Head & Neck Tumours Part II

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1 Head & Neck Tumours Part II
Dr. Khalid AL-Qahtani MD,MSc,FRCS(c) Assistant Professor Consultant of Otolaryngology Advance Head and Neck Oncology , Thyroid and Parathyroid,Microvascular Reconstruction, Skull Base Surgery

2 Content Tumours of the Ears Tumours of the Nose Tumours of the Mouth
Tumours of the Pharynx Tumours of the Larynx

3 Neoplasms of the Ear and Lateral Skull Base
Lesions of the Pinna and EAC Lesions of the Middle Ear and Mastoid Lesions of the Petrous Apex and Clivus Lesions of the IAC, CPA, and Skull Base

4 Introduction Generally classified by location, and occasionally by cell-type Causes of these neoplasms are largely unknown?

5 Neoplasms of the pinna and external auditory canal
Cutaneous carcinoma    Squamous cell carcinoma    Basal cell carcinoma Malignant melanoma Glandular neoplasm    Ceruminous adenoma    Ceruminous adenocarcinoma    Pleomorphic adenoma    Adenoid cystic carcinoma Osteoma and exostosis Miscellaneous neoplasm    Merkel cell carcinoma    Squamous papilloma    Pilomatrixoma    Myxoma    Auricular endochondrial pseudocyst    Chondrodermatitis nodularis chronica helicis (Winkler disease)

6 Lesions of the Petrous Apex and Clivus
Adenomatous neoplasm    Benign middle ear adenoma    Endolymphatic sac tumor Chordoma Congenital neoplasm    Dermoid    Teratoma    Choristoma Cholesterol granuloma Langerhans cell histiocytosis    Eosinophilic granuloma    Hand-Schüller-Christian disease    Letterer-Siwe disease    Sarcoma    Rhabdomyosarcoma    Chondrosarcoma    Ewing sarcoma    Osteogenic sarcoma    Fibrosarcoma

7 Neoplasms of the internal auditory canal and cerebellopontine angle
Schwannoma    Vestibular schwannoma    Facial nerve schwannoma    Trigeminal schwannoma    Jugular foramen schwannoma Meningioma Lipoma Metastases

8 Neoplasms of the Pinna and EAC
Cutaneous Carcinoma BCC BCC (20% of ear/TB neoplasms) Most on pinna Sun exposure is initiator Locally infiltrative, rolled border central crusting ulcer May invade TB if left untreated

9 Cutaneous Carcinoma SCCA
Pinna and EAC are common Sun, cold, radiation are all factors Scaly irregular indurated maculopapular lesion, often ulcerated with sero-sang d/c Can be confused with OE Other symptoms VII, CHL, SNHL (with invasion of TB) Met. To LN more common than BCC

10 Cutaneous Carcinoma Treatment
Moh’s micro surgery for most scc and bcc pinna lesions TB lesions require TB resection and RT Address LN in SCC

11 Osteomata and Exostoses
Benign bony growths in EAC Osteoma’s – solitary, pedunculated, smooth, round lesions arising from tympanomastoid and squamous suture Exostose’s – broad, more medial, multiple, often bilateral Related to cold water exposure

12 Lesions of the Middle Ear and Mastoid
Paragangliomas Most common neoplasm of middle ear but still rare Glomus tympanicum Originate on promontory of cochlea (jacobson or Arnold’s nerve) Fill ME space and ossicles involved May extend to hypotympanum and expose jugular or petrous carotid Present with HL and pulsatile tinnitus and ME mass Glomus jugulare Arise in jugular fossa Become large before symptomatic (multiple CN)

13 Lesions of the Middle Ear and Mastoid
Paragangliomas Brown sign +ve pressure leads to blanching Aquino sign – ipsilat CA compression decreases pulsation Vernet syndrome (or JF syndrome) – paresis of CN’s 9, 10, 11 Villaret Syndrome = JF syndrome plus Horners

14 Paragangliomas Rx is complete surgical excision
If secretory must address this (alpha or beta blockade) Trans canal, trans mastoid-lab, trans cervical, infra temporal, intra cranial Pre-op embolization is a neccessity If you think it invades the ICA, balloon occlusion studies must be done RT or stereotactic radiosurgery can halt disease in up to 90%

15 Lesions of the Petrous Apex and Clivus
Cholesterol granulomas Most common lesion of the petrous apex Negative pressure in lumen causes hemorrhage Expansile lesion Hearing loss, tinnitus, vertigo, facial twitching HRCT MRI diagnostic T1 and T2 hyperintense

