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36.8.2 Hemangiomas
• Often appear bluish and are compressible.
• CT or MRI may help dene the extent of the
neoplasm, especially intrathoracic.
• Treatment: (depend on site, size, and severity
of symptoms) most often resolve spontaneously within the rst decade.
• Surgical treatment is reserved for lesions with
rapid growth involving vital structures, which
fail medical therapy (laser or oral propranolol
in infantile type).
36.8.3 Branchial Cleft Cysts
S. S. Mohamed et al.
• Remnant of branchial cleft (second).
• Most commonly occur in the second or third
decades.
• Pain +/- (severe throbbing pain, can be
painless).
• Usually presents as a smooth, uctuant non-
tender (or tender), non-transluminal mass
mobile forwards and downwards, underlying
the anterior border of the SCM muscle.
• Branchial stula or sinus.
• Primary treatment is with control of infection
by antibiotics, followed by surgical excision.
36.8.4 Thyroglossal Duct Cyst
• This is a common congenital midline neck
mass.
• Can present as a mass in the lateral edge of the
thyroid cartilage.
• Pain and tenderness +/-.
• During clinical examintion, swelling can be
moved transversally but cannot be moved
vertically.
• Elevates on protrusion of the tongue.
• Treatment is with initial control of infection
with antibiotics, followed by surgical excision, including the mid-portion of the body of
the hyoid bone (Sistrunk’s procedure).
Occasionally, these lesions become infected
and resolve or persist following surgery as a
thyroglossal stula.
36.8.5 Sebaceous Cysts
• These are common masses often occurring in
older people but can occur at any age.
• They are slow-growing, but sometimes uctuant and painful when infected.
• Diagnosis is made clinically; the skin overlying the mass is adherent, and a punctum is
often identied.
• Excisional biopsy conrms the diagnosis.
36.8.6 Cervical Lymphadenopathy
• Acute lymphadenitis present as tender
swelling
• Antibiotic trial, less acute inammatory nodes
generally regress in size over 2–6 weeks. If
lymph node did not subside then biopsy of the
lymph node is advised.
infectious:
• Tuberculosis
• Toxoplasmosis
• syphilis
• glandular disease
Metastatic diease :
from head and neck, chest
or abdomen
Lymphoma
lymphosarcoma
Reticulosarcoma
causes of
cervical lymph
adenopathy
sarcoidosis
36 An Approach toNeck Masses
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36.8.6.1 TB Cervical Lymphadenitis
• Upper and middle deep cervical LN
• Onset: gradually
• Pain: +/-
• Systemic symptoms unusual in young
(patients with abscess (painful, increase size,
and skin discoloration))
• Mass: indistinct, rm, matted, uctuate in
case of abcess collection
• Treatment with anti-TB (6–9 months)
– Rifampicin
– Ethambutol
– INH
– Pyrazinamide
403
Coronal and axial CT neck with contrast for a patient with
TB lymphadenitis
36.8.7 Carotid Body Tumor
• Rare tumor of chemoreceptors (age of presentation: 40–60 years).
• Slow-growing painless sometimes pulsating
lump, may be bilateral.
• During examination: mass can be moved side
to side.
• Symptoms of transient cerebral ischemia
• Potato tumors (hard, non-tender)
• Palpation may induce a vasovagal attack.
• Biopsy is contraindicated: MRI angiography
is the investigation of choice.
• Surgical removal is based on patient presenting symptoms.
36.8.8 Pharyngeal Pouch
• Diverticulum of the pharynx through the gap
between the horizontal bers of the cricopharyngeus muscle inferiorly and the lowermost
oblique bers of the inferior constrictor muscle superiorly.
• History of halitosis regurgitation of froth and
food. There is no bile or acid taste to it.
• Pressure on the swelling causes gurgling
sounds and regurgitation.
• Treatment: cricopharyngeal myotomy.
AL GRAWANY
404
Anteroposterior and lateral view of barium swallow in a pharyngeal pouch
36.8.9 Thyroid Masses
• Thyroid neoplasms are a common cause of
anterior compartment neck masses in all age
groups, with a female predominance and are
mostly benign.
• Ultrasound and ne needle aspiration of thyroid masses have become the standard of care.
• Unsatisfactory aspirates should be repeated,
and negative aspirates should be followed up
with a repeat FNAC and examination in 3
months time.
