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Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_30_библиотеки_им_акад_М_И_Перельмана

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G. Nayak et al.
6.2.1.2 Histopathology
It is circumscribed, non-capsulated mucosa cov­ered lobular mass lesion. Colour depends on the vascular component of the tumour (pinkish- white to red). Microscopically, it is formed by bro­cellular stoma with spindle cells and haphazardly arranged dense collagen matrix with the irregular vascular pattern. The vessels are dilated and lacks true muscle layer [42].
Differential Diagnosis Angiomatous polyp, infected antrochoanal polyp, benign (hemangi­oma, paraganglioma) or malignant tumour (hemangiopericytoma) are differential tumours for angiobroma. The demographic features, clinical features, and pattern of spread helps in differentiating these tumours from JNA [35, 43].
6.2.1.3 Treatment
Surgical treatment is the gold standard treatment. The choice of approach can vary between centres based on the surgeon's experience and hospital setup. The majority of centres are shifted from open surgical methods (lateral rhinotomy, Weber– Fergusson, transpalatal, sublabial, midfacial degloving, Le Fort 1 approach) to endoscopic method because of improving anatomical knowl­edge, advancement in technology, and expertise [44]. Endoscopic Denker’s, septal window, ante- rior maxillotomy, and small posterior sublabial incision allow easy dealing of ITF extension of JNA by endoscopic approach (Fig.6.15). Posterior
septectomy increases space within the nasal cav­ity. Literature supports the use of endoscopic exci­sion till stage IIIa of the Radkowski staging system [44]. Tumour removal in toto or piecemeal showed a similar recurrence pattern if the extension of tumour is well assessed intraoperatively.
Open anterior (lateral rhinotomy, Weber
Ferguson or maxillary swing) and lateral approach (subtemoral approach with or without craniotomy) is indicated for large volume tumour with gross skullbase involvement and when tumour is lateral to ICA, signicant residual vascularity after emboliza­tion (equal to more than 50%) (Figs.6.16 and 6.17).
SS
JNA
IT
Fig. 6.15 Endoscopic picture is showing a bloodless eld with coblator while separating JNA from the surrounding
Fig. 6.16 Maxillary swing approach. (a) Weber–Fergusson’s incision is made, (b) plating for postoperative approxi- mation, (c) raising of palatal ap
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a
c
Zygomatic arch
b
mandible
Temporal fossa
angiofibroma
Temporalis muscle
Fig. 6.17 Preauricular subtemporal approach for right side. (a) JNA 3b, (b) ap elevation, (c) exposure of tumour
Excision of residual tumour lies in close prox-
imity with ICA, dura, optic nerve and cavernous
inexperienced surgeon, and early age of onset are
the major factors for tumour residual. sinus is challenging as tumour handling can lead to signicant morbidity [45]. Hypotensive anaes­thesia, reverse Trendelenburg position, proper decongestion, preoperative embolisation, and coblation of peritumoral tissues are the measures to reduce the amount of intraoperative bleeding signicantly (Fig. 6.15). Large-volume tumour, an extension of tumour into vidian canal and inltration into the cancellous bone of skull base,
Medical Management Immunohistochemistry
shows the presence of oestrogen α, oestrogen β,
androgen, and vascular endothelial growth factor
(VEGF) receptors in tumour tissue. It is an oes-
trogen suppressive tumour and testosterone
responsive tumour. Diethylstilbestrol was ini-
tially used for shrinkage but it has a long list of
adverse effects. Flutamide therapy is a non-
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Fig. 6.18 Radiology is showing a reduction of the stage from pretreatment II C to II A of the Radkowski stage
G. Nayak et al.
Fig. 6.19 Clinical photograph is showing bilateral proptosis, with fullness in the right cheek. CECT is showing hyper­intense mass with 360-degree involvement of the bilateral carotid artery
steroidal, anti-androgenic drug without feminis­ing side effects and limited reversible adverse effects. The dose is 10mg/kg/day for 6weeks. The response rate ranged from 7 to 44% and it was found more in post-puberty patients (Fig.6.18). It is also effective in reducing episode number and amount of blood loss in the preoper­ative period [46, 47].
