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
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 Anterior- midline Posterior- SCM Inferior- clavicle Superior- mandible
Posterior Triangle Anterior- post border of SCM Posterior- trapezius Superior- junction of SCM & trapezius Inferior- clavicle Midline Congenital Thyroglossal duct cyst Dermoid
Beware of the cystic neck mass in an adult Differential of Congenital Neck Masses Based on Location Differential Diagnosis of Neoplastic Neck Masses based on Location Lateral Anterior Benign Schwanomas CBTs Salivary gland
Midline Anterior Thyroid Larynx cancer Direct extension Metastasis
Posterior Benign Schwanomas Malignant Lymphoma Nodal metastasis Skin UADT Non H & N 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 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 Warthins tumor Necrotic Neck Mass Infectious Abscess Tuberculosis
Malignant Squamous cell carcinoma Work-Up of a Neck Mass History Physical Inspection Palpation Endoscopy Diagnostic Imaging US CT MRI PET Biopsy FNA Other Intraoperative endoscopy TB test History 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
Keep the differential diagnosis in mind History Past medical history Skin cancer UADT malignancy Sarcoidosis Fungal infection Dental caries/dental work Trauma to head and neck Family history Thyroid cancer Paragangliomas
Immunosupression Transplant patients- Skin cancers, head and neck cancer
Occupational Wood working, leather work paranasal sinus cancer Risk Factors Contd Previous head and neck cancer Develop second cancer in 18% of patients
Radiation exposure Salivary gland cancers, thyroid cancer, head and neck sarcomas
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 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 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 lyres sign MRI get salt & pepper pattern from the flow voids
Carotid Body Tumor Treatment Excision Proximal and distal control of CA Prepared to bypass
Squamous Cell Carcinoma FNA Dx of SCC Primary detected No Primary identified; Aka unknown primary Stage tumor Treat primary tumor Treat neck Imaging to stage the neck disease and help identify the primary source 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 2 nd 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 Local regional control Survival
Palliation 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 Parotid- 80% (80%benign:20%malignant) Submandibular 15% (50:50) Sublingual (40:60)
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 Serology if suspect auto-immune process
Biopsy 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 Ultrasound Identifying a mass Guide FNA Assessing adenopathy
Technitium-99m Scan Diagnosis of Oncocytoma or Warthins 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 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 Pleomorphic adenoma Malignant degeneration into carcinoma ex-pleomorphic adenoma in 2-10% of pleomorphic adenomas Warthins 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 Patients 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% Freys 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 (Sipples) 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 Treatment 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 (R-A) Repeatedly Nondiagnostic (R-A) Cystic nodule Solid nodule Observation or surgery Surgery strongly considered Suspicious for papillary ca or Hurthle cell neoplasm Surgery (R- A) Indeterminate Cytology (suspicious, follicular lesion or neoplasm) Follicular lesion Benign Follow (R-A) Thyroid scan Hot Cold (R-B) FNA 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
Patterns of Failure by Risk Groups Differentiated Thyroid Cancer 5 10 18 10 14 17 2 12 34 Low Intermediate High 0 5 10 15 20 25 30 35 40 Local % Regional % Distant % % of pts 13% 26% 50% Overall % 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 - I 131 Effect
Goal of therapy 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 2 nd half of pregnancy surgery may be performed after delivery Low TSH if persists after 1 st trimester thyroid scan after pregnancy