What Every Surgeon Should Know About Head and Neck Surgery David P Goldstein MD FRCSC Otolaryngology-Head & Neck Surgery Surgical Oncology University Health Network David.goldstein@uhn.on.ca

Objectives Focus on approach to evaluation and management of a neck mass and Parotid masses Briefly highlight key issues in diagnosis & management of following types of neck mass Congenital disorders thyroglossal duct and branchial cleft cyst Salivary gland masses Carotid body tumor Squamous cell carcinoma

Differential Diagnosis Congenital Thyroglossal duct cyst Branchial cleft cyst Lymphangioma Inflammatory Infectious Non-infectious Neoplastic Primary malignancies Metastases to nodes

Approach to the Differential Diagnosis of Neck Masses Age Location, Location, Location Duration of symptoms Risk factors Contents of neck mass

Differential Diagnosis Age is a major determinant < 20 years – C I N 20 – 40 years - I C N > 40 years - N I C C= congenital I= inflammatory N= neoplastic

Location Anterior Triangle Posterior Triangle Anterior- midline Posterior- SCM Inferior- clavicle Superior- mandible Posterior Triangle Anterior- post border of SCM Posterior- trapezius Superior- junction of SCM & trapezius

Differential of Congenital Neck Masses Based on Location Midline Congenital Thyroglossal duct cyst Dermoid Lateral Neck/Ant Δ Branchial cleft cyst Thymic cyst Posterior Neck Δ vascular/Lymphatic malformation Beware of the cystic neck mass in an adult

Differential Diagnosis of Neoplastic Neck Masses based on Location Midline Anterior Δ Thyroid Larynx cancer Direct extension Metastasis Posterior Δ Benign Schwanomas Malignant Lymphoma Nodal metastasis Skin UADT Non H & N Lateral Anterior Δ Benign Schwanomas CBTs Salivary gland Malignancies Lymphoma Nodal metastasis UADT Skin Supraclavicular nodes (virchow nodes) Classically represents nodal metastases from below the diaphragm

Differential Based on Growth Rate Slow growing over years Tend to be benign or low grade malignancy Rapidly growing neck masses Infectious Malignant – tend to progress over period of weeks to a few months

Cystic Neck Mass Congenital Squamous cell cancer Thyroid Cancer Thyroglossal duct cyst Branchial cleft cyst Squamous cell cancer Oropharyngeal/ tonsil primary Thyroid Cancer WDTC present with cystic mass Classically has dark brown appearance Tail of parotid masses Warthin’s tumor

Necrotic Neck Mass Infectious Malignant Abscess Tuberculosis Squamous cell carcinoma

Work-Up of a Neck Mass History Physical Diagnostic Imaging Biopsy Inspection Palpation Endoscopy Diagnostic Imaging US CT MRI PET Biopsy FNA Other Intraoperative endoscopy TB test

History Keep the differential diagnosis in mind Duration & growth rate of the mass Malignant lesions tend to have progressive growth at more rapid rate than benign disease Location Anterior, posterior or midline Symptoms of inflammation or infection Malignant neck masses with necrosis and skin involvement may mimic invasion Associated symptoms Dysphagia, odynophagia, otalgia, hoarseness, oral cavity pain, nasal obstruction, epistaxis Suggests UADT malignancy B symptoms – fever, weight loss & night sweats Risk factors Malignancy TB exposure Cat scratch

History Past medical history Family history Skin cancer UADT malignancy Sarcoidosis Fungal infection Dental caries/dental work Trauma to head and neck Family history Thyroid cancer Paragangliomas

History- Risk Factors for Malignancy Tobacco Cigarettes, chew, betel nut, cigar Alcohol Two together are synergistic Viruses HPV- oropharynx cancer EBV- nasopharynx cancers HIV- kaposi’s sarcoma, lymphoma Immunosupression Transplant patients- Skin cancers, head and neck cancer Occupational Wood working, leather work – paranasal sinus cancer

Risk Factors Cont’d Previous head and neck cancer Radiation exposure Develop second cancer in 18% of patients Radiation exposure Salivary gland cancers, thyroid cancer, head and neck sarcomas Autoimmune disorders Sjogren’s syndrome lymphoma of salivary glands Hashimoto’s thyroiditis thyroid lymphoma