Radiotherapy is indicated for unresectable tumours (Fig. 6.19), in patients with persistent residual lesion even after multiple surgeries and when residual lies close to the vital structure where surgery is very much challenging. The dose ranges from 30 to 55Gy with tumour con-
trol rate ranging from 70 to 90%. The side effects are cataract, panhypopituitarism, growth retarda­tion, temporal lobe necrosis, and secondary malignancy. Recent techniques such as IMRT can reduce the risk of complication rate, but lim­ited literature is available [48, 49].
Surveillance
The advised radiological investi-
gation is MRI and it can be performed after pack removal or at 3 months postoperative period after [46]. The surrounding inammation interferes in the diagnosis of residual lesion. PMSA PET scan is indicated when MRI is doubtful. When no lesion is found in 5years’ follow-up period, then
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Fig. 6.20 Preoperative CECT and postoperative surveillance (3-months interval) MRI scan for residual detection
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the patient can be labelled as disease-free (Fig.6.20).
6.3 Part C: Cancer ofNose
andParanasal Sinuses
Cancers of the nose and PNS are very rare, and account for 0.2–0.8% of all neoplasms. Overall, they form <3% of all head and neck cancers. The symptoms of nose and PNS tumours are similar to those of common disorders such as chronic rhinosinusitis and thus cause a delay in diagnosis. The commonest age of presentation is in the fth and sixth decade of life with a male predilection (Male:Female:: 2:1) [50]. The most commonly involved sinus is the maxillary (70%)>ethmoid (20%) > frontal and sphenoid sinuses. A wide variety of histologies may be encountered although Squamous Cell Carcinoma (SCC) is the most common malignant tumour. The TNM stag­ing system is slightly complex and different for different regions of the nose and paranasal sinuses. A correct documentation of staging is required for treatment planning, prognostication, and information transfer.
6.3.1 Aetiology
Several carcinogenic compounds have been identied with inhalation of these carcinogens being responsible for ~40% of reported sinona­sal malignancies. Exposure to industrial fumes,
nickel rening processes, alkaline battery man­ufacture industry, leather tanning, soldering, welding has been reported to cause cancer of paranasal sinuses.
Squamous cell carcinoma has been associated with softwood dust, nickel, mustard gas, asbestos industry workers. African mahogany appears to be the most carcinogenic of all agents. Adenocarcinoma, especially the interstitial vari­ant, is mostly associated with hardwood dust in the furniture industry, clothes and leather indus­try. Biologically active compounds in wood dust impair mucociliary clearance and predispose to carcinogenesis [
51].
Human Papilloma Virus (HPV) infection has an increased risk of malignant transformation of inverted papilloma. However, no signicant asso­ciation between cigarette smoking and alcohol in sinonasal cancers has been noted.
6.3.2 Patterns ofTumour Spread
The most common route of spread is by local invasion. Orbit and cranium are seperate from nose and paranasal sinuses by thin bone. The paranasal sinuses other than the maxillary sinus are in close proximity to orbit and cranial cavity which makes them more vulnerable even at low volume of tumour. Natural foreman allows early spread of tumour.