S. S. Mohamed et al.
36.8.10 Ludwig’s Angina
• Rare but serious connective tissue infection of
the oor of the mouth
• Mostly due to dental infections
• Signs of inammation present (pain, swelling,
redness, tendrenss and uctuation)
• Treatment: priority to protect the airway,
drainage of the pus + antibiotic to cover aerobes and anaerobe
36 An Approach toNeck Masses
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405
36.8.11 Salivary Gland Neoplasm
1. Major salivary gland
(a) Parotid gland
(b) Submandibular gland
(c) Sublingual gland
2. Minor salivary gland
• 600–1000 minor salivary glands distributed throughout the mucosa of the upper
aerodigestive tract (more common in the
soft and hard palate).
• 80% of salivary gland tumors occur in the
parotid.
• 10–15% in the minor salivary gland.
• 5–10% in the submandibular gland.
• 80% of the parotid tumors are benign.
• The most common benign parotid neoplasm is pleomorphic adenoma.
• 50% of the submandibular gland tumors
are benign.
• 30% of the minor salivary gland tumors are
benign.
36.8.11.1 The Most Common Benign
Tumor oftheParotid
• Pleomorphic adenoma (benign mixed tumor)
• Warthin’s tumor (papillary cyst adenoma
lymphomatosum)
• Monomorphic adenoma
– Basal cell adenoma
– Canalicular adenomas
– Oncocytoma
– Myoepitheliomas
• Granular cell tumor
• Hemangioma
• FNA (ne needle aspiration) The accuracy is
around 90% depending on the techniques of
aspirate and the cyto pathologist.
• Supercial parotidectomy is considered as a
diagnostic and therapeutic for most benign
tumors.
36.8.11.2 The Most Common
Malignant Neoplasm
oftheParotid Gland
1. Mucoepidermoid carcinoma—40%
2. Adenoid cystic carcinoma—10%
3. Acinic cell carcinoma—10–15%
It is considered a low-grade tumor.
4. Malignant mixed tumor—7%
It is considered a high-grade malignancy.
5. Polymorphous low-grade
adenocarcinoma—10%
It is a low-grade variant of adenocarcinoma.
6. Adenocarcinoma—10%
It is a high-grade with poor prognosis.
7. Squamous cell carcinoma—4%
It is high-grade, more common in elderly
patients
*A case of pleomorphic carcinoma invading the skin
AL GRAWANY
406
Coronal and axial CT with contrast for left parotid mass
S. S. Mohamed et al.
36.8.12 Metastatic Lymph Nodes
• Upper cervical lymph nodes (upper aerodigestive tract).
• Accessory chain of nodes in the posterior triangle (nasopharyngeal malignancies).
• In many cases (occult primary), most common
sites are tonsil, base of the tongue, nasopharynx, and pyriform sinus.
• Virchow’s LN (left supraclavicular lymph
node): abdomen and thoracic malignancies
painless, non-tender, and hard masses.
• Workup: Search for primary, treatment
according to the primary disease.
36.8.12.1 Characteristics
ofMalignant Neck Lumps
Lymphomas
• Painless lump, non-tender, smooth, and dis-
crete slow growing.
• Patient presented with malaise, weight loss,
pallor, fever, rigor, and hepatosplenomegaly.
• Abdomen pressure on IVC (inferior venacava)
may cause bilateral leg edema, other lymph
nodes in the axilla, groin, and abdomen should
be examined.
• Treatment: according to the stage:
– Chemotherapy.
– Radiation therapy.
– Immunotherapy.
36 An Approach toNeck Masses
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Take-Home Messages
• Neck masses are common and most
often due to lymphadenopathy secondary to a self-limited infection or
inammation.
• Basic knowledge of neck anatomy and
structures is required.
• Thorough history and examination usually suggest a diagnosis.
• In the differential diagnosis, the three
most important categories to distinguish
are: infective/inammatory, congenital,
and neoplastic masses.
• Appropriate investigations may be performed at a GP (General practitioner) or
specialist level.
• Reasonable rst-line management for a
suspected infective/inammatory mass
is a course of a broad-spectrum antibiotic with referral to a specialist if the
mass does not resolve within 2–4 weeks.
• All suspected neoplastic and congenital
masses should be referred for specialist
attention.
407
Further Reading
1. Flint PW, Haughey BH, Robbins KT, Thomas JR,
Niparko JK, Lund VJ, Lesperance MM Cummings
otolaryngology- head and neck surgery E-Book: head
and neck surgery.