Physical Examination Neck mass Location Size Firmness Fixation Pulsatile Presence of other neck masses or enlarged nodes Movement with tongue protrusion Auscultate for bruits if pulsatile

Investigations If diagnosis of infectious or inflammatory is probable no further work up is necessary and appropriate therapy instituted Suspected inflammatory disorders may require serologic tests If there is any uncertainty in diagnosis or the suspected diagnosis is congenital or neoplastic further investigations are required When in doubt on your exam – do further investigations

Fine Needle Aspiration Diagnostic accuracy 70% to 90% Simple/ cost effective US guidance increases yield & accuracy Indication – almost any neck mass Only relative contraindication to FNA is pulsatile neck mass MOST IMPORTANT TEST- WHEN IN DOUBT PERFORM

Fine Needle Aspiration Diagnose most head and neck cancers Suspect lymphoma Send for flow cytometry Cystic neck mass Send washings Stain for thyroglobulin Still a role for FNA in infectious and inflammatory disorders C & S Presence of pus does not necessarily exclude malignancy Squamous cell carcinoma can present with necrotic nodes

Open Biopsy Almost NO role in the initial work-up of a neck mass Contraindications Pulsatile masses Parotid masses Suspected malignancies and FNA not been attempted When to do Only after work-up is completed including FNA and diagnosis is still in question FNA is non-diagnostic FNA is negative but not in keeping with clinical picture

Open Biopsy Situations in which may be indicated Lymphoma FNA is suspicious for lymphoma & further tissue needed Cystic neck mass FNA often inconclusive Send cyst fluid for cytology Do full work-up prior to open biopsy Imaging and panendoscopy of UADT

Open Biopsy Incisional vs excisional biopsy Depends upon size, location and involvement if surrounding structures and suspected pathology Keep in mind future surgery/neck dissection Make the incision in line with potential incision one would use if further neck surgery is required

Diagnostic Imaging Plain films Ultrasound/Doppler CT scan & MRI Limited role CXR Ultrasound/Doppler Useful noninvasive test Vascularity Solid vs Cystic Sensitive for adenopathy Guided FNA CT scan & MRI Location Relation to other structures Vascularity Bone invasion MRI for soft tissue Tongue No dental artifact MRA/MRV

MRI Soft tissue No dental artifact – oral & oropharynx Bone invasion

CT scans Bone imaging Soft tissue imaging Dental artifact

The Pulsating Neck Mass Differential Diagnosis Non-vascular mass situated near carotid artery Carotid body tumor (paraganglioma) Carotid artery aneurysm Work-up Image first CT with contrast or MRI If confirmed vascular mass get MRI (MRA & MRV) Avoid FNA but not end of world Incisional biopsy contraindicated

Presentation & Management of Specific Diagnosis

Thyroglossal Duct Cyst Presentation May occur at any age but most common in first 2 decades of life Midline at level of hyoid to thyroid, may be off centre May have hx of infection Classic sign is rising with tongue extrusion Diagnosis History & Physical Imaging

Thyroglossal Duct Cyst Cautions May have papillary ca arising in thyroglossal duct cyst – rare but I perform FNA Cystic nodal metastasis from papillary thyroid ca to delphian node may have similar presentation Treatment Excision – sistrunk procedure (remove cyst with track up to tongue base including central portion of hyoid bone) Cosmetic and prevent recurrent infection

Branchial Cleft Cyst Presentation Treatment mass along the anterior border of the SCM +/- a sinus tract Smooth painless slow growing unless infected, may fluctuate in size Treatment Surgical excision with removal of the tract Nerves at risk – CN IX, X, XI XII

Lymphoma hx of lymphadenopathy – non-resolving B symptoms – fever, night sweats, weight loss nodes soft mobile and rubbery, may be very large “bull neck” Diagnosis FNA- special solution & adequate amount Open biopsy- after FNA & lymphoma suspicious clinically must be sent fresh immunophenotyping & flow cytometry

Carotid Body Tumor Carotid body tumors (Paraganglioma) Arise from carotid body located at bifurcation between ICA & ECA Familial in up to 30% Bilateral or multiple Diagnosis Classic imaging characteristics Vascular mass splaying ICA and ECA – lyre’s sign MRI get salt & pepper pattern from the flow voids