Local Invasion Periosteum, perichondrium, and dura appear to act as a momentary fence and
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oppose tumour growth to some degree [52, 53]. The bone of the anterior maxilla, medial wall and oor of orbital, roof of the frontal sinus and olfac­tory cleft are very thin and readily destroyed by tumour. The clinical presentation is based on the area of involvement. Maxillary sinus tumours generally present with medial spread or anterior/ inferior wall breach. Inferior orbital ssure and thin orbital oor allows early orbital involvement but periorbita is the relatively tough lining that limits orbital tissue involvement to certain extent. Posterior breach involves infratemporal fossa, pteryomaxillary space and pterygoid plates which is relatively rare [
54]. Öhngren described a
line running from the medial canthus of the orbit to the angle of the mandible. Superiorly based tumours are more aggressive and poorly differen­tiated, whereas inferiorly based tumours are more amenable to treatment with a better prognosis. Orbit is involved by the lateral and inferior spread of ethmoid and frontal sinus tumours. Anterior cranial fossa is involved by the superior spread of tumours. Cavernous sinus is involved by lateral spread, nasopharynx by inferior spread, nasal cavity and ethmoid sinuses by anterior spread of sphenoid sinus tumours.
Regional spread: Nodal disease is a marker of
locally advanced disease and lymphatic spread to regional nodes is seen in around 25–35% of patients. Submandibular and jugulodigastric nodes are the most commonly involved lymph nodes by anteriorly placed tumours, when tumour breaches anterior bony wall, whereas, posteriorly placed tumours involve retropha­ryngeal lymph nodes. Anterior spread has a better prognosis than posterior spread.
Distant metastases: It is unusual at the time of
presentation and develops in less than 20% of patients with adenocarcinomas, whereas, for SCC, it is around 10% [55]. The most com­mon sites for metastases are bone, brain, liver, lung, and skin (Fig.6.21).
6.3.3 Clinical Features
G. Nayak et al.
Fig. 6.21 Tumour of left maxillary sinus involving ITF, pterygoid plates, pterygoid muscles, and nasal cavity
common. The usual manifestations are unilateral nasal obstruction, persistent sinusitis, recurrent epistaxis, foul-smelling nasal discharge, facial pain, etc. Epiphora is due to blockage of the nasolacrimal duct (Fig.
6.22).
Oral symptoms are seen in 25–30% of patients. These symptoms manifest in the form of loose teeth, pain in maxillary teeth, and palatal fullness (Fig. 6.23). Muscles of mastication, nerves are involved by tumour extension into infratemporal fossa or by pterygoid muscle inltration and patients are present with symptoms of trismus.
Numbness of the cheek is the sign of inferior orbital nerve inltration. Distortion of nose and face is a late feature (Fig.
6.22). Pattern of eye-
ball displacement is helpful in pinpointing the involved sinus. Auditory manifestations are in the form of hearing loss, secondary to serous otitis media due to nasopharyngeal extension. Cranial nerve II, III, IV, V1, V2, and VI nerves are seen after orbital apex and cavernous sinus involve­ment. Cranial nerve involvement is a manifesta­tion of advanced disease; it indicates poor prognosis.
The clinical presentation is depending on the site of involvement. Nasal symptoms are the most
Diagnosis Clinical staging requires detailed
local examination with neck evaluation for cervi-
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cal lymphadenopathy, cranial nerve function, and ophthalmological evaluation. Nasal endoscopy is done with a diagnostic purpose to see the charac­teristic of mass and with a therapeutic purpose to
obtain tissue for biopsy (Fig.6.24). Contrast CT scan of nose and paranasal sinuses 3–5mm axial, coronal, and sagittal images are required to docu­ment staging of the tumour. Coronal images are obtained for involvement of the orbital oor and skull base and axial images are obtained for extension through the posterior wall of the maxil­lary sinus into the pterygopalatine fossa and infratemporal fossa (Fig. 6.25). It also helps to evaluate the tumour involvement of the retro­orbital and orbital apex region. Sometimes, reconstructed images are required to pinpoint bony erosion. However, the limitations of CT scan are in the evaluation of periorbita, dural involvement to see whether the tumour has invaded or is just abutting these structures. It is
Fig. 6.22 Nasal mass (Right) with fullness of right nasal cavity ulceration of skin in medial canthus and lateral/ inferior displacement of right eyeball (clinical stage is T4). (Courtesy—Dr. Hitesh Verma, Associate Professor, AIIMS, New Delhi, India)
Fig. 6.23 Palatal bulge and ulceration with loosening of teeth by inferior spread of maxillary sinus tumour
Fig. 6.24 Nasal endoscopy is revealing a mass in the left nasal cavity (clinical stage T2)
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Fig. 6.25 CECT Nose and PNS showing the extent of a sinonasal tumour (T4a stage). MRI is showing right periorbital inltration with retained section in right maxillary sinus (T4a stage)