16 Lesions of the Petrous Apex and Clivus

17 Cholesterol Granuloma
Causes: poor drainage of ME, hemmorhage, obstruction of ventilation, FB reaction to cholesterol crystals from HB catabolism Rx is surgical drainage

18 Lesions of the IAC, CPA, and Skull Base
Schwannomas (no longer acoustic) Arise from sheaths of cranial nerves Vestibular, facial, trigeminal, jugular Varied presentation HRCT Inhomogeneous enhancement Smooth mass effect MRI – definitive diagnosis T1- low intensity Marked enhancement with gadolinium on T1

19 Neoplasms of the Nose and Paranasal Sinus
Introduction Benign Lesions Malignant lesions

20 Neoplasms of Nose and Paranasal Sinuses
Very rare 3% Delay in diagnosis due to similarity to benign conditions Nasal cavity ½ benign ½ malignant Paranasal Sinuses Malignant

21 Neoplasms of Nose and Paranasal Sinuses
Multimodality treatment Orbital Preservation Minimally invasive surgical techniques

22 Epidemiology Predominately of older males Exposure:
Wood, nickel-refining processes Industrial fumes, leather tanning Cigarette and Alcohol consumption No significant association has been shown

23 Location Maxillary sinus 70% Ethmoid sinus 20% Sphenoid 3% Frontal 1%

24 Presentation Oral symptoms: 25-35% Nasal findings: 50%
Pain, trismus, alveolar ridge fullness, erosion Nasal findings: 50% Obstruction, epistaxis, rhinorrhea Ocular findings: 25% Epiphora, diplopia, proptosis Facial signs Paresthesias, asymmetry

25 Benign Lesions Papillomas Osteomas Fibrous Dysplasia

26 Papilloma Vestibular papillomas
Schneiderian papillomas derived from schneiderian mucosa (squamous) Fungiform: 50%, nasal septum Cylindrical: 3%, lateral wall/sinuses Inverted: 47%, lateral wall

27 Inverted Papilloma 4% of sinonasal tumors
Site of Origin: lateral nasal wall Unilateral Malignant degeneration in 2-13% (avg 10%)

28 Inverted Papilloma Resection
Initially via transnasal resection: 50-80% recurrence Medial Maxillectomy via lateral rhinotomy: Gold Standard 10-20% Endoscopic medial maxillectomy: Key concepts: Identify the origin of the papilloma Bony removal of this region Recurrent lesions: Via medial maxillectomy vs. Endoscopic resection 22%

29 Osteomas Benign slow growing tumors of mature bone Location:
Frontal, ethmoids, maxillary sinuses When obstructing mucosal flow can lead to mucocele formation Treatment is local excision

30 Fibrous dysplasia Dysplastic transformation of normal bone with collagen, fibroblasts, and osteoid material Monostotic vs Polyostotic Surgical excision for obstructing lesions Malignant transformation to rhabdomyosarcoma has been seen with radiation

31 Malignant lesions Squamous cell carcinoma Adenoid cystic carcinoma
Mucoepidermoid carcinoma Adenocarcinoma Hemangiopericytoma Melanoma Olfactory neuroblastoma Osteogenic sarcoma, fibrosarcoma, chondrosarcoma, rhabdomyosarcoma Lymphoma Metastatic tumors Sinonasal undifferentiated carcinoma

32 Squamous cell carcinoma
Most common tumor (80%) Location: Maxillary sinus (70%) Nasal cavity (20%) 90% have local invasion by presentation Lymphatic drainage: First echelon: retropharyngeal nodes Second echelon: subdigastric nodes

33 Staging of Maxillary Sinus Tumors

34 Staging of Maxillary Sinus Tumors
T1: limited to antral mucosa without bony erosion T2: erosion or destruction of the infrastructure, including the hard palate and/or middle meatus T3: Tumor invades: skin of cheek, posterior wall of sinus, inferior or medial wall of orbit, anterior ethmoid sinus T4: tumor invades orbital contents and/or: cribriform plate, post ethmoids or sphenoid, nasopharynx, soft palate, pterygopalatine or infratemporal fossa or base of skull

35 Treatment 88% present in advanced stages (T3/T4)
Surgical resection with postoperative radiation Complex 3-D anatomy makes margins difficult

36 Olfactory Neuroblastoma Esthesioneuroblastoma
Originate from stem cells of neural crest origin that differentiate into olfactory sensory cells. Kadish Classification A: confined to nasal cavity B: involving the paranasal cavity C: extending beyond these limits