Carotid Body Tumor Treatment Excision Proximal and distal control of CA Prepared to bypass Complications Vascular injury Stroke CN injury – CN IX,X,XII

Squamous Cell Carcinoma FNA Dx of SCC Primary detected No Primary identified; Aka unknown primary Stage tumor Imaging to stage the neck disease and help identify the primary source Primary Identified Treat primary tumor Treat neck Panendoscopy in OR with biopsies of tongue base, hypopharynx, nasopharynx and unilateral tonsillectomy Treat neck and potential primary sites with radiation

Squamous cell carcinoma General Management Principles Staging Hx, Px (flex scope) Imaging CT Head and neck MR for tongue/tongue base Chest CT r/o synchronous primary Panedoscopy/Quadroscopy (EUA under GA) Esophagoscopy, Bronchoscopy, Laryngoscopy, +/- nasopharynx Used for cancers of larynx, hypopharynx and +/- oropharynx Assess the extent of the tumor & surgical resectabilty Obtain biopsy specimens Assess for 2nd primary

Squamous cell carcinoma General Management Principles Treatment Options Surgery Radiation Chemotherapy Combination of both Rads or chemo can be given pre- or post op Treat the primary site and the cervical lymph nodes Try and treat cervical lymph nodes with the same modality of therapy used for the primary site

How do we decide which treatment to offer Provide the treatment that will offer the highest survival & control rate based on literature Early stage disease often similar Advanced disease usually combination QOL and morbidity Organ preservation (larynx, hypopharynx) Preserve form and function (oropharynx Swallowing, speech, cosmesis

Goals of Treatment Cure Palliation Local regional control Survival Pain Bleeding Cosmesis

Squamous cell carcinoma General Management Principles Oral cavity – surgery Oropharynx (tonsil, tongue base)- radiation or chemoradiation Hypopharynx cancer – radiation or chemoradiation Larynx- transoral laser surgery for small tumors, radiation or chemoradiation for most Nasopharynx- chemoradiation or radiation

Adenocarcinoma FNA diagnosis of adenocarcinoma in the neck – from a distant site Lung, breast, GI, GU May require an open biopsy to get more tissue for analysis to help identify site Image chest, abdo, pelvis Rarely treat the neck b/c metastatic disease - palliative therapy to prevent obstruction of trachea or esophagus Neck dissection - Only if primary site is controlled and patient is potentially curable

Salivary Gland Masses Major Salivary Glands Minor Salivary Glands Parotid- 80% (80%benign:20%malignant) Submandibular 15% (50:50) Sublingual (40:60) Minor Salivary Glands Oral cavity/ oropharynx Larynx Nose & paranasal sinuses

Classification Hemangiomas Vascular malformations Lymphatic malformations 1st Branchial cleft cyst

Classification HIV TB Atypical TB Actinomycosis Cat-Scratch Toxoplasmosis Tularemia Fungal

History & Physical Exam Majority of neoplasms (benign or malignant) present as asymptomatic swelling Risk factors for malignancy Majority idiopathic Ionizing radiation Sjogren’s syndrome Lymphoma Skin cancers

Clinical Presentation of Cancers Pain Fixation & invasion of surrounding structures i.e. dermis, mandible Trismus Facial nerve paralysis Adenopathy

Facial Nerve Paralysis with a Parotid Mass Very rarely occurs with benign tumors 12% to 15% parotid malignancies will exhibit facial paralysis Pathologies Adenoid cystic carcinoma Poorly differentiated carcinoma SCC

Lab Tests Biopsy Serology if suspect auto-immune process FNA – mainstay Open biopsy Very rarely indicated for parotid masses: AVOID in most cases

Fine Needle Aspiration Debate about utility of FNA in parotid masses Among all H & N sites the parotid gland is associated with the highest FNA inaccuracy rates False negative rates higher then false positive Sensitivity rates reported can be as low as 38% when comes to recognizing malignant nature of parotid masses Diagnostic precision is difficult Determine high vs. low grade tumors is also difficult