G. Nayak et al.
difcult to differentiate tumour from soft tissues and secretions owing to their similar densities. MRI is prescribed in such cases as it provides us better soft tissue details and it differentiates tumour from secretion [54, 56]. Angiography and embolisation have limited indications in highly vascular malignant lesions, such as hemangio­pericytoma. Histopathology is compulsory for tissue diagnosis. Unusual presentation and abnor­mal recurrence are the indications for PET scan.
6.3.4 Histopathology
Squamous cell carcinoma (SCC) is the leading histopathological diagnosis. Adenocarcinoma and adenoid cystic carcinoma accounts for around 10% of nose and PNS tumours. The tumours have a male preponderance and are seen mostly in sixth to seventh decades of life. Sino­nasal tumour mostly arise from the lateral nasal wall whereas tumours arise from paranasal sinuses. SCC is well-differentiated in a majority of cases (85%). Esthesioneuroblastoma origi­nates from basal cells of the olfactory epithelium. It accounts for less than 5% of sinonasal malig­nancies and it has bimodal age distribution (peak at 20 and 50 of age). It has female preponderance
with the potential of inducing paraneoplastic syn­drome by releasing peptides. Sinonasal undiffer­entiated carcinoma, melanoma, and hemangiopericytomas are other common differ­ential diagnoses for sinonasal tumours [
57, 58].
Staging it is devised for carcinomas of the max-
illary sinus, ethmoid sinus, and the nasal cavity and is not applicable to mesodermal tumours, olfactory neuroblastoma (OAN) for which other grading systems have been devised. TNM Classication (AJCC 2018) is proposed for nose and paranasal sinuses lesions [
59].
6.3.5 Stage withDescription
6.3.5.1 T Staging
Maxillary Sinus
T1 Tumour conned to the maxillary antrum
mucosa without bone erosion.
T2 Tumour produces bone erosion or destruc-
tion, except the posterior wall of the antrum, and it also includes tumour extension into the hard palate and/or middle meatus.
T3 Tumour inltrates in whichever of the sub-
sequent: maxillary sinus posterior wall, sub-
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cutaneous tissues over maxillary sinus, ethmoid sinus, medial wall and oor of orbit, pterygomaxillary space.
T4a Tumour inltrates in whichever of the subsequent: anterior orbit stufng, pterygoid plates, cheek skin, cribriform plate, infratem­poral fossa, sphenoid or frontal sinuses (Fig.6.25).
T4b Tumour inltrates in whichever of the subsequent: apex of orbit, dura, brain, cranial nerves except maxillary division of trigeminal nerve, nasopharynx, or clivus.
Ethmoid Sinus
T1 Tumour conned to the ethmoid sinuses with or without bony erosion.
T2 Tumour extends into the nasal cavity.
T3 Tumour extends into anterior orbit and/or maxillary sinus.
T4a Tumour inltrates in whichever of the subsequent: anterior orbital stufng, skin over nose or cheek, negligible anterior intracranial extension, pterygoid plates, sphenoid or fron­tal sinuses.
T4b Tumour inltrates in whichever of the subsequent: orbit apex of orbit, dura, brain, cranial nerves except maxillary division of tri­geminal nerve, nasopharynx, or clivus.
Nasal Cavity
T1 Tumour involves only one subunit of the subsequent: septum, oor, lateral wall, and vestibule of the nasal cavity.
T2 Tumour involves two subunits or ethmoid.
