Head and Neck 3 Flashcards
What is Orbital Apex syndrome?
- Affects CNs’
- II
- III
- IV
- V
- VI
- https://casereports.bmj.com/content/2017/bcr-2016-217382
- Orbital apex syndrome is a rare neuro-ophthalmic manifestation of herpes zoster virus infection. We report one such case with favourable outcome in an immunocompetent patient. A 60-year-old woman presented with rash in the dermatome of the left ophthalmic nerve (V1), followed by sudden loss of vision with complete left-sided external and internal ophthalmoplegia. MRI of brain and orbits with contrast revealed optic perineuritis and myositis without intracranial involvement confirming the diagnosis of orbital apex syndrome. Functional visual recovery was achieved after a course of intravenous and oral steroids under antiviral cover over a follow-up period of 3 months.
*
What is the pathology behind this condition?
- Thyroid Ophthalmopathy
- Orbital pathology
- Cellular infiltration and deposition of Glycoproteins and mucopolysaccharides in the orbit
- Caused by autoantibodies targeting thyrotropin receptors found in the orbit (in addition to the thyroid)
What are the clinical signs of Thyroid ophthalmopathy?
- painless proptosis
- patients may be euthryoid, hypothyroid or hyperthyroid
Functional classification of Thyroid Ophthalmopathy
ie Clinical Severity and risk
Functional classification (clinical severity and risk):
- Mild:
- eyelid lag and retraction with
- proptosis
- in setting of active hyperthyroidism
- Moderate:
- soft tissue inflammation
- intermittent myopathy;
- stabilizes without major sequelae
- Severe:
- rapid and fulminant,
- greater mass effect,
- severe sequelae
- including optic nerve compromise
Treatment of Thyroid ophthalmopathy
- prednisolone
- radiation therapy
- surgical decompression
- surgery or 131 iodine for thyroid
Radiographic features of Thyroid Ophthalmopathy
- exophthalmos/proptosis
- >2/3 globe anterior to septal line
- CT
Assessment of proptosis on cross-sectional imaging is difficult and dependant on the study being acquired in the correct plane:
the plane of the study must be parallel to the head of the optic nerve and the lens
the patient must have their eyes open and be looking forward with no eye movement
The reference line for measurement of proptosis is the interzygomatic line (a line is drawn at the anterior portions of the zygomatic bones):
the distance from this line to the posterior sclera is normally 9.9 +/- 1.7 mm 2
the distance from this line to the anterior surface of the globe should be <23 mm 4
The thickness of the extraocular muscles can also be used 1.
- muscle involvement
- mnemonic for muscle involvement
- I’m SLow
- Inferior (most common)
- Medial
- Superior
- Lateral
- I’m SLow
- Enlargement is maximal in the middle of the muscle and tapers toward the end.
- infiltrative not inflammatory disease
- spares tendon insertions, although may be involved in the acute phase
- Often bilateral, symmetric
- Straightened or streatched optic nerve
- expansion or orbital fat
- Lacrimal gland enlargement
what is this?
- Idiopathic Orbital inflammation
- AKA orbital pseudotumour
- Inflammation of orbital soft tissues of unknown origin
- Clinically
- painful proptosis
- typically unilateral (25% bilateral)
- Steroid responsive
what are the variants of idiopathic orbital inflammation?
- Tolosa Hunt:
- extends into the cavernous sinus
- Pic 1
- Sclerosing orbital inflammatory pseudotumor
- chronic progressive fibrosis
- Pic 2
- In this case, computed tomographic (CT) scanning demonstrates a mass in the nasal orbit. The arrow demonstrates the optic nerve on stretch. The eye wall (sclera) is indented and the eye pushed out (proptosis).
Rad features of idiopathic orbital inflammation
- infiltrating intraconal or extraconal inflammation presenting as ill defined infiltration or less commonly as a mass
- Typically
- unilateral
- Myositic form involves extraoular muscles
- most common
- followed by lacrimal gland involvement
- followed by other areas of involvement
- any muscle can be affected
- superior complex and medial rectus are most frequent
- Muscle enlargement
- Tendons of themuscles are involved (unlike thyroid ophthalmopathy) because it is an inflammatory disease, not an infiltrative disease like thyroid ophthalmopathy.
- Stranding of orbital fat secondary to inflammation
- DWI may be helpful for distinguishing pseudotumour from lymphoma/malignant disease
- pseudotumour generally has higher values on ADC (ie less restricted)
Detection of Occular FB
Metal
Glass
Wood
- CT > xray
- Metal
- CT 100% sensitive when object is >0.06mm
- Glass
- depends on type of glass
- Overall high sensitivity of CT for denser glass types > 1.5mm
- Wood
- difficult to detect bc wood density is similar to soft tissue
- On CT wood appears as air density with geographic margin
- Dry wood lower attenuation than fresh wood
What is the molecular pathophysiology of NF 2?
https://disorders.eyes.arizona.edu/disorders/neurofibromatosis-type-ii
what is Erdeim Chester disease
- Lipid granulomatosis
- aka non langerhans histiocytosis
- retro-orbital deposition or mass,
- xanthelasma of eyelids
- skeletal manifesations
- medullary sclerosis
- cortical thickening
- cardiopulmonary manifesations
- result of cholesterol emboli
- rare
- diffuse infiltrative mass most commonly involving intraconal space or other area in orbit
What are 6 Occular Manifestations of NF 1?
