MALIGNANT TUMORS of the HEAD and NECKRamon P. Ramos III M.D, DPBOHNS, FPSO-HNS

PARANASAL SINUS MALIGNANCYUncommon in the general population Most common malignancy is SCCA females (95%) Neck mets. is infrequent ( metastatic potential as compared to the upper alveolus. (sub mandibular LN, deep cervical LN). 30% incidence of neck mets. Over all 5 year survival 65%. IgA-anti-VCA titer is highly sensitive and > IgA-anti-EA is highly specific. For early dis. Detection. MRI imaging of choice (soft tissue) CT better for bone involvement, LN. compliments MRI.

TREATMENTInitial treatment for all forms is radiation to the primary and both sides of the neck. Chemo. And radiation for distant mets. Better over all survival and disease free state in advance disease. Neck dissection for persistent neck disease with control of the primary.

STAGINGNo universally accepted staging classification for NPCA.American Joint Committee for Cancer Union Internationale Contre le Cancer Ho System

AJCC StagingNasopharynx: T1 confined to NP T2 tumor extends to soft tissues of orophaynx and or nasal fossa T2a no pararpharyngeal (PP) extension T2b with PP extension T3 invades bony structures and/or PNS T4 intracranial extension/cranial nerves/infra temporal fossa/hypo- pharynx/orbit involvement.

Nx cannot be assessed N0 no LN mets. N1 single ipsi. LN < 3cm N2a single ipsi. LN >3cm but 6cm

Mx cannot be assessed M0 no distant mets. M1 distant mets.

STAGE GROUPINGSTAGE 0 Tis N0 M0 STAGE I T1 N0 M0 STAGE II T2 N0 M0 STAGE III T3 N0 M0, T1-T2-T3 N1 M0 STAGEIV T4 N0-N1 M0, any T N2-N3 M0, any T any N M1

5 yr. survival WHO type 1 10%. 5 yr. survival WHO type II-III 50%. Poorer survival with higher stages.

CANCER OF THE SALIVARY GLANDSRare and accounts for 6% of all head and neck malignancies.

RISK FACTORS FOR MALIGNANCYIncreased risk Radiation exposure Full mouth dental x rays Skin cancer Rubber industry Nickel exposure Hair dye Silica dust Kerosene cooking fuels Vegetables preserved in salt

Decreased risk High intake of liver High intake of dark yellow vegetablesnote: high in vit A and C.

DIAGNOSIS OF SALIVARY GLAND CANCERClinical presentation is indistinguishable bet. benign and malignant dis. Mobile, painless non rapid growing mass is common for both benign and malignant dis. Malignant salivary neoplasms is painless mass in 75% of patients. 6%-29% patients initially have pain. 6%-13% patients with facial palsy. Pain, nerve palsy, trismus, LN pos., fixation, numbness, loose dention and bleeding suggest CA.

IMAGINGWell defined mobile tumors may be approached with out imaging. Radiologic evaluation is helpful to det. extent of disease. SIALOGRAPHY-not useful in diagnosis of malignancy of salivary glands. ULTRASOUND-limited for tissue biopsy guidance in FNAB.

CT SCAN- with contrast provides excellent detail of tumor volume, vascularity, bony structures and deep tissue involvement as well as survey of LN. MRI- excellent soft tissue detail, vascular anatomy. PET SCAN- seems to have a role in staging and to rule out distant and regional spread

FNABFor histologic confirmation and counseling. Over all accuracy of FNAB is bet. 54%98% False negative of 4% False positive of 16% Relies on ability and experience of cytopathologist

FROZEN SECTIONDistinguishes benign from malignant with 94.7% accuracy, sensitivity of 100% specificity of 87.5%. Other studies show false positive bet. 3%12%. Analysis of salivary gland CA with FS is risky. It is helpful in determining LN pathology/involvement.

STAGING

HISTOLOGIC TYPES

MUCOEPIDERMOID CAmost common salivary gland malignancy. Most common salivary malignancy in children. classified into low (G-I), intermediate (G-II) and high grade (G-III) malignancy. 5 year survival for G-I, G-II, G-III is 95%, 72%, 0% respectively.

ADENOID CYSTIC CAMost common malignancy of minor salivary glands and submandibular glands. 71% arising from the minor salivary glands. Hard palate most commonly involved in the oral cavity. 10%-15% malignancy of the parotid. Tenacious tumor. Tendency towards local and distant recurrences. Prediliction for neurotropic/nerve spread. LN involvement infrequent. High degree of distant spread.