T3 Tumour inltrates into the orbit anteriorly and/or maxillary sinus.
T4a Tumour invades any of the following: anterior orbital stufng, skin over nose or cheek, negligible anterior intracranial exten­sion, pterygoid plates, sphenoid or frontal sinuses.
T4b Tumour inltrates in whichever of the subsequent: apex of orbit, dura, brain, middle cranial fossa, cranial nerves except maxillary division of trigeminal nerve, nasopharynx or clivus.
N Staging
NX Local lymph nodes cannot be assessed.
N0 No local lymph node metastasis.
N1 Metastasis in a solitary ipsilateral lymph node, 3cm or less in maximum measurement exclusive of extranodal extension.
N2
N2a—Metastasis in a solitary ipsilateral lymph node, more than 3 cm but not more than 6cm in maximum measurement exclu­sive of extranodal extension.
N2b—Metastasis in several ipsilateral lymph nodes, none more than 6 cm in maximum measurement exclusive of extranodal extension.
N2c—Metastasis in bilateral or contralateral lymph nodes, none more than 6cm in greatest dimension, without extranodal extension.
N3a Metastasis in a lymph node more than 6cm in greatest dimension without extranodal extension.
N3b Metastases in a solitary or several lymph nodes with clinical extranodal extension.
The staging template is mentioned in Table6.5.
The staging system for olfactory neuroblas-
toma is rst proposed by Kadish and then it was modied by Morita [60]:
Stage 1: Tumour conned to nasal cavity Stage 2: Tumour conned to the nasal cavity
and paranasal sinuses
Stage 3: Extension of tumour away from nose
and paranasal sinuses (intra-orbit or intracranial)
Stage 4: Any previous stage with cervical
lymph node or distal metastasis
Table 6.5 Staging template
Stage O Tis N0 M0 I T1 N0 M0 II T2 N0 M0 III T1/2 N1 M0
T3 N0/1 M0
IVa T1/2/3 N2 M0
T4a N0/1/2 M0
IVb T4b Any N M0
Any T N3 M0
IVc Any T Any N M1
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G. Nayak et al.
Dulgurov proposed a new staging system for
esthesioneuroblastoma [61]:
• T1 is conned to the nose and PNS without superior ethmoid cells and sphenoid sinus involvement.
• T2 is T1+sphenoid sinus with or without ero­sion of cribriform plate.
• T3 is any stage with intraorbital and intracra­nial extension without dural inltration.
• T4 is brain parenchyma involvement.
The presence of regional and distal metastasis
is marked as N1 and M1. Dural involvement is noticed in 25% and lymph node metastasis in 5% of cases. Hyems etal. proposed a histopathologi­cal grading system on the basis of tumour cell architecture, degree of differentiation, nuclear pleomorphism, mitotic index, tumour necrosis, and nature of the matrix. They graded histology into four subtypes where type 1 is least aggres­sive and type 4 is most aggressive [62].
Surgical and other management for sinonasal
malignancies is mentioned in Chaps. 7 and 10.
6.4 Part D: Nasopharyngeal
Carcinoma
6.4.1 Summary
Nasopharyngeal carcinoma (NPC) is the most common neoplasm arising in the nasopharynx which shows remarkable geographical difference in incidence across the world. NPC is uncommon in the United State but is endemic in many areas in the world, particularly southeastern China and Hong Kong where the incidence is as high as 20–30 cases per 100,000 population. The main factors believed to be associated with the devel­opment of NPC are EBV, genetic, and environ­mental; however, we are still unable to assimilate these information to translate knowledge into prevention, early detection, and improved sur­vival outcome. The most common clinical pre­sentation is palpable cervical lymphadenopathy (50%) followed by blood-stained nasal discharge (41%), deafness (30%), and cranial nerve palsy
in about 10% of cases. Nasopharyngosocopic examination reveals growth and provides a suit­able area for biopsy under direct visualisation. The use of immunohistochemical markers will help to differentiate NPC from other malignan­cies in the nasopharynx.