- Lisch nodules
- Optic pathway glioma
- Optic nerve sheath ectasia
- Sphenoid bone dysplasia
- Choroidal hamartoma
- Plexiform neurofibroma
Patterson, Brittany & Barton, Christopher & Lakhotia, Arpita. (2019). Unequaled overgrowth inside and out—an exceptional example of hypertrichosis overlying plexiform neurofibroma. JAAD Case Reports. 5. 670-671. 10.1016/j.jdcr.2019.04.018.
Ocular manifestations of NF 2
- Juvenile Cataracts 80%
- Epiretinal Membranes12-40%
- retinal hamartomas
- Optic nerve damage from schwannoma
- CT scans reveal calcification along the optic nerve in a “tram-track” configuration which occurs in 20-30% of patients with NF type II.
Clinical Characteristics
Ocular Features:
It is not uncommon for children to present with visual complaints secondary to cataracts, retinal hamartomas, or optic nerve damage from a schwannoma. The most common eye findings are juvenile cataracts (up to 80% of patients), followed by epiretinal membranes in 12-40%, and retinal tumors in 6-22%. Lens opacities may be located in the posterior subcapsular region or as cortical wedge-shaped opacities in the periphery where they are easily missed if the pupil is not fully dilated. Translucent, grayish epiretinal membranes with white edges are present in a significant number of patients, including children. Elevated retinal hemartomas in the macula are often associated with pigmentary changes. Lisch nodules characteristic of type I neurofibromatosis (162200) are generally not found in type II. Corneal damage may occur as a complication of hypesthesia resulting from damage to the fifth cranial nerve.
CT scans reveal calcification along the optic nerve in a “tram-track” configuration which occurs in 20-30% of patients with NF type II.
Systemic Features:
Type II neurofibromatosis often presents in the third or fourth decade of life as hearing loss accompanied by tinnitus and dizziness.
A significant proportion of children (30%) present with the same symptoms although they are more likely to complain of visual disturbances.
Type II accounts for about 10% of neurofibromatosis cases.
Acoustic neurinomas, usually bilateral, are far more common in type II (95%) and are considered diagnostically distinctive by some.
Such schwannomas also occur in other cranial and peripheral nerves.
Neurofibromas are uncommon but meningiomas, ependymomas, and astrocytomas are seen frequently.
Schwannomas can form anywhere along peripheral nerves and at least a third of patients require surgical excision of one or more of these lesions.
These account for the majority of skin plaques and lumps and are found in more than half of patients.
Café-au-lait spots are uncommon or even absent in many patients with type II.
Patients with type II neurofibromatosis do not have the cognitive problems sometimes seen in those with type I.
Longevity overall is reduced.
The average patient lives about 15 years after diagnosis and the average age of death is 36 years.
Genetics
Type II neurofibromatosis is an autosomal dominant disorder caused by mutations in the NF2 gene (22q12.2) which encodes neurofibromin-2, sometimes called merlin or schwannomin. This protein product, like neurofibromin in type I (162200), functions as a tumor suppressor. New mutations are responsible for approximately half of cases.
Cognitive deficits and Lisch nodules on the iris are more commonly found in neurofibromatosis type I (162200) but acoustic neuromas are less common. Type I results from mutations in NF1.
Treatment
Early surgical treatment of small acoustic and facial schwannomas can preserve nerve function in many cases. This is a progressive disease requiring lifelong monitoring for disease progression. The peripheral lens opacities usually do not progress and therefore cause little visual morbidity.
https://disorders.eyes.arizona.edu/disorders/neurofibromatosis-type-ii
Ocular Manifestations of Sturg-Weber
aka encephalotrigeminal angiomatosis
- choroidal haemangioma
- Buphthalmos
- Glaucoma
- Abnormal occular enhancement on MRI, thickest of the the posterior portion of the globe
Sturge-Weber syndrome, or encephalotrigeminal angiomatosis, is a phakomatosis characterised by facial port wine stains and pial angiomas.
Approximately a third of patients have choroidal or scleral angiomatous involvement, which may be complicated with retinal detachment, buphthalmos or glaucoma 1.
Sturge-Weber syndrome is a rare syndrome, with an incidence estimated at 1 case in 20,000-50,000 persons 11.
Ocular Manifesations of Sturg-Weber
https://www.researchgate.net/figure/Bilateral-Sturge-Weber-syndrome-Postcontrast-axial-MR-image-A-shows-gross_fig1_14472655
Bilateral Sturge-Weber syndrome. Postcontrast axial MR image ( A ) shows gross leptomeningeal thickening and enhancement related to the right hemisphere and less extensive abnormality of the left occipital region. Unenhanced axial T1-weighted image ( B ) shows thickening of the posterior globes bilaterally; after administration of contrast material ( C ), marked enhancement is seen in both globes. Abnormal high signal is present in this region on the proton density–weighted image ( D ). Bilateral, diffuse choroidal hemangiomas were found at ophthalmoscopy (not shown).
Ocular manifestations of Tuberous Sclerosis
- Retinal hamartomas
- Punched out areas of retinal depigmentation
- Angiofibromas of the eyelids
- Colobomas
What is the Ocular manifestation of Von Hipple Lindau?