37 Olfactory Neuroblastoma Esthesioneuroblastoma
Aggressive behavior Local failure: 50-75% Metastatic disease develops in 20-30% Treatment: En bloc surgical resection with postoperative XRT

38 Oral Cavity Cancer Introduction Premalignant Lesions Malignant Lesions

39 Epidemiology 95% are squamous cell carcinoma Risk factors
Smoking (depends on dosage and type) Alcohol Snuff dipping / tobacco chewing HPV (subtype 16) Reverse cigar smoking (India) Betel-nut chewing (Asia) ?Poor dentition / mechanical irritation (dentures)

40 Epidemiology 75% of cases occur on 10% of mucosal surface area
Area from ant FOM along gingivobuccal sulcus and lat border tongue to retromolar trigone and ant tonsil pillar Flow and pooling of carcinogen-contaminated saliva here Incidence 4% cancers in males, 2% in females (increasing in females)

41 Evaluation and Diagnosis
Lesions generally easy to see Simple biopsy under local anesthesia Important goals: Stage full extent of disease Rule out synchronous primary Evaluate for possible metastatic disease CT or MRI for T2 or greater Staging endoscopy

42 AJCC TNM Staging Primary Tumor (T) Tx: unassessable
T1: tumor 2cm or less in greatest diameter T2: tumor 2-4cm T3: tumor >4cm T4: tumor invades adjacent structures Cortical bone, deep tongue musculature, maxillary sinus, skin

43 Differential Diagnosis
Granular cell myoblastoma Minor salivary gland neoplasm Adenoid cystic, mucoepidermoid, adeno-ca. Sarcomas (rhabdo, lipo, MFH, leiomyo) Hodgkin and NH lymphoma Malignant melanoma Hairy leukoplakia, Kaposi sarcoma HIV, immunocompromised

44 Premalignant Lesions Leukoplakia Erythroplasia
Hyperkeratosis, dysplasia Malignant transformation greater in non-smokers Treatment: Surgical or laser excision Topical bleomycin, retinoids, Erythroplasia Greater risk of malignancy

45 Prognostic Factors Poor prognostic tumor factors include
Tumor thickness (3mm FOM, 5mm tongue) Stage Perineural invasion Lymphatic invasion Vascular invasion Neck/distant mets DNA ploidy Pathology

46 Treatment and posttreatment follow-up: neoplasms of the oral cavity
SURGERY Primary Resection with adequate margins; frozen section as needed Tracheostomy as needed Feeding tube optional Surgical orientation of specimen for pathologist Neck Modified/radical dissection for unilateral metastatic disease and bilateral dissections for metastases in both necks Suction drainage Perioperative care Antibiotics Hospitalization for 3–10 days Tube feedings Suction drainage for necks(s)—remove when output <25–30 mL/24-h period Suture removal 5–10 days postoperatively

47 Tumours of Pharynx Nasopharyngeal Carcinoma Oropharyngeal Carcinoma
Hypopharyngeal Carcinoma

48 Nasopharyngeal Carcinoma Introduction
Rare in the US, more common in Asia High index of suspicion required for early diagnosis Nasopharyngeal malignancies SCCA (“nasopharyngeal carcinoma”) Lymphoma Salivary gland tumors Sarcomas

49 Classification WHO classes Type I - “SCCA”
Based on light microscopy findings All SCCA by EM Type I - “SCCA” 25 % of NPC (in North Amer population) 1-2 % NPC of endemic populations moderate to well differentiated cells similar to other SCCA ( keratin, intercellular bridges)

50 Classification Type II - “non-keratinizing” carcinoma 12 % of NPC
variable differentiation of cells (mature to anaplastic) minimal if any keratin production may resemble transitional cell carcinoma of the bladder Lumped with Type III in 1991 WHO revision

51 Classification Type III - “undifferentiated” carcinoma
60 % of NPC in North Amer population, majority of NPC in young patients, and 95% of endemic cases Difficult to differentiate from lymphoma by light microscopy requiring special stains & markers Diverse group Lymphoepitheliomas, spindle cell, clear cell and anaplastic variants

52 Epidemiology Chinese native (esp Guangdong province) > Chinese immigrant > North American caucasian Both genetic and environmental factors Genetic HLA histocompatibility loci possible markers HLA-A2, B17 and Bw46

53 Epidemiology Environmental Viruses Nitrosamines - salted fish
EBV- well documented viral “fingerprints” in tumor cells and also anti-EBV serologies with WHO type II and III NPC HPV - possible factor in WHO type I lesions Nitrosamines - salted fish Others - polycyclic hydrocarbons, chronic nasal infection, poor hygiene, poor ventilation