2. National Comprehensive Cancer Network clinical
practice guidelines in oncology: thyroid carcinoma
version 2.2013
3. Cooper DS, et al. Revised American Thyroid
Association management guidelines for patients with
thyroid nodules and differentiated thyroid cancer: the
American Thyroid Association (ATA) guidelines taskforce on thyroid nodules and differentiated thyroid
cancer. Thyroid. 2009;19(11):1167–214.
4. Current surgical therapy: management of thyroid
nodules
5. Philip S, etal. Current surgical therapy: evaluation of
the isolated neck mass.
6. Pierce, Niel. Surgery at a glance. 5th ed.
AL GRAWANY
Principles ofManagement ofHead
andNeck Cancers
AnilK.D’Cruz, RichaVaish, andHarshDhar
37
37.1 Introduction
Head and neck cancers is a term synonymous
with the squamous cell carcinomas arising from
the mucosal surfaces of the oral cavity, pharynx
(oropharynx, hypopharynx), larynx and nasal
cavity/paranasal sinuses. They are the seventh
leading cause of cancer worldwide accounting
for nearly 900,000 new cases and 450,000 deaths
annually [1]. The majority of these cancers are a
result of tobacco and alcohol abuse. With their
decreasing consumption in parts of the developed
world, there is lowering in the incidence of these
cancers. Parallel to this, the last decade has wit-
A. K. D’Cruz (*)
Apollo Group of Hospitals,
Mumbai, Delhi, Chennai, India
Department of Oncology, Apollo Hospital,
Navi Mumbai, Maharashtra, India
R. Vaish
Department of Head and Neck Oncology, Tata
Memorial Hospital, Mumbai, Maharashtra, India
Department of Head and Neck Oncology, Homi
Bhabha National Institute,
Mumbai, Maharashtra, India
H. Dhar
Department of Head and Neck Oncology, Narayana
Superspeciality Hospitals,
Howrah, West Bengal, India
nessed a rise in the incidence of oropharyngeal
cancers attributed to infection with human papillomavirus (HPV) type 16 and 18 [2]. HPV-related
cancers are the cause of 60–70% of oropharyngeal cancers in the developed world. This change
is not apparent in low- and middle-income countries where oropharyngeal cancers are still primarily tobacco related [3]. HPV-related cancers
are recognized as a distinct biological entity with
differences in epidemiology, pathophysiology
and responses to treatment (discussed in Chap.
38). This chapter aims to conceptualize the over-
all principles of management of head and neck
cancers in light of recent developments. Because
of their unique characteristics, nasopharyngeal
cancers are distinct and are covered in detail in a
separate section.
• Natural history of the disease
• Evaluation and diagnosis
• Treatment philosophy
• Principles of management of head and
neck cancers
• Management of recurrent/metastatic
cancers
• Recent advances
© Springer Nature Switzerland AG 2021
A. Al-Qahtani et al. (eds.), Textbook of Clinical Otolaryngology,
https://doi.org/10.1007/978-3-030-54088-3_37
409
410
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A. K. D’Cruz et al.
37.2 Natural History
oftheDisease
Tobacco-related head and neck cancers develop
after years of substance abuse through a welldened tumour progression model [4]. Dysplastic
and molecularly altered changes occur in the
entire upper aerodigestive tract exposed to the
carcinogenic effects of tobacco. This phenomenon, termed as eld cancerization proposed initially by Slaughter [5], predisposes patients to the
development of second primary cancers. In contrast, HPV-related oropharyngeal cancers occur
de novo in normal mucosa and therefore do not
have the increased risk of second primary
cancers.
Second primary cancers occur following treatment of the index cancer at the rate of 1–5% per
year [6]. The cumulative risk at 20years varies to
as high as 25–40% and more so if patients persist
with their addictions [7–9]. These tumours occur
in the head and neck region, oesophagus or lung.
They could be simultaneous (diagnosed at the
time of index cancers), synchronous (within
6months of diagnosis of index cancers) or metachronous (6months after diagnosis of index cancers). This must be kept in mind during workup
and follow-up.
37.3 Diagnostic Workup
Treatment is primarily inuenced by a) patientrelated factors—comorbidities, performance status, medical/family history; b) tumour
factors—tumour characteristics (HPV status) and
extent of disease. Diagnostic workup is therefore
directed towards these two areas to help glean
maximum information in an attempt to select the
best treatment, improve outcomes and limit
morbidity.
(a) Patient-related factors: A thorough history of
disease-related information and addictions/
substance abuse is elicited. Associated
comorbidities which occur in as high as
20–50% of patients must be sought for as it
has a bearing on treatment decisions [10, 11].