Retinal Hemangioblastomas
Lymph node level 1a
superiorly:
inferiorly:
anteriorly:
posteriorly:
There are two sublevels:
Submental LNs
superiorly: mylohyoid muscle and mandible
inferiorly: inferior border of the hyoid bone
anteriorly: platysma muscle
posteriorly: posterior border of the submandibular gland
There are two sublevels:
level Ia (submental nodes): anteromedial between the anterior bellies of both digastric muscles
level Ib (submandibular nodes): posterolateral to the anterior belly of the digastric muscles
Level II lymph nodes
Boundaries
superiorly:
inferiorly:
anteriorly:
posterolaterally:
medially:
There are two sublevels:
Level II: upper internal jugular (deep cervical) chain
superiorly: base of the skull at the jugular fossa
inferiorly: inferior border of the hyoid bone
anteriorly: posterior border of the submandibular gland
posterolaterally: posterior border of the sternocleidomastoid muscle
medially: medial border of the internal carotid artery
There are two sublevels:
level IIa: inseparable from or anterior to the posterior edge of the internal jugular vein; includes jugulodigastric nodal group
level IIb: posterior to and separable by a fat plane from the internal jugular vein
Level III lymph nodes
Boundaries
superiorly:
inferiorly:
anteriorly:
posterolaterally:
medially:
There are two sublevels:
Level IV lymph nodes Boundaries
superiorly:
inferiorly:
anteriorly:
posterolaterally:
medially
Level IV: lower internal jugular (deep cervical) chain
superiorly: inferior border of the cricoid cartilage
inferiorly: level of the clavicle/manubrium
anteriorly: anterior border of the sternocleidomastoid muscle
posterolaterally: oblique line drawn through the posterolateral edge of the sternocleidomastoid muscle and the lateral edge of the anterior scalene muscle 2
medially: medial border of the common carotid artery
includes medial supraclavicular nodes including Virchow node 1
Level V lymph nodes
Boundaries
superiorly:
inferiorly:
anteriorly:
posterolaterally:
medially
There are two sublevels:
Level V: posterior triangle
superiorly: skull base at the apex of the convergence of sternocleidomastoid and trapezius muscles
inferiorly: level of the clavicle
anteromedially: posterior border of the sternocleidomastoid muscle
posterolaterally: anterior border of the trapezius muscle
There are two sublevels:
level Va: superior half, superior to inferior border of the cricoid cartilage (posterior to levels II and III); includes spinal accessory nodes
level Vb: inferior half, inferior to inferior border of the cricoid cartilage (posterior to level IV); includes lateral supraclavicular nodes 1
Level VI lymph nodes
Boundaries
- superiorly:
- inferiorly:
- anteriorly:
- posterolaterally:
- medially
Level VI lymph nodes
Boundaries
Level VI: central (anterior) compartment
superiorly: inferior border of hyoid bone
inferiorly: superior border of manubrium (suprasternal notch)
anteriorly: platysma muscle 8
posteriorly: trachea (medially) and prevertebral space (laterally) 8
laterally: medial borders of both common carotid arteries (medial to levels III and IV)
includes anterior jugular, pretracheal, paratracheal, prelaryngeal/precricoid (Delphian), and perithyroidal nodes
Level VII lymph nodes
Boundaries
superiorly:
inferiorly:
anteriorly:
posterolaterally:
medially
There are two sublevels:
Size Criteria for Cervical lymph nodes
Calcified Lymphnodes
- infection
- inflammatory
- metastatic papillary thyroid cancer
- adenocarcinoma
- Treated lymphoma
Mnemonic for Lymph Node evaluation
Definition of the oral cavity (OC)
- superior:
- sinonasal region
- separated by:
- soft palate
- posterior:
- Oropharynx (OP)
- separated by
- anterior Tonsilar pillars
- circumvallate papillae
- Distinction important as OC and OP lesions are Rx’d differently
Fig. 4.2
Coronal graphic (a) shows the relationship of the sublingual and the submandibular spaces with the mylohyoid muscle. Also note the neurovascular pedicle (consisting of lingual artery and vein, lingual nerve) within the sublingual space. The hypoglossal nerve courses both in the posterior submandibular and sublingual spaces and runs along the surface of hyoglossus muscle before terminating within the tongue. Coronal MR (b) shows the mylohyoid muscle as a dark curvilinear sling (arrow)-like structure separating the upper sublingual and the inferior submandibular spaces. Also note the paired anterior belly of digastric below the mylohyoid (star)
Floor of Mouth boundaries
- anteriorly
- lingual surface of mandible
- posteriorly
- body of the hyoid bone
- Laterally
- lingual surface of the mandible
- Medially
- Muscles of the tongue
- superiorly
- oral mucosa
- inferiorly:
- mylohyoid which extends from the inner aspect of the lower alveolar ridge to the undersurface of the oral tongue/hyoglossus muscles as well as posterior ot the hyoid bone
- Contents
- Sublingual gland
- Whatons duct
- Sublingual A + N
- Lingual N
- The submandibular duct or Wharton duct or submaxillary duct, is one of the salivary excretory ducts. It is about 5 cm. long, and its wall is much thinner than that of the parotid duct. It drains saliva from each bilateral submandibular gland and sublingual gland to the sublingual caruncle at the base of the tongue.
8 subsites of the Oral mucosal space
which structures make up the root of the tongue?
- lingual septum
- inferior part of the genioglossus
- geniohyoid muscle
Sublingual space
- nonfascial line space superior medial to mylohyoid muscle
- lateral to genioglossus/geniohyoid muscle complex on each side
- posteriorly communicates with the posteriorsuperior part of the submandibular space and inferior parapharyngeal space through the glossomylohyoid gap
- CONTENTS
- sublingual gland
- sublingual duct
- deep ortion of SMG
- Wharton duct
- lingual nerve artery and vein.