ACINIC CELL CARare and composes 6%-8% of salivary malignancies. Low grade behavior and assoc with best survival rate of any salivary malignancy. Second most common salivary malignancy in children.

SQUAMOUS CELL CARare and most are a result of lymphatic or direct spread from skin and aerodigestive tract SCCA. Over all 5 year survival 24%-50%. Mucoepidermoid of high grade may be mistaken for SCCA.

MALIGNANT MIXED TUMORSGeneric category encompassing carcinoma expleomorphic adenoma, carsinosarcoma and metastasizing mixed tumor. Account for 5%-12% of salivary malignancies. Malignant degeneration of 3%-7% is seen in pleomorphic adenomas (carcinoma expleomorphic adenoma) True malignant mixed tumors are composed o simultaneous elements of sarcoma and carcinoma. Assoc with 50% mortality in 5 years.

ADENOCARCINOMAComprise 16%-20% of salivary malignancies. Low and high grade forms. Palate is most commonly affected in the oral cavity. Minor salivary glands-68%, Parotid-28%, submandibular 4%

MANAGEMENTSURGERY Superficial parotidectomy is the minimal surgery Total parotidectomy for deep tumor extension Extended parotidectomy involves resection of masseterand part os ascending mandible. facial nerve sacrifice is not advocated. Every attempt is made to preserve the nerve. Grafting when necessary with another sensory nerve.

NECK DISSECTION advocated for clinically positive disease Elective neck dissection of levels 1-3 is advocated for tumors > 4cm, SCCA, adeno. CA, Undiff. CA and high grade mucoepidermoid CA.

RADIATIONIndications of post op radiation: high grade tumors SCCA malignant mixed CA adeno CA high grade mucoepidermoid CA close of positive margins CN VII involvement perineural spread bone/connective tissue involvement LN mets. extranodal extension recurrent dis.

CHEMOTHERAPYPrimarily for patients with recurrent, metastatic or unresectable disease.

CANCER OF THE LARYNX

Risk factors:Laryngeal Ca - cigarette smoking thirteenfold increase among smokers - thirty-four fold increased risk if also a drinker of 1.5 li/day of wine - chemical carcinogens in workplace (asbestos, nickel, mineral oils) -genetics and susceptibility to cancer are hard to separate from lifestyle and environment - gastroesophageal reflux noted in 84% of cases

Diagnosisevaluation of hoarseness of more than 4 weeks dysphagia usually due to supraglottic or hypopharyngeal lesions airway obstruction with no apparent voice changes may represent large supraglottic or subglottic lesions endoscopy with biopsy imaging studies

MANAGEMENTUsually SCCahyperkeratosis, hyperkeratosis with atypia, carcinoma in situ, superficially invasive carcinoma (invasion deep to the basement membrane), invasive carcinoma

Glottic Caless biologically aggressive than supraglottic and hypopharyngeal Ca due to sparse submucosal lymphatics radiotherapy or consaervative management for early stage partial laryngectomy salvage surgery with total laryngectomy/ postop radiotx

Sub glottic Caunusual clinically present with airway obstruction usually require total laryngectomy because involvement of laryngeal framework is frequent ipsilateral thyroidectomy with paratracheal node dissection is necessary

Supraglottic Caearly (epiglottic) tumor may be excised endoscopically or with carbon dioxide laser preepiglottic space invasion worsens the prognosis (due to lymphatic spread to both sides of the neck) partial (supraglottic) laryngectomy transglottic involvement with cord fixation warrants total laryngectomy

NECK DISSECTIONNeck dissection or cervical lymphadenectomy refers to the systematic removal of lymph nodes with their surrounding fibrofatty tissue from the various compartments of the neck Eradicates cancer metastases to the regional lymph nodes of the neck Indications for neck dissection depend not only on the presence of palpable disease (therapeutic neck dissection) but on factors that increase the risk of occult disease, such as size and characteristics of the primary tumor (elective neck dissection)

CERVICAL LYMPH NODE GROUPSLevel I submental and submandibular (lip, buccal mucosa, anterior nasal cavity, soft tissues of the cheek)

Level II upper jugular lymph nodes - upper third of IJV adjacent to spinal accessory, extending from level of carotid bifurcation (surgical landmark) or hyoid bone (clinical landmark) to skull base Level III mid jugular nodes - below level II to junction of omohyoid muscle with internal jugular (surgical landmark) or cricothyroid memberane (clinical landmark)