Computed tomography has been the corner­stone of radiological staging for many years; however, magnetic resonance imaging has been used increasingly because of its superior deni­tion in detecting soft tissue changes and intracra­nial involvement. Bone scan and positron emission tomography are other reliable investiga­tions to look for distant metastasis.
In recent years, treatment of NPC had under­gone several paradigm shifts resulting in improved prognosis. Advances in the planning and delivery of radiation treatment and the addition of chemo­therapy have been demonstrated to improve dis­ease control rates in locally advanced NPC. In general, patients with stage 1 are treated by radia­tion therapy alone, whereas, stage II, III, and IV are treated by concurrent radiotherapy and che­motherapy. Surgery should be considered as a rst-line treatment of residual or recurrence dis­ease at the primary site (rT1 and limited rT2). Technical advances have enabled surgeons to per­form endoscopic or robotic surgeries as salvage, reducing the morbidity from traditional open resections. Patients with NPC cases are followed up and assessed with ofce-based rigid and/or bro-optic nasal endoscopy. Positron emission tomography–computed tomography, CT, or MRI scan should be carried out at 3 months from com­pletion of treatment to assess response.
6.4.2 Anatomy ofNasopharynx
It is also known as postnasal space, located in the centre of the head behind the nasal cavity and above the oropharynx. It is a region where thor­ough clinical examination as well as adequate exposure for surgical resection is difcult. Anatomically, the roof is formed by the body of sphenoid bone, sphenoid further slants downwards in front of arch of atlas and body of axis to form the posterior wall. The oor is formed by the upper
ossa of Rosenmuller
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surface of the soft palate. The lateral wall is formed by the eustachian tube opening in the upper part and superior constrictor muscle in the lower part. Fossa of Rosenmuller, also called lateral pharyn­geal recess, is an important structure lying in the lateral wall, which is bounded anteriorly by torus tubarius and levator palatine muscle, posteriorly by the posterior wall of the nasopharynx and retro­pharyngeal space, laterally by parapharyngeal space, and inferiorly by the upper edge of superior constrictor muscle (Fig. 6.26a, b). Its superior boundary is the skull base with important open­ings including foramen spinosum, foramen ovale, and carotid canal. The internal carotid artery is
Nasopharynx on axial section
separated from the apex of the lateral recess by only a thin layer of broconnective tissue.
Blood supply of the nasopharynx is from branches of the internal maxillary artery, ascend­ing pharyngeal artery and venous drainage is to the pterygoid plexus. Sensory supply is from branches of the maxillary nerve and lymphatic supply drains into the retropharyngeal and cervi­cal lymph nodes.
Tumours of Nasopharynx A broad range of neoplasms arise in the nasopharynx from epithe­lial to mesenchymal, lymphoid, and neuroecto­dermal cells (Table6.6) [63].
F
Torus tubaris
Eustachian tube
Fig. 6.26. Axial view of nasopharynx and the endoscopic view of nasopharynx from left choana
Table 6.6 (Pathology fourth edition WHO classication)
Carcinomas Nasopharyngeal
Salivary gland tumours
Benign and borderline lesions
carcinoma
Nasopharyngeal papillary adenocarcinoma
Adenoid cystic carcinoma
Salivary gland anlage tumours
Hairy polyp Ectopic pituitary adenoma Craniopharyngioma
Non­keratinising SCC
Keratinising SCC
Basaloid SCC
8072/3 Soft tissue
8071/3 Haemato-
8083/3 Extra-osseous
8260/3 Extramedullary
8200/3 Notochordal
8272/0 9350/1
tumours
lymphoid tumours
tumours
Nasopharyngeal angiobroma
Diffuse B cell lymphoma
plasmacytoma
myeloid sarcoma
Chordoma 9370/3
9160/0
9680/3
9734/3
9930/3