- distal CN IX branches
- Distal VN XII branches
Submandibular space location and contents
- space inferolateral to mylohyoid muscle and superior/deep to the platysma below
- Contents
- large superficial portion of the submandibular gland
- Lv 1a and Lv 1b LNs
- facial vein
- facial artery
- anterior belly of digastric muscle
- inferior loop of CN XII
What are the suprahyoid neck spaces?
- carotid
- retropharyngeal
- paravertebral
- pharyngeal mucosal
- parapharyngeal
- masticator
- buccal
- parotid
- sublingual
- submandibular
What are the infra hyoid neck spaces?
6
- extend from the inferior margin of the hyoid to the mediastinum
- carotid
- retropharyngeal
- paravert
- visceral
- posterior cervical
- anterior cervical
Pharyngeal Mucosal Space
- superficial lining and structures of the suprahyoid neck
- mucosa
- minor salivary glands
- lymhatic ring
- constrictor muscles
Parapharyngeal space
Carotid space
AKA
Contents
- AKA
- post-styloid space
- Boundaries
- lower jugular foramen and carotid canal
- to aortic arch
- Contents
- Carotid artery
- IJV
- CN IX, X, XI, XII
- ansa cervicalis,
- the sympathetic plexus, and
- deep cervical lymph nodes
Masticator Space
Buccal space
- fat filled space in the midface
- forms padding of the cheeks
- medial boundary is the buccinator muscle
- lateral boundary is the muscles of facial expression
- posterior:
- masseter
- mandible
- pterygoid muscles
- Parotid
- Contents
- fat
- small nodes
- part of the parotid duct
- +/- accessory parotid
Subdivisions of the Pharynx
- Nasophaynx
- superior most part
- extends to the soft palate
- oropharynx
- palate to superior margin of the hyoid or valleculae
- defined by the gloosoepiglottic and pharyngoepiglottic folds
- Hypopharynx
- laryngeal part of the pharynx
- caudal part of the pharynx
- between the oropharynx and the cervical oesophagus
- includes the piriform sinuses
Laryngeal cartilages
- Thyroid cartilage
- largest laryngeal cartilage
- Cricoid cartilage
- complete ring in the endolarynx
- lower border is the jucntion between larynx and trachea
- Arytenoid cartilage
- paired pyramidal shaped cartilages on top of the posterior part of the cricoid cartilage
- Corniculate cartilage
- small cartilage on top of the superior process of the arytenoid cartilage
What is the innervation to the larynx?
- CN X
- recurrent laryngeal nerve
- motor innervation to all laryngeal muscles
- except the CRICOTHYROID
- Also has a sensory component
- Superior laryngeal nerve
- motor innervation to the cricothyroid muscle
- also some sensory funciton
What are the components of the larynx?
- Laryngeal ventricle (laryngeal sinus)
- fossa between the vestibular and vocal folds
- Supraglottis:
- estends from the tip of the epiglottis superior to the laryngeal ventricle inferiorly
- Three subsites
- epiglottis
- aryepiglottic folds
- projections from tip of arytenoid cartilages to inferolateral margin of the epiglotts that form the superolateral part of the sypraglottis
- divides supraglottis ifrom pyriform sinus of the hypopharynx
- False Vocal cords
- mucosal surfaces of laryngeal vestibule of supraglottis
- Glottis
- consists of the true vocal cord
- aka thyroarytenoid muscle
- overlying the paraglottic spaces
- Subglottis
- part of the larynx extending from under surface of true vocal cords to inferior surface of cricoid cartilage
- the mucosal linding of the subglottic area is closely apposed to cricoid cartilage and should
what is the land mark for the level of true cords on axial Imaging?
what is absent at the true cord level?
Cricoarytenoid joints
there is no paraglottic fat at the true vocal cord level
What is the hallmark for the supraglottic lcoation on Axial CT?
- the presence of Paraglottic fat
What specific things should you report in Oral cavity malignancies?
- lesion depth
- extension to midline
- bone/mandible invasion
- Retromolar trigone
- triangular area of mucosa posterior to the lower and upper third molars,
- covers the anterior surface of the lower mandibular ramus
- cancers at this site are frequently diagnosed at an advanced stage
- are prone to bone involvement
- poorer prognosis
Tumors of the minor salivary glands
- higher chance of being malignant when compared to parotid
- Adenocystic Carcinoma
- high propensity for perineural spread of tumour
- mucoepidermoid ca
- Adenoca (uncommon)
- carcinoma ex pleomorphic (uncommon)
- Adenocystic Carcinoma
where does lymphoma tend to occur in the Oral cavity?
Oropharynx/Waldeyer Ring
Oral cavity lymphomas represent the third most common malignancy in the oral cavity, surpassed by squamous cell carcinoma and malignancies of the salivary glands. Lymphomas in the oral cavity are rare; only 3% of all lymphomas in the general population and 4% on patients with AIDS [25].
Background Plasmablastic lymphoma (PBL) is a rare and aggressive subtype of non-Hodgkin diffuse large B-cell lymphoma originally with a predilection to the oral cavity of patients infected with HIV. H
Oral Cavity dermoid Cyst
Dermoid cysts are cysts filled with sebum like material with evidence of specialised skin derivatives 1. Floor of mouth dermoid cysts account for 1.6% of all dermoid cysts 2 and they usually present as a midline symmetrical slowly enlarging lesion. Cysts superficial to geniohyoid may cause posterosuperior displacement of the tongue, dysphonia, dysphagia 3 or airway obstruction 4. While cysts inferior to geniohyoid can cause submental swelling 3.