54 Clinical Presentation
Often subtle initial symptoms unilateral HL (SOM) painless, slowly enlarging neck mass (70%) Lymphatic channels cross midline in NP, bilateral disease common Larger lesions nasal obstruction epistaxis cranial nerve involvement

55 Staging EUCC T1 – tumor confined to NP
T2 – tumor extends to soft tissue T2a: into OP or nasal cavity with no parapharyngeal extension T2b: with parapharyngeal extension (beyond the pharyngobasilar fascia) T3 – Tumor invades bony structures and/or paranasal sinuses T4 – intracranial extension, involvement of cranial nerves, infratemporal fossa, hypopharynx, orbit or masticator space

56 Treatment External beam radiation Dose: 6500-7000 cGy
Primary, upper cervical nodes Consider 5000 cGy prophylactic tx of clinically negative lower neck

57 Treatment Adjuvant Chemotherapy Stardard of care
Cisplatnium (hematologic sideeffects therefore not overlapping toxicity) 5-FU

58 Oropharyngeal Cancer Introduction
Relatively uncommon 6th and 7th decades mainly Increasing in 4th and 5th decades Male predominance SCC = 90% Tobacco and alcohol Complex, multimodal treatment Team approach

59 Anatomy Connects nasopharynx to hypopharynx Ant Circumvallate papillae
Anterior tonsillar pillars Junction of hard and soft palates

60 Anatomy Pharyngeal walls Tonsils sit in tonsillar fossa Soft Palate
Mucosa, submucosa, pharyngobasilar fascia, constrictor muscles, buccopharyngeal fascia Tonsils sit in tonsillar fossa Soft Palate Palatine aponeurosis Tensor veli palatini Levator veli palatini Uvular muscle Palatoglossus palatopharyngeus

61 Etiology SCC arise from the accumulation of multiple genetic alterations to genes important to the regulation of cell growth and death Cells have selective growth advantage Genetic Environmental Tobacco and alcohol Dose related Synergistic HPV and EBV Dietary factors Immunosuppression

62 Histopathology Premalignant lesions SCC and variants >90%
Leukoplakia Erythroplakia Lichen planus SCC and variants >90% Spindle cell – clinically and biologically similar to SCC Verrucous – fungating and slow growing, with well differentiated keratinizing epithelium and rare cellular atypia or mitosis Both invade deeply with rare mets

63 Histopathology Lymphoepitheliomas
Grow rapidly and readily mets Tonsillar region Younger patients without risk factors Adenoid squamous, adenosquamous, and basaloid SCC are rare and highly aggressive (latter two have early mets)

64 Treatment Team approach T1 and T2 – surgery or radiation
Surgeons and Radiation Oncologists SLP Oral Surgeon T1 and T2 – surgery or radiation T3 and T4 – combined modality Neck N0 and N1 – surgery or XRT N2 and N3 - combined modality Both necks treated with central lesions Retropharyngeal nodes are always treated

65 Hypopharyngeal Cancer
Incidence – 5-10% of all upper aerodigestive cancers (0.5% of all malignancies) M>F: males have 8X increased risk Females with Plummer-Vinson** Large increase in risk of developing SCC of the post-cricoid region

66 Hypopharyngeal Cancers
Risk Factors Smoking EtOH Chronic reflux disease Treatment Challenge: Patients often present with advanced disease May be complicated by severe malnutrition

67 Hypopharynx - Anatomy Abuts the oropharynx at the level of the hyoid, extends to the level of the inferior border of the cricoid 3 sub-sites: piriform fossa(e), post-cricoid region, posterior pharyngeal wall

68 Hypopharynx - Anatomy Piriform apex – junction between the post-cricoid area and the inferior aspect of the piriform fossae

69 Staging Endoscopy Most important component of procedure (secondary to obtaining Bx samples for diagnosis) is determining the inferior limit of the tumour Common site: piriform fossae, post pharyngeal wall, post-cricoid region

70 Pathology ~95% of cancers of the hypopharynx are SCC Lymphomas
Angiocentric T-cell lymphoma MALT (mucosa associated lymphoid tissue) Non-hodgkins lymphoma Adenocarcinomas May originate in the minor salivary glands of the hypopharynx Benign lesions Limpoma: < 1%, usually resected due to risk of airway obstruction

71 Surgical Tx Options Hypopharynx Based on Site of Involvement
Piriform Fossa (64%) Posterior Pharyngeal Wall (30%) Post-cricoid (4%) Treating the Neck Neck mets in 75% In N0 neck risk of occult nodes ~30-40% (all patients get neck dissections)** Risk of disant mets at presentation ~20%

72 Hypopharynx Tx Surgical Options
Procedure T stage Reconstruction Partial Pharyngectomy T1, T2 Primary closure Partial Laryngopharyngectomy T1, T2, T3 Regional or free flap Supracricoid hemilaryngectomy Endoscopic CO2 laser resection T1, T2 (possibly T3, T4) Secondary intention Total Laryngectomy with partial-total pharyngectomy T3 Primary closure vs. regional or free flap Total Laryngo-Pharyngo-esophagectomy T4 Gastric pull-up

73 Laryngeal Tumours Introduction Benign Lesions Malignant Lesions

74 Epidemiology 11 600 new cases laryngeal cancer per year in USA
~1% of all cancers (excluding skin) 79% occur in ♂ >90% are squamous cell carcinomas (SCC)

75 Etiology EtOH – supraglottic Tobacco – glottic
GERD – chronic laryngeal irritation Viral infection Asbestos Nickel Wood Isopropyl alcohol Radiation

76 Laryngeal Papillomatosis
Most common benign laryngeal tumor, HPV etiology Vocal folds and subglottis most common laryngeal sites

77 Laryngeal Papillomatosis
More prevalent in children, less common in individuals over 30 years of age HPV is transmitted to child through birth canal from cervix Risk of transmission 1:400 Papillomas appear multinodular, and may be either sessile or exophytic May resemble carcinoma-in-site or even invasive SCC

78 Exophytic, warty, friable, tan-white to red growths

79 Laryngeal Papillomatosis
Most common viral subtypes are 6 or 11, but 16 or 18 have higher potential for malignant change Hoarseness is common early symptom followed by airway obstruction and respiratory difficulty

80 Laryngeal Papillomatosis
Laryngeal papillomas presenting in adults seem to be less aggressive than juvenile form but remission rate unpredictable In adults, growth may be rapid during periods of hormone change such as during pregnancy Malignant degeneration of laryngeal papillomas rare and usually associated with history of radiotherapy, tobacco abuse or both

81 Treatment Surgery Laser microlaryngoscopy (most commonly CO2 [10.6um] or Nd:YAG [1.06um]) at power setting of 2-8W pulse or continuous Powered microdebrider Always biopsy before remainder of case proceeds

82 Cont Treatment Interferon
Bad chronic side-effects (myalgias, flu-like symptoms) Lesions tend to return after interferon finished Intralesional cidofovir (acyclic nucleoside analogue) Indole-3-carbinol (found in cruciferous vegetables, works via inhibition of estrogen metabolism) Acyclovir Photodynamic therapy

83 Supraglottic vs Glottic Disease
North America glottic cancer > supraglottic (2:1) France supraglottic > glottic (2:1)

84 Anatomy - Glottis True vocal cords Anterior and posterior commissures
Superior limit – apex of ventricle Inferior limit – 1 cm inferior to line through apex

85 Staging – Early Glottic
Tis – no invasion beyond basement membrane T1 – confined to glottis with normal mobility T1a – tumor limited to one vocal cord T1b – tumor involves both cords, no limitation in mobility

86 Staging – Early Glottic
T2 – extend into supra- or subglottis without complete vocal cord fixation T2a – involve supra- or subglottis but do not impair movement T2b – impair movement of vocal cords, but not complete fixation

87 Staging – Advanced Glottic
T3 – complete vocal cord fixation, ± paraglottic space, ± minor thyroid cartilage erosion (inner cortex) T4 – extends beyond larynx, into thyroid cartilage

88 Symptoms Hoarseness >4 weeks – investigate
Occasionally may present without hoarseness Dysphagia Hemoptysis

89 Glottic Carcinoma Early: irregular area of mucosal thickening
Advanced: exophytic, fungating, endophytic, ulcerated mass More commonly keratinizing, well to moderately differentiated In situ component Invasive component predominantly infiltrative

90 Glottic Carcinoma Up to 20% of T1 cancers have some degree of vocal cord ligament invasion

91 Glottic Carcinoma Most tumors originate on free surface of vocal cord
Anterior 2/3

92 Glottic Carcinoma

93 Treatment Early Stage: Laser or Radiation Advance Stage:
Chemo+Radiation Surgery+Radiation

94 Thank You