinuses is crucial (Fig.31.9).
Treatment of ACP is surgical removal focus­ing on its attachment site either by cauterising
or removing/drilling underlying bone, which can nowadays successfully managed through EEA.Various EEA techniques can be utilised like middle meatal antrostomy, medial maxil­lectomy and prelacrimal endoscopic or modi­ed Denker’s approach. Total removal is curative, but if ACPs are not removed totally at its origin site, recurrence rates are high [4852].
Respiratory Epithelial Adenomatoid Hamartoma (REAH)
REAH is a benign self-limited proliferative glan­dular lesion containing disorganised mature cells commonly found medial to middle turbinate that is mostly seen in adult and male population and can be associated with nasal polyps in <48% of patients. They present as a soft tissue mass, often seen endoscopically along the olfactory groove. CT and MRI features include widening of the olfactory cleft without bony erosions. Endoscopic biopsy is recommended. The denitive diagnosis is conrmed after excision and histology, although it can cause some histopathological uncertainty and may be confused with inverted papilloma. The aim of treatment is complete endoscopic resection without taking undue risk as this is a benign lesion. The prognosis is excel­lent [53, 54].
Fig. 31.9 Antrochoanal polyp: computed tomography (CT) of the sinuses shows antrochoanal polyp causing maxillary sinus opacity and extending through natural ostium to the left nasal cavity (black arrow: extension of ACP, (*) left maxillary sinus)
Salivary Gland Tumours
Pleomorphic adenoma (PA), myoepithelioma and oncocytoma are among the benign salivary gland tumours that occur within the sinonasal cavity. They are all rare tumours, but the PA is the most frequent, usually originating from nasal septum, even though secretory glands are mostly located at the lateral nasal wall.
Typical symptoms include nasal obstruction, epistaxis, mucopurulent rhinorrhoea, epiphora and rarely external nasal deformity if the tumour arises in the anterior nasal cavity.
Imaging includes CT and MRI with contrast. Biopsy is necessary to establish precise diagnosis, especially with the risk of malignant transforma­tion, which increases with time (Fig. 31.10).
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Fig. 31.10 CT and MRI images of adenoid cystic carcinoma of nasal septum. Initial biopsy reported as salivary ade­noma. (Courtesy of Andrew Swift)
Clinical acumen and suspicion is particularly
Schwannomas andNeurobromas
important as a benign biopsy may mask underly­ing malignancy.
Malignant transformation of PA into invasive Carcinoma-Ex-PA is seen approximately 6% of pre-existing PA.Carcinoma-ex-PA can turn into an aggressive tumour [5557]. Sinonasal oncocy­tomas are also locally aggressive and have a greater potential of malignant transformation [58]. Likewise, malignant transformation of myoepitheliomas also shows a more aggressive biological course [59].
Given the risk of malignancy, the important principle is that all sinonasal benign salivary gland tumours require total surgical excision with safe margins and histological review. Endoscopic surgery (EEA) is normally possible, but should it fall short of being able to completely manage the site of tumour attachment, a traditional external approach should be utilised to achieve complete removal of the tumour and reduce the risk of recurrence [5559].
Schwannomas: Sinonasal schwannomas are benign tumours that differentiate from Schwann cells of the sensory and autonomic nerve bres along the sinonasal cavity. They are uncommon (4% of all schwannomas) and malignant transfor­mation is extremely rare. They present with non­specic nasal symptoms and occasionally have intracranial extension. Although a denitive diagnosis can be established with biopsy, it can also be suggested with a degree of condence by modern imaging: CT images show a well­demarcated solid mass with remodelling and expansion of bone due to compression; MRI with contrast displays the mass with inhomogeneous uptake and heterogenous enhancement on T1­and T2-weighted images. The main treatment is complete removal, and in most cases, this can be achieved safely by an EEA [6062] (Fig.31.11).
Neurobromas (NF): Neurobromas are
benign peripheral nerve sheath tumours and rarely
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b
c
e
d
g
Fig. 31.11 Sinonasal schwannoma (*) involving bilat­eral sphenoid sinuses, right pterygopalatine fossa (PPF) and right infratemporal fossa (ITF), (a) and (b) coronal scans of preoperative T2-weighted MRI, (c) and (d) axial scans of preoperative T1-weighted MRI with gadolinium enhancement, (e) intraoperative 45 degree endoscopic
view after resection showing right PPF and ITF, (f) intra­operative 45 degree endoscopic view after resection showing middle fossa dura (blue arrow), (g) coronal and (h) axial scans of postoperative MRI after 16years show­ing no recurrence of schwannoma after endonasal endo­scopic resection
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f
Fig. 31.11 (continued)
h
Fig. 31.12 Sinonasal neurobroma of skull base (courtesy of Andrew Swift)
seen in the sinonasal cavities. NF arise from the endoneurium of peripheral nerve sheaths, and it usually originates within the sinonasal cavity from trigeminal nerve extracranial divisions. They are uncommon and can be solitary or multiple (in patients with neurobromatosis Types 1 and 2).
Sinonasal NF has non-specic symptoms such as nasal obstruction, epistaxis, pain or asymmetry of the face. CT and MRI of the sinuses are helpful to show the extension of the disease, but histopatho­logical examination is essential to establish diag­nosis (Fig.31.12). Nevertheless, it may be difcult
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to differentiate NF from other non-epithelial sino­nasal tumours. Treatment is total surgical resec­tion; currently for sinonasal NF, EEA is a safe and effective approach. If total removal is achieved, recurrence rates are rare [6365].
Haemangioma
Haemangiomas are benign vascular tumours commonly seen in the head and neck but rarely in sinonasal cavity. Aetiological factors are uncer-
a
tain: Suggestions include trauma (multiple nasal packing, digital trauma, nasogastric tube place­ment), hormonal changes (pregnancy), viral oncogenes, arteriovenous malformations and excess production of angiogenic growth factor.
Subtypes include the lobular capillary haeman­gioma (LCH) and cavernous haemangioma (CH). The most common type is LCH and characterised by submucosal vascular proliferation and capillary lob­ules, seen mostly in nasal cavity and septum (Fig.31.13). CH has larger endothelium lined vascu­lar spaces and mostly seen in sinuses (Fig.31.14).
b
c
Fig. 31.13 Endoscopic view of lobular capillary haeman­gioma (LCH), (a) right nasal cavity (b) left nasal cavity (c) coronal scan CT showing anterior nasal cavity mass and
d
septal perforation (d) coronal T2-weighted MRI showing hyperintense nasal mass and septal perforation obstructing nasal cavity bilaterally through septal perforation
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a
Fig. 31.14 Cavernous haemangioma (CH) (*) of right pterygopalatine fossa (PPF) and infratemporal fossa (ITF) compressing on right Eustachian tube causing persistent
The most common presenting symptoms are epistaxis and nasal obstruction. Contrast­enhanced CT and MRI should differentiate vas­cular tumours from other neoplasms and may show expansile lesions without bony destruction or erosion and heterogenous high/low signals, respectively.
Biopsy may cause a severe bleed and should be done with caution. Angiography and selective embolisation should be considered in some situa­tions, and the MRI should be discussed with the radiologist, but is not always required as the main vessels are capillary.
Treatment is a surgical resection with curative intent. Endoscopic resection is the preferred choice (EEA) and facilitates precise clearance with removal of the tumour origin/stalk.
Pregnancy-related haemangiomas, known as a pyogenic granuloma, typically present with lesions on the anterior nasal septum. They nor­mally regress postpartum in 1–2months, but if they bleed excessively, it should be addressed during the pregnancy, according to symptom severity and pregnancy status [66, 67].
b
otitis media with effusion and mastoiditis (arrow), (a) axial and (b) coronal scans of T2-weighted MRI
Solitary Fibrous Tumour (SFT)
SFT is a very rare neoplasm consisting of vas­cular branching with spindled broblastic cells between the branches. Only 5–27% of all SFTs are located in the head and neck and even more uncommon in sinonasal cavity with non-spe­cic symptoms. Although it is a benign lesion, its aggressive clinical behaviour is unpredict­able. Characteristics include local invasion, recurrences and distant metastasis; thus the differential diagnosis from mesenchymal tumours is crucial. Biopsy should conrm the diagnosis; histology should include immuno­histochemistry stains for CD34. The optimum treatment method is total surgical excision with safe margins that can be achieved in most cases by EEA. Nevertheless, in cases where complete tumour removal is impossible with­out morbidity, or aggressive biological behav­iour is suspected, multimodal therapy methods (chemotherapy/radiotherapy) can additionally be employed although these are often unneces­sary [68, 69].
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Glomangiopericytoma
Glomangiopericytomas are borderline and low malignant potential soft tissue tumours of the sinonasal cavity. Occurrence is very rare (<0,1% of all sinonasal tumours). They are described as haemangiopericytoma-like intranasal tumours containing vascular structures but have perivas­cular myeloid differentiation. The WHO Classication in 2005 renamed the tumour glo­mangiopericytoma, but the term sinonasal-type haemangiopericytomas are used in the literature and still in widespread use. However, they are very distinct from other haemangiopericytomas in other sites within the body.
Immunohistochemistry staining is helpful in conrming the diagnosis and shows a strong diffuse reactivity to actin, similar to a glomus tumour, but they lack strong diffuse staining for CD34.
Complete surgical resection with negative margins is the best treatment option increasing disease-free survival rates, although recurrence rates are relatively high at around 10%. If total resection is not possible, chemotherapy/radio­therapy could be helpful. Metastatic disease is rare and overall survival rates are high [70, 71].
Inammatory Myoblastic Tumour
Inammatory myoblastic tumour (IMT) is an uncommon intermediate soft tissue tumour of unknown aetiology and pathogenesis. In most cases IMTs act as a benign tumours, but may be invasive and recurrent tumours that rarely metasta­size. Tumour contains spindle cells with myobro­blastic differentiation, plasma cells and lymphocytes. IMTs are more common in adults and they most commonly occur in the lung and abdomen, but can rarely be seen in the head and neck area. Sinonasal IMTs most frequently affect maxillary sinus followed by nasal cavity, nasal septum, ethmoid and sphenoid sinuses. Clinically the most common symptom is nasal obstruction, but depending to the site of origin, it may cause epistaxis, proptosis, visual changes and numbness. On CT and MRI, a soft tissue mass associated with
bony destruction may be seen, but precise diagno­sis is possible only with tissue biopsies and histo­pathology. Treatment is total surgical excision and radiotherapy. Cases with a high risk of malignant transformation (tumours >4cm, located in maxil­lary sinus and preoperative neutrophil-to-leuco­cyte ratio over 1.958, have higher risk of malignant transformation) and recurrent cases can benet from postoperative radiotherapy. Postoperative long-term follow-ups are necessary [7274].
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