- Well circumscribed
- thin walled
- unilocular mass
- Low density on CT
- Low T1, high T2
- Prescence of fat globules/fat density or intensity is diagnostic
- SACK OF MARBLES appearance
- Compound dermoid cyst
- Multipel discernible low attenuation nodules may occasional be seen as a result of coalescence of fat globules within the fludi matrix
Lipoma of the tongue with diffuse lipomatosis
Oral lipomas (OLs) commonly present as a soft, mobile, lobulated, yellow mass. It is usually observed among adults, between the age of 40 and 60 years, with no gender preference. The most common site for OL is the buccal mucosa, a region that is normally rich with adipose tissue, followed by the tongue, lips, floor of the mouth, palate, and gingiva [3].
OL consists of mature adipocytes organized into lobules that are separated by septa of fibrous tissue. Although the OL is morphologically indistinguishable from normal fat, it differs from normal body fat, in that lipids contained in the OL are metabolically inactive [4].
Oral IML seems to be larger in size and displays an infiltrative pattern of growth, as compared with the classic OL. It is characterized by a much deeper localization, slow-growing, painless mass that often can cause swelling and deformity. Its consistency is often rubbery. In the rare occasion, when the infiltration is very extensive, it may cause local muscle dysfunction or sensory disturbance, due to local pressure on a branch of the trigeminal nerve [5].
Oral IMLs are very rare and are described most commonly in the tongue [6, 7, 8].
https://www.hindawi.com/journals/crim/2018/3529208/
Oral cavity pleomorphic adenoma
- Aka benign mixed tumours
- the most common salivary gland tumours.
- The salivary glands are the most common site of pleomorphic adenomas.
- well-circumscribed rounded masses, most commonly located within the parotid gland, hypoechogenic on ultrasound, and bright on T2WI with homogeneous enhancement on MRI.
Epidemiology
Pleomorphic adenomas account for 70-80% of benign salivary gland tumours and are especially common in the parotid gland (see below) 1,6. Patients are typically middle-aged and the incidence is slightly higher in females than males (2:1) 1,13. The oncogenic simian virus (SV40) may play a role in onset or progression and prior head and neck irradiation is a risk factor for the development of these tumours 13,14.
Terminology
Historically pleomorphic adenomas have also been called benign mixed tumours, however this name is now discouraged. It was previously thought that these were a form of teratoma, however it is now clear that the neoplasm forms from a single layer of germ cells and is purely epithelial in nature 15.
Clinical presentation
Patients typically present with a smooth, painless, enlarging mass.
Distribution
Distribution among the salivary glands is as follows 1:
parotid gland: 84% 1
commoner in the superficial lobe
submandibular gland: 8%
minor salivary glands: 6.5%
widely distributed including the nasal cavity, pharynx, larynx, trachea 5
sublingual glands: 0.5%
They are less common in salivary glands other than the parotid but remain the most common benign tumour of each gland.
Pleomorphic adenomas are also commonly found in the lacrimal glands where they account for approximately 50% of lacrimal gland tumours 7.
Pathology
As the name suggests, pleomorphic adenomas are composed of a mixture of variable histology. They contain both epithelial and myoepithelial (mesenchymal) tissues, with mixed histology. They appear encapsulated and well-circumscribed however the pseudocapsule is delicate and incomplete with microscopic extensions reaching beyond it, accounting for the high risk of recurrence when these tumours are enucleated (see below) 5,6.
The gross appearance depends upon the relative proportion of epithelial elements and a stromal component which may range from myxoid to cartilage. Tumours with a prominent cartilaginous matrix have a bluish-grey opalescent appearance.
Three histological types have been described:
myxoid (hypocellular): most common, highest rate of recurrence
cellular
classic
Radiographic features
On all modalities, these tumours typically appear as rounded masses with well-defined, “bosselated” or “polylobulated” borders (many small undulations, not truly lobulated). They are most commonly located within the parotid gland, particularly the superficial lobe.
When they arise from the deep lobe of the parotid they can appear entirely extra parotid, seen in the prestyloid parapharyngeal space, without a fat plane between it and the parotid, and widen the stylomandibular tunnel. Pleomorphic adenomas can also arise from salivary rest cells in the parapharyngeal space itself without connection to the parotid gland.
Ultrasound
They are typically hypoechoic and may show posterior acoustic enhancement.
Ultrasound is also useful in guiding a biopsy (both FNAC and core biopsies) but needs to be carried out with care to avoid facial nerve damage 8,9.
CT
When small, they have homogeneous attenuation and prominent enhancement. When larger, they can be heterogeneous with less prominent enhancement, foci of necrosis, and possible delayed enhancement. Small regions of calcification are common 1,10.
MRI
The signal characteristics are homogeneous when the tumour is small. Larger tumours may be heterogeneous.
T1: usually of low intensity
T2
characteristically of very high intensity (especially myxoid type) 6
often have a rim of decreased signal intensity on T2-weighted images representing the surrounding fibrous capsule
T1 C+ (Gd): usually demonstrates homogeneous enhancement
Angiography (DSA)
typically hypovascular
Nuclear medicine
FDG-PET may show some uptake; SUV more than 3 in 25% of cases 11.