Level IV lower jugular chain - from level III to clavicle Level V posterior triangle group - nodes located along the spinal accessory, along cervical transverse artery and along supraclavicular area Level VI anterior neck compartment nodes - from hyoid bone to suprasternal notch - perithyroid, paratracheal, precricoid (Delphian) nodes - thyroid gland, apex of piriform sinus, subglottis, cervical esophagus, trachea

CLASSIFICATION OF NECK DISSECTIONRadical Neck Dissection standard cervical lymphadenectomy including nonlymphatic structures (SCM, IJV, SA Modified Radical preservation of one or more nonlymphatic tissues (SCM, IJV, SA) Selective preservation of one or more nodal groups Extended - removal of additional lymphatic and/or nonlymphatic tissues

RADICAL NECK DISSECTIONDefinition - removes all ipsilateral cervical node groups extending from body of mandible to clavicle, lateral border of sternohyoid, hyoid and contralateral anterior belly of digastric, to anterior border of trapezius - levels I-V, SA, IJV, SCM, few nodes at tail of parotid Indication - extensive lymph node metastasis or extension beyond capsule of the node to involve the spinal accessory and internal jugular - node disease surrounding spinal accessory even without gross SA or IJV involvement

MODIFIED RADICAL NECK DISSECTIONDefinition - en bloc removal of lymph node bearing tissues from one side of the neck (levels I-V) with preservation of one or more nonlymphatic tissues (SCM, SA, IJV) Indication - remove probable or grossly pathologic visible lymph node disease that is not directly infiltrating or fixed to the nonlymphatic tissue; - because SA is rarely directly invaded by metastatic disease like the hypoglossal and vagus nerves which also lie in the same proximity to the nodal disease

SELECTIVE NECK DISSECTIONDefinition - en bloc removal of one or more nodal group at risk for harboring metastatic cancer, an assessment of which is based on the location of the tumor Rationale - lymphatic drainage of mucosal surfaces follow relatively constant and predictable routes - in the absence of metastasis to the first echelon nodes, lower nodes are most likely uninvolved

Supraomohyoid (Levels I-III) - oral cavity cancer who are at risk for harboring occult nodal disease - 20% risk for occult disease even if no clinical evidence of nodal disease - done as elective neck dissection on contralateral side for primary lesions involving floor of mouth, ventral or midline tongue in whom ipsilateral neck dissection is planned and no definite postop irradiation is indicated

Lateral (Levels II-IV) - removing nodal diseases associated with carcinomas originating in the pharynx, larynx, and hypopharynx - because the primary site is at midline with bilateral lymphatic drainage, neck dissection is usually done on both sides.

Posterolateral (Levels II-V) - removing nodal diseases associated with cutaneous malignancies and soft tissue sarcomas located in the posterior scalp, nuchal ridge, occiput or posterior upper neck - encompass the lympn node-bearing areas of posterior and lateral compartments of the neck

Anterior Compartment (Level VI) - eradicate nodal metastasis from the anterior compartment of the neck, with cancers originating in the thyroid gland, hypopharynx, cervical trachea, cervical esophagus, and laryngeal tumors below the glottis - removal of perithyroidal, paratracheal, pretracheal, precricoid (Delphian) nodes and those along the recurrent nerve - may be done on one side only for unilateral laryngeal and hypopharyngeal lesions

EXTENDED NECK DISSECTION- neck dissection extended to remove the retropharyngeal nodes (primary sites from pharyngeal wall or oral cavity ), hypoglossal nerve, levator scapulae muscle or the carotid artery

Loss of trapezius function due to removal of spinal accessory nerve decreased ability to abduct shoulder above

COMPLICATIONSdeformity of

90degrees at the shoulder with pain, weakness and

shoulder girdle Air leaks - circulation of air thru a wound drain - communication of wound with tracheostomy Facial/cerebral edema - due to mechanical problems with venous drainage - resolves in time after collateral circulation is established Blindness - 5 cases reported in literature - intraorbital optic nerve infarction due to intraop hypotension and severe venous distention

Chylous fistula - occurs in 1-2% of neck dissection - when apparent immediately after surgery and chylous leak exceeds 600 ml/day, early exploration is preferred before the tissues become markedly inflamed and fibrinous materials coats the tissues which may obscure important structures (e.g. vagus, phrenic n.) - if less than 600 ml/D and becomes apparent only after enteral feeding, conservative management with closed wound drainage, pressure dressing and low fat nutritional support