Fanconi’s anaemia, ataxia telangiectasia,
xeroderma pigmentosa and immunosuppressive states are known to predispose to head
and neck cancers and appropriate history
must be elucidated. Dental history and examination of dental hygiene such as sharp teeth,
ill-tting dentures and dental caries are
important.
(b) Tumour factors: Diagnosis is established by a
punch biopsy avoiding obviously necrotic
areas in the majority of head and neck tumours.
Imaging is preferably performed prior to
biopsy in obvious cases to avoid biopsyinduced artefacts. This is particularly important before positron emission tomography
(PET) imaging to avoid false positives as the
consequence of inammation. Fine needle
aspiration biopsy is a useful alternative in
deep-seated inaccessible lesions and for neck
nodes. Open node biopsy is to be avoided as it
violates tissue planes and may cause tumour
spillage. Excisional biopsy of nodes is done
only when more material is needed for further
studies (immunohistochemistry (IHC), microbiology, etc.).
Assessment of HPV status is particularly
important for oropharyngeal cancers and
patients who present with unknown primary.
p16 IHC staining because of its simplicity and
easy availability is recommended to establish
HPV infection. The expression should be
present both at the cytoplasmic and nuclear
level with an intensity of +2/3 and a diffuse
overexpression of 75% to be considered as
positive. HPV DNA (deoxyribonucleic acid)
in situ hybridization (ISH) and polymerase
chain reaction (PCR) tests have a greater specicity but are not routinely used due to cost,
the complexity of the procedure and the need
for the fresh tissue [
12].
Assessment of the extent of disease is important to stage and plan treatment. The mucosal
spread is best evaluated endoscopically, usually
an ofce procedure. Direct/microlaryngoscopic
evaluation is required for glottic cancers and to
evaluate blind spots, e.g. post cricoid region
and apex of the pyriform fossa. Evaluation
under anaesthesia is useful when exible
AL GRAWANY
37 Principles ofManagement ofHead andNeck Cancers
411
endoscopy and imaging are incongruous with
clinical ndings, e.g. root of tongue involvement. Imaging gives the true deep extent of disease. Contrast enhanced computed tomography
(CECT) is the workhorse and is the investigation of choice in the majority of head and neck
tumours. It is excellent for assessment of cortical bone and cartilage involvement and also has
a high sensitivity for neck nodes. Magnetic
resonance imaging (MRI) with its better soft
tissue delineation is usually preferred for the
tongue, oor of the mouth and oropharyngeal
cancers. It is ideal for perineural spread, dural/
intraorbital involvement and marrow inltration. Its limitation is that early cartilage
and bone erosion may be overestimated.
Involvement of skull base, encasement of the
internal carotid artery, inltration of prevertebral fascia and mediastinal extension of the disease are denite signs of inoperability [13].
PET scan is indicated when there is a high
likelihood of distant metastasis (locally
advanced disease (T4), large bulky nodes (N2/
N3), lower level nodes (levels III/IV), hypopharyngeal cancers and recurrent tumours)
[14]. In cost constraint settings, a CECT thorax is a suitable replacement given that the
lung is the most common site of distant metastasis. Kim etal. demonstrated that the detection rates for pulmonary metastasis between
PET CT and CECT thorax were essentially the
same [15]. PET CT scan is also indicated for
response assessment following chemoradiotherapy (CRT) which is ideally performed
12 weeks after completion of treatment to
avoid false positive nding [16]. Given its
very high negative predictive value in this setting, a negative PET can be interpreted as the
absence of disease with upwards of 90%
surety [17].
PET scan is also the imaging modality of
choice for an unknown primary and helps identify the site of origin in 25–50% of cases [18,
19]. Blind biopsies which were in vogue in the
past are to be deprecated and should be directed
based on imaging ndings. With changing epidemiology, 90% of patients presenting with an
unknown primary are HPV associated of oro-
pharyngeal origin [12] and establishing HPV
status is useful. Some authors recommend a
routine tonsillectomy (unilateral/bilateral) and
carpet excision of the ipsilateral base of tongue
which can locate the primary in as high as 90%
of cases [20]. Another site commonly associated with the unknown primary is the nasopharynx which is often Epstein–Barr Virus
(EBV) associated. Detecting EBV encoded
RNA (EBER) with ISH helps establish a nasopharyngeal primary [12].
37.4 Pathology
World health organization (WHO) describes various histological variants of SCC, namely acantholytic, adenosquamous, basaloid, cuniculatum,
papillary, spindle cell or sarcomatoid and verrucous carcinoma. Although having specic pathological characteristics and disease biology, these
variants are all essentially treated similarly [21].
Verrucous cancers are unique in that differentiating them from verrucous hyperplasia is often difcult and deep biopsies may need to be performed
to obtain representative sections demonstrating
invasion of the basement membrane (essential
prerequisite to establish cancer). Up to 10–20%
of verrucous carcinoma may be hybrid and reveal
foci of SCC on nal histology.
Histopathology is preferably reported by dedicated pathologists and captured in a synoptic
reporting proforma to ensure recording of essential parameters to prognosticate and plan treatment. Histological parameters are best captured
under the headings of essential, desirable and
optional (Table37.1).
37.5 Current American Joint
Committee onCancer (AJCC)
Staging Eighth Edition
Highlighting Major Stages
The current staging system followed is the eighth
edition AJCC TNM staging system [22]. This
replaced the seventh edition from 2018 [23]. The
major changes are in the T stage of oral cancers
412
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Table 37.1 Synoptic pathology report of head and neck tumours
Essential Desirable Optional
•
T stage
•
– Size (bidimensional)
– Depth of invasion
•
Contiguous structure involvement
– Skin
– Muscle
– Bone
– Cartilage
•
Nodes
– Size
– Number (positive/total nodes harvested)
– Level
– Extra nodal extension
• p16 IHC for HPV status in oropharyngeal
cancer
• Tumour morphology
– Endophytic
– Exophytic
– Ulcerative
– Inltrative
•
Grade
Lymphovascular invasion
•
•
Perineural invasion/spread
Extra nodal extension
•
– Microscopic (≤2mm)
– Macroscopic (>2mm)
Worst pattern of invasion (WPOI)
•
•
Histological variants of SCC
EGFR expression
Markers of
•
immunotherapy
when relevant (e.g.
PDL1, PD1)
A. K. D’Cruz et al.
and N stage of head and neck cancers. In addition, HPV-related oropharyngeal cancer is recognized as a separate entity with a dedicated
staging.
Depth of invasion (DOI) has been incorporated into the T stage of oral cancers. DOI is
different from tumour thickness and is measured
as the perpendicular line dropped from the basement membrane of the most adjacent normal
mucosa to the deepest point of invasion. This line
is also referred to as the ‘plumb line’. The restaging denes the tumours which are ≤2 cm in
greatest dimension with DOI ≤5 mm as T1;
tumours >2cm and ≤4cm in the greatest dimension with DOI ≤10 mm and ≤2 cm in greatest
dimension with DOI >5mm and ≤10mm as T2;
tumours >4cm in greatest dimension with DOI
≤10 mm and ≤4cm in the greatest dimension
with DOI >10mm as T3; and tumours >4cm in
greatest dimension with DOI >10mm considered
now as T4 [22].
The other important change is in the N stage
with the incorporation of extranodal extension
(ENE). ENE is dened as the extension of the
carcinoma through the brous capsule of the
node into the surrounding tissue. Presence of
ENE upstages nodal disease to N2 if the size is
≤3cm and to N3 if nodal size >3cm. Both minor
and major ENE are at present given similar
importance but the AJCC task force recommends
capture and documentation of ENE as minor
<2 mm and major >2 mm from the capsule.
Studies have shown a difference in outcomes
with minor and major ENE and hence the need to
capture this data which might be of help to modify staging subsequently [24]. ENE places the
patients into a high-risk group and warrants adjuvant chemoradiotherapy.
With the growing evidence that HPV-related
oropharyngeal cancers are a biologically different disease with better prognosis, the new staging system identies these tumours as distinct
and accords a separate staging system.
Carcinoma in situ (Tis) and subdivision of T4a
and T4b have been removed from HPV positive
cancer. N staging for HPV cancers has undergone signicant change with separate clinical
and pathological N staging. Metastatic neck
nodes ipsilateral single or multiple ≤6 cm is
classied as N1 while bilateral/contralateral
nodes ≤6cm are staged as N2. Any node >6cm
in size is staged as N3. Pathological N stage is
applicable to the group of patients undergoing
surgery. The size of the node and laterality did
not impact the outcome in patients treated with
surgery. The N staging is done based on positive
neck nodes: N1 being ≤4 and N2 being >4 metastatic lymph nodes.
Another minor change is the elimination of
the stage T0 category from the staging of
unknown primary for all subsites of head and
neck except HPV-related oropharyngeal and
nasopharyngeal cancers, where a diagnosis can
be established with HPV ISH and EBER ISH in
AL GRAWANY
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