Sialoblastoma
- Rare malignancy of infants
http: //www.ajnr.org/content/cow/10192017/tab-legends
Malignant peripheral nerve sheath tumors (MPNSTs) are sarcomas that originate in peripheral nerves or neurilemma cells. Here, we report an extremely rare case of an intraosseous MPNST in the mandible of a patient with neurofibromatosis type 1 (NF1). A 57-year-old woman with a history of NF1 was referred to our hospital because she had abnormal sensations at her left mandible. She was diagnosed with MPNST and underwent radical resection, although local recurrence and multiple metastases were detected during follow-up. Despite receiving palliative radiotherapy, the patient died at 13 months after the initial diagnosis.
https://www.sciencedirect.com/science/article/pii/S1348864318300077
Aggressive (desmoid type) fibromatosis:
- Heterogenous spectrum of benign and malignant lesions with complex classifications
- Typically described as aggressive but non-metastazing locally invasive tumours with a tendency for recurrence after surgical excision.
- https://www.oralhealthgroup.com/features/desmoid-fibromatosis-of-the-maxillofacial-region-a-case-report-and-review-of-the-literature/
- Desmoid tumor (DT), desmoid fibromatosis (DF) or aggressive fibromatosis, is a rare, benign, but locally aggressive, soft tissue tumor.1
- The most common sites are the abdominal wall, mesentery, and chest wall.2
- Approximately 7-15% of DTs occur in the head and neck with the most common subsite being the supraclavicular fossa.
- It appears, particularly in the head and neck subsites, to be a disease of children and young adults with a median age of 30 years, although it has been reported from newborns to the elderly.3
- In most series it is more common in females than males.2,3
- DTs are a fibrous neoplasm that can arise in connective tissues like skeletal muscle, fascia, and periosteum and although locally destructive, DTs cannot metastasize.4
- They are not encapsulated and have the ability to infiltrate along fascial planes and invade neurovascular structures and bone. This pattern of infiltration and local destruction may mimic malignant entities complicating the diagnostic process.
- Because of its aggressive behavior and locally destructive pattern, it can cause functional impairment and can be life threatening on rare occasions.
- This aggressive behavior also makes complete surgical resection challenging with only 11% of children and 38-55% of adults having clear surgical margins.3
Torus palatinus
Mandibular Osteoma
Ludwig Angina
Ludwig angina is a rapidly-spreading life-threatening cellulitis of the floor of mouth, involving the submandibular, sublingual, and submental spaces.
Most cases (85%) are thought to originate from an untreated odontogenic infection (most commonly the second and third mandibular molars 7), mainly Streptococcus spp., Staphylococcus spp., and Bacteroides spp. 4. Of the other 15% of cases, causes include 4:
Ranula
- rare, benign, acquired, cystic lesions that occur at the floor of the mouth
- sublingual or minor salivary gland retention cysts.
- Epidemiology
- more common in the Maori of New Zealand and
- Pacific Island Polynesians.
- Clinical presentation
- mass either in the floor of the mouth, where they elevate the mucosa, often with a bluish tinge, or in the neck
- Pathology
- Ranulas arise either spontaneously or as a result of trauma to the floor of mouth, including surgery.
- They result from obstruction of a sublingual gland or adjacent minor salivary gland with resultant formation of a mucous retention cyst
- Classification:
- A ranula can be classified based on its extent:
- simple ranula:
- confined to the sublingual space
- plunging ranula
- AKA diving ranula, cervical ranula):
- extends into the submandibular space
- as a simple ranula enlarges it dissects along fascial planes beyond the confines of the sublingual space, either:
- around the posterior edge of the mylohyoid muscle, or
- directly through a deficiency of the mylohyoid muscle (mylohyoid boutonniere)
- simple ranula:
- A ranula can be classified based on its extent:
The key to diagnosing a ranula, especially in cases where they are large and have dissected some distance away from their origin, is the identification of a connection to the sublingual space.
This may be no more than a thin tail of fluid or a significant local fluid collection 1.
Simple ranula is within the sublingual space above the mylohyoid muscle but plunging ranula dives into the submandibular space with a collapsed sublingual portion called the “tail”.
-
Differential diagnosis
- dermoid/epidermoid cyst
- cystic hygroma
- usually in infants
- cervical abscess
- extensive adjacent fat stranding
- usually associated with tooth caries
- thyroglossal duct cyst
- usually midline
- 2nd branchial cleft cyst
- usually at the anterior border of the sternocleidomastoid muscle
- cystic/necrotic lymph node
oral cavity Hemangioma
oral cavity Hemangioma
MRI of the head and neck shows a lobulated mass involving the anterior part of the oral tongue. The mass is hyperintense T2 and hypointense T1. Small foci of hypointense T2 signal are seen, compatible with flow voids. Although an atypical location, the findings are characteristic of haemangioma.
Case Discussion
Haemangiomas are the most common vascular malformation in the head and neck.
Infantile Haemangioma of the oral cavity
Tumour that appears early in infancy, rapidly enlargs and then tends to spontaneously involue.
Glucose transporter 1 (GLUT1) immunohistochemical postive.
Lingual artery aneursym
Thyroglossal duct cysts
Thyroglossal duct cysts (TGDC)
- Intro
- most common type of congenital neck cysts and paediatric neck masses
- typically located in the midline
- most common midline neck mass in young patients.