Why do an FNA? Accuracy in determining benign from malignant disease Rates of ~ 90% It may help in planning surgery especially informed consent It may help in timing of surgery in resource restricted climate Change clinical approach in up to 30% of patients Results interpreted in the face of the clinical presentation and imaging

Diagnostic Imaging CT Scan and/or MRI Ultrasound Identifying a mass Guide FNA Assessing adenopathy Technitium-99m Scan Diagnosis of Oncocytoma or Warthin’s tumor Sialography Rarely used Little role in routine work-up of a parotid mass CT Scan and/or MRI Main modalities for imaging parotid neoplasms

Value of Imaging Know what you are getting into Malignancy “tip of iceberg” with deep lobe involvement Approach Malignancy Resectability Skull base Structures requiring resection Nodal status Facial nerve status Adenoid cystic carcinoma- proximal portion

Common Pathologies Benign Malignant Pleomorphic adenoma Malignant degeneration into carcinoma ex-pleomorphic adenoma in 2-10% of pleomorphic adenomas Warthin’s tumor 10% bilateral Malignant Mucoepidermoid carcinoma Adenoid cystic carcinoma Metastases from skin cancers

Prognostic Factors with Malignancy Histology High Grade Malignancies Older Age Pain at presentation Stage of primary tumor & nodal metastases Skin invasion Facial nerve dysfunction Peri-neural growth Positive margins

Malignant Secondary Neoplasms Direct extension Cutaneous SCC/BCC Lymphatic metastases SCC Melanoma Hematogenous Metastases Lung, Kidney, Breast Direct extension Metastatic SCC

Factors in Decision Making Patient factors Age Co-morbidities Patient’s concerns Tumor Factors Histology Benign vs malignant Do you have a diagnosis & how certain are we Growth rate Risk factors for malignancy

Surgery Majority can be managed with a superficial parotidectomy Subtotal parotidectomy Involvement of deep lobe Parotidectomy and transcervical approach to parapharyngeal space tumours

Surgical Complications Temporary VII nerve paresis=21% Frey’s syndrome=6% Infection=3.6% Hematoma=2.7% Hypertrophic scar=2.4% Seroma=0.8% Salivary fistula=0.4%

Indications for Post-operative Radiotherapy High grade cancers Recurrent cancers Gross or microscopic residual disease Regional lymph node metastases Evidence of locally advanced tumors

Thyroid Cancer

Epidemic of Thyroid Cancer 3.6 per 100 000 in 1973 → 8.7 per 100 000 in 2002 represents 2.4 fold increase Davies, L. et al. JAMA 2006;295:2164-2167.

Thyroid Malignancies Well-Differentiated Carcinomas (80-85%) Papillary Thyroid Carcinoma (PTC) Follicular Thyroid Carcinoma (FTC) Medullary Thyroid Carcinoma (5-10%) Anaplastic Thyroid Carcinoma (5-10%) Other malignancies Lymphomas Distant Metastases

Well-Differentiated Thyroid Carcinoma Papillary Thyroid CA 75-80% of thyroid carcinomas Frequently Multifocal Dx on FNA or FS Common Nodal Dz Infrequent Distant Dz Slightly Better Prognosis Follicular Thyroid CA 5-10% of all thyroid carcinomas more aggressive natural history Solitary Lesion Dx on final path Infrequent Nodal Dz Common Distant Dz Slightly Worse Prognosis

Medullary Thyroid Carcinoma C - cell/parafollicular cell origin May be sporadic/nonfamilial (80%) or familial (20%) Familial forms Medullary thyroid carcinoma alone MEN 2A (Sipple’s) MTC, Pheochromcytoma, Hyperparathyroidism MEN 2B MTC, Pheochromocytoma, Mucosal Neuromas, Mutations on chromosome 10 for the RET proto-oncogene Regional lymph node metastases - 50% Distant metastases

Medullary Thyroid Carcinoma Diagnosis / Screening Pentagastrin Stimulation with measurement of calcitonin levels Ret proto-oncogene screening Patients who screen positive should undergo early thyroidectomy Early intervention has resulted in 85% DFS at 15-20 years Serum calcitonin levels are used as a tumor marker in follow-up

Medullary Thyroid Carcinoma Treatment exclude pheochromocytoma total thyroidectomy central compartment lymphadenectomy elective lateral neck dissection for patients with palpable thyroid disease therapeutic lateral neck dissection for patients with palpable neck disease Adjuvant external beam radiation may be used to enhance locoregional control The role of chemotherapy remains to be defined

Anaplastic Carcinoma Rare tumor noted for its rapid growth and nearly uniform lethal nature Typically develops in a pre-existing well differentiated thyroid carcinoma or a goiter Poor prognostic factors Advanced age Presence of regional or distant metastases

Lymphoma of Thyroid Gland

Thyroid Nodules Approximately 95% of thyroid nodules are benign 4-7% of adults have thyroid nodules Women > men Likelihood of malignancy=5% Malignancy in clinically apparent nodules=20%

Work-up of Thyroid Nodule History exposure to ionizing radiation family history of thyroid carcinoma or other endocrine neoplasms (MEN syndromes) Physical examination Vocal cord paralysis Fixed and firm Cervical nodes

Investigations FNA Thyroid U/S TSH No role for calcitonin, thyroglobulin and thyroid scintigraphy in the initial work-up

FNA FNA (R-A) Repeatedly Nondiagnostic (R-A) Indeterminate Cytology (suspicious, follicular lesion or neoplasm) Benign Follicular lesion “Suspicious” for papillary ca or Hurthle cell neoplasm Cystic nodule Solid nodule Thyroid scan Among solitary nodules with in determinant (“suspicious”, follicular neoplasm or Hurthle cell neoplasm) risk of malignancy is ~ 20% Procedure of choice in the evaluation of a thyroid nodule (Recommendation A) Cold (R-B) Hot Surgery strongly considered Surgery (R- A) Observation or surgery Follow (R-A)

Risk-group Definitions AGES A – age (> 40) G – grade E – extent of tumor extrathyroidal invasion distant metastases S – size Other TNM & MACIS AMES A – age(M>40,F>50) M – metastases (distant) E – extent of tumor S – size

Treatment Surgery Post-operative radioactive iodine Post-operative thyroid suppression External beam radiation Post-operative screening

Total vs Less than Total Thyroidectomy Eliminates all cancer and potential cancer (up to 50% CL) Allows RAI Allows monitoring with thyroglobulin Deals with tall cell and insular Ca & prevents transformation of PTC to anaplastic ca No compelling evidence for survival advantage Difficult for RAI Thyroglobulin not possible Spares the parathyroids & RLN

Hemi vs Total Thyroidectomy Low risk disease Controversial R.R decreased with total thyroidectomy Some studies shown no difference High risk patients Local & regional RR lower in total thyroidectomy Possibly improved cause specific survival

Complications of Thyroidetcomy Hypoparathyroidism Temp vs Permanent Recurrent Laryngeal Nerve Injury Unilat vs bilat Temp vs Perm

Complications Post-operative hematoma Concern re: airway Prevent obstruction with incomplete strap muscle reapprox inferiorly Drains do not prevent Management Airway emergency Open at bedside if patient in resp distress To OR

Neck Management Clinically negative neck no neck dissection Nodal metastases at presentation Do not adversely affect survival Does increase risk of locoregional recurrence 80% of nodal metastases are central compartment Lateral ND only if clinically positive nodes or identified intra-op Functional neck dissection level II-V Spare IJV, SCM, CN XI, cervical plexus

Radioactive Iodine Agent - I131 Effect Goal of therapy Complications Scan Thyroid ablation Therapeutic Complications Short term Long term

Radioactive Iodine Only useful in cases of well differentiated thyroid malignancies Results Overall efficacy difficult to clearly delineate Studies have shown decreased locoregional recurrences and increased survival in some series Less efficacious in unresectable disease Pulmonary metastases respond better than bony metastases

Thyroid Nodules in Pregnancy Uncertainty if nodules in pregnancy are more likely to be malignant than those found in non-pregnant women No population based studies Recommendations (C) FNA unless low TSH Malignancy- follow with U/S Significant growth by 24 wks gestation surgery can be performed at that time point Remains stable or diagnosed in 2nd half of pregnancy surgery may be performed after delivery Low TSH if persists after 1st trimester thyroid scan after pregnancy Evaluation is the same except no RAI scan