Bleeding Carotid artery rupture - most feared and most commonly lethal complication - exposure of carotid from flap breakdown or fistula formation (malnutrition, DM, infection, radiotherapy) - use flawless surgical techniques in closure of oral and pharyngeal defects, use of dermal grafts, levator scapulae muscle flaps and controlled pharyngostomes

LYMPHOMA OF THE HEAD AND NECKUsually present as cervical lymphadenopathy Approximately 10% of lymphomas occur in head and neck extranodal sites including Waldeyers ring paranasal sinuses, nasal cavity, larynx, oral cavity, salivary glands, thyroid, and orbit In the US, it is the second most common tumors in the head and neck region In children, presents as the most common head and neck malignancy

Hodgkins Diseaseusually present as cervical lymphadenopathy unusual to present at an extranodal site more common in male patients with a major peak in the 3rd decade of life Most important predictor of outcome is the stage of the disease increased risk for family members of patients with the disease, 10X grater incidence for same-sex siblings may have a relationship with EBV Reed-Sternberg cells pathognomonic spread from the neck to the mediastinum, spleen and liver (staging laparotomy) bone marrow

Non-Hodgkins Disease5X more frequent than Hodgkins disease in the head and neck region extranodal presentation is twice as frequent as nodal presentation predominantly disease of elderly, peak at 5th and 6th decade of life but is now changing due to association with HIV most important predictor of outcome is histologic appearance of the node classified according to morphologic appearance with usual clinical behavior (low, intermediate, high grade)

Ann Arbor StagingI A single lymph node or extralymphatic site II Two or more lymph node regions on the same side of the diaphragm or localized extralymphatic site with one or more lymph node regions on the same side of the diaphragm III Lymph ode regions on both sides of the diaphragm and possible localized involvement of an extralymphatic site or the spleen IV Disseminated involvement of one or more extralymphatic organs or tissues

ManagementHodgkins Radiotherapy for early stage (Stage I, II) Radiotherapy with chemotherapy for late stages Non-Hodgkins Low grade lymphomas are treated palliatively because they are usually not curable Truly localized diseases are treated with radiation Advanced diseases in patients below 55 y/o may evaluated for experimental chemotherapy and bone marrow transplantation Asymptomatic elderlies may be observed

Non-Hodgkins Low grade lymphomas are treated palliatively because they are usually not curable Truly localized diseases are treated with radiation Advanced diseases in patients below 55 y/o may evaluated for experimental chemotherapy and bone marrow transplantation Asymptomatic elderlies may be observed Intermediate or high grade types are approached with curative intent with combined chemo- and radiotherapies

EVALUATION OF UNKNOWN PRIMARY NECK MASSIf history, PE and routine tests do not lead to a definite diagnosis, any unknown neck mass, particularly a unilateral, asymptomatic mass corresponding to the location of known lymph node groups must be considered a metastasic neoplastic lesion unless proven otherwise Endoscopy with guided biopsy Fine needle aspiration and open biopsy Open excisional biopsy

MANAGEMENT OF UNKNOWN PRIMARYAssymmetric enlargement of one or more cervical lymph node in an adult is almost always cancerous Primary cervical malignancy is rare Almost all malignant cervical tumors are metastatic except for lymphomas Immediate removal of an enlarged lymph node for diagnostic purposes is a disservice to the patient with metastatic cervical carcinoma (increased incidence of distant mets, late regional recurrences and wound complications due to disruption of lymphatic drainage and manipulation of a metastasis decrease the chance for clean surgical excision and cure)

50-67% of cases, primary sites identified by careful routine PE Independent second survey of less visible areas of the upper digestive and respiratory tract Needle biopsy of neck mass Endoscopy is negative, sites most likely to contain an occult tumor should be biopsied Location of the node is a guide to sites for biopsy posterior triangle nasopharynx jugulodigastric tonsils, tongue base, supraglottic larynx supraclavicular digestive tract, breast, tracheobronchial tree, thyroid, genitourinary If still negative, open excision with planned surgery and neck dissection

Postop irradiation is sometimes advocated but still controversial - may compromise management of mucosal carcinoma appearing later - may induce later mucosal carcinoma - cause prolonged morbidity in the form of xerostomia, dysphagia, dental caries - cure rates higher with surgery alone - best candidates are those with N2, N3 (N1 with nodal capsular penetration)