- typically present during childhood (90% before the age of 10 years)
- gradually growing painless fluctuant cervical mass
- remain asymptomatic until they become infected, in which case they can present at any time.
- Thyroglossal duct cysts account for 70% of all congenital neck anomalies
- second most common benign neck mass after lymphadenopathy.
-
Associations
- ectopic thyroid: ~40%
-
Path
- Thyroglossal duct cysts are epithelial-lined cysts.
- They result from failure of normal developmental obliteration of the thyroglossal duct during development (8th - 10th gestational week
- can thus occur anywhere along the course of the duct.
-
Location
- The cysts can occur anywhere along the course of the thyroglossal duct:
- from the foramen caecum to the
- thyroid gland
- infrahyoid location is most common:
- suprahyoid: 20-25% (less common in adults ~5%)
- at the level of hyoid bone: ~30% (range 15-50%)
- infrahyoid: ~45% (range 25-65%)
- Typically, they are located in the midline (~70%)
- those off-midline characteristically adjacent to the thyroid cartilage.
- Almost all thyroglossal duct cysts are located within 2 cm of the midline, with more inferior lesions tending to be off midline.
- The cysts can occur anywhere along the course of the thyroglossal duct:
-
CT
- thin-walled, smooth, well-defined homogeneously fluid-density lesions with an anterior midline or paramedian location.
- they should be within 2 cm of the midline.
- They may demonstrate slight rim (capsular) enhancement.
- SCM typically displaced posteriorly or posterolaterally.
- may be embedded in the infrahyoid (strap) muscles.
-
Complications
- infection
- malignancies do occur but are rare
- seen in <1% of cysts
- when they do occur they are most frequently papillary thyroid carcinoma
- presence of calcification is suggestive of malignancy
- intracystic soft tissue is less specific
-
Differential diagnosis of midline neck masses:
- branchial cleft cyst:
- three times less common, and
- usually well away from the midline
- Delphian adenopathy
- epidermoid cyst:
- superficial to the strap muscles
- thyroid cyst or thyroid neoplasm
- laryngocele
- ranula
- parathyroid adenoma
- ectopic thyroid
- branchial cleft cyst:
Lingual Thyroid
No thyroid tissue is seen at the normal thyroid bed anterior to the larynx and upper trachea.
Instead, a rounded, well defined, homogeneously enhancing ectopic thyroid tissue is seen at the base of the tongue.
Case Discussion
Ectopic thyroid gland is a congenital anomaly with female predilection. Lingual thyroid makes up approximately 90% of ectopic thyroid glands.
Pathology
A lingual thyroid results from failure of the normal caudal migration of the thyroid from the foramen caecum down to its normal location anterior to the larynx and upper trachea. Thyroid tissue may be found anywhere along the course of the thyroglossal duct, however, complete arrest with thyroid tissue located at the base of the tongue is most common and represents 90% of all cases of ectopic thyroid 1,2. Microscopic deposits of thyroid tissue along its route of descent have been identified in up to 10% of the population, representing small amounts of tissue being ‘left behind’ during normal development 2.
The thyroid tissue is normal histologically and functionally.
FIG 4. A, Axial T1-weighted postcontrast MR image of the nasopharynx in a 53-year-old man with BH1 (open arrows). An area of diffuse symmetric mucosal thickening with homogeneous contrast enhancement is visible. B, Axial T1-weighted postcontrast MR image of the nasopharynx in a 28-year-old man with BH2 of the adenoid (open arrow). The symmetric lesion exhibits contrast-enhancing septa that run perpendicular to the nasopharyngeal wall and are separated by columns of low contrast enhancement. C, Axial T1-weighted postcontrast MR image of the nasopharynx in a 48-year-old woman with BH2 along the nasopharyngeal walls (open arrows). An area of diffuse, symmetric homogeneous low contrast enhancement and an intact deep mucosal white line along the deep margin are visible
Tornwaldt Cyst
- Midline cyst of notochordal remnant
- relatively common
- incidental
- nonenhancing cyst at midline
- may have high signal on T1 if proteinaceous
- no internal/solid enhancement
Where does NPC typically start?
What is NPC strongly associated with?
- Mucosal tumour typically starting in the lateral pharyngeal recess
- AKA
- Fossa of Rosenmuller
- Strongly Associated with EBV
Axial T1-weighted postcontrast MR imaging of a 48-year-old man with NPC (arrow). A, Note a small moderately contrast-enhancing NPC in the right pharyngeal recess on MR imaging at presentation (grade 4), which was not detected by endoscopy or endoscopic biopsy or at repeat biopsy targeted to the site of the MR imaging abnormality. B, Persistent NPC on MR imaging is seen at 31 months, but without a tumor on endoscopic examination. A further biopsy was declined. C, An increase in the size of the NPC on MR imaging at 43 months when the tumor was confirmed by endoscopy and biopsy.
In which CT/MRIs should you check for incidental NPC?
- Older patients with serous otitis medial on clinical examation or on CT/MRI
- check nasopharynx of obstructive mass
Nasopharyngeal Carcinoma with Eustachian Tube Extension
Chapter 53
Nasopharyngeal Carcinoma with Eustachian Tube Extension
Epidemiology
Nasopharyngeal carcinoma (NPC) usually originates in the lateral pharyngeal recess (fossa of Rosenmüller). The tumor can easily invade the opening of the eustachian tube, which is located immediately anterior and inferior to the fossa of Rosenmüller. Patients in the high-risk group who present with tinnitus or serous otitis media should be carefully evaluated. Risk factors include several factors: age (> 30 years), sex (male > female), ethnic group (especially ethnic Chinese), and a family history of NPC in a first-degree relative.
Clinical Findings
The most common ear symptom is unilateral hearing loss. This symptom is related to tumor obstructing the eustachian tube resulting in serous otitis media. Eustachian tube dysfunction can also be caused by tumor infiltration of the muscles of deglutination. In the absence of any infection or allergic nasal symptoms, a nasopharyngeal biopsy is indicated in all high-risk patients with unilateral serous otitis media. Epstein-Barr virus (EBV) serology may provide further useful information, especially in patients with a clinically occult primary.
waldeyer ring lymphoma
waldeyer ring lymphoma
- AKA nasopharyngeal lymphoma
- extranodal lymphoma
- suspect in patients with known lymphoma
- evaluate for cervical LAD (present in 50%)
- evaluate for involvement of other tonsils
- palatine tonsils
- nasopharyngeal adenoids
- lingual tonsils
- Bulky homogenous mass
- may have intermedial to low sig on T2
- May not be distinguishable from other malignancies on imaging
NHL involving the Waldeyer ring. Contrastenhanced axial CT section showing circumferential soft tissue thickening of the nasopharynx (arrows). This proved to be extranodal NHL of Waldeyer ring.
https://www.researchgate.net/figure/NHL-involving-the-Waldeyer-ring-Contrastenhanced-axial-CT-section-showing_fig1_41721748
Nasopharyngeal inflammatory pseudotumor (IPT)
- appears as homogeneously enhancing nasopharyngeal soft tissue on contrast-enhanced CT examination.
- Associated destruction of the skull base (Figure 1) and narrowing of the neighboring skull base foramina/canals may occur.
- There is usually no enlargement of the cervical lymph nodes.
- On MRI, IPTs are typically ill-defined, infiltrative masses (Figure 2) with moderate, homogeneous contrast enhancement.
- There is involvement of the submucosa of the nasopharynx with preservation of normal hyperintense signal of the nasopharyngeal mucosa.
- Contiguous structures that may be involved include the clivus, eustachian tube, carotid and parapharyngeal spaces, Meckel cave, orbital apex, and cavernous sinus.
- Encasement of the internal carotid artery may occur. Ipsilateral opacification of the mastoid air cells is a common finding due to eustachian tube obstruction.
- Differential diagnosis
- includes nasopharyngeal carcinoma (NPC),
- chordoma,
- chondrosarcoma,
- skull base lymphoma,
- plasmacytoma, and
- metastasis.
- Rosai-Dorfman disease,
- Epstein-Barr virus-related inflammatory pseudotumor, calcifying fibrous pseudotumor, and
- sclerosing lymphoma are less likely considerations.
DISCUSSION
Inflammatory pseudotumor (IPT) of the nasopharynx is a benign, idiopathic disease usually mistaken for a neoplasm/infection owing to its aggressive appearance1,2,3 Patients routinely present with recent history of otitis media, hearing loss, and/or single or multiple cranial nerve neuropathies.2
IPT is an inflammatory lesion of unknown etiology. Various theories, including autoimmune reaction linked to viral infection, infectious process due to sinusitis, or syphilis and aberrant production of fibrogenic cytokines, have been proposed.1 However, recent studies have suggested cytogenetic clonal abnormality and anaplastic lymphoma kinase expression, suspicious for a neoplastic cause.4
On gross pathology, IPTs resemble inflamed fibrous tissue with contiguous reactive bone. Routine microscopy demonstrates mixed acute/chronic inflammatory cells and spindle cells with a densely fibrotic background.5,6 (Figure 3).
On MRI, IPT is typically seen as a mass which is T2 hypointense compared to brain parenchyma (Figure 2), while NPC (the most common differential diagnostic consideration) is more hyperintense on T2 imaging.1,4 Internal carotid artery encasement and narrowing of the vessel lumen are common features with IPT.1 In comparison to NPC, IPT involves the submucosal layer of the nasopharynx. The hyperintense mucosal layer is well-delineated from the hypointense submucosal lesion on T2 imaging (Figure 2). Extensive pachymeningeal thickening and enhancement is a more common feature of IPT but not of NPC. Cervical lymphadenopathy is more commonly encountered in patients with NPC than with IPT.1,2,6 Systemic steroids are the treatment of choice in patients with IPT involving the nasopharynx and skull base.7
Juvenile nasopharyngeal angiofibroma
This is a classical presentation of juvenile nasopharyngeal angiofibroma. This is a histologically benign lesion with very aggressive local behaviour.
It is almost exclusively encountered in adolescent males 1. The most common presenting symptom is painless nasal obstruction or epistaxis; however, other symptoms may develop depending on the size and extent of the tumour mass.
The lesion tends to spread via the pterygopalatine fossa and osseous destruction is common. Orbital and intracranial extension can be seen at presentation which complicates treatment.
These lesions are highly vascular and biopsy is contraindicated due to the risk of haemorrhage. CT or MRI have important roles in assessing the tumour mass and extension and assist with treatment planning. DSA elegantly demonstrates the vascular supply and allows pre-operative embolisation of the feeder vessels. Post-embolisation surgical resection is the treatment of choice 2.
Q: Would a biopsy be helpful in this case? hide answer
A: No! This is a case of juvenile nasopharyngeal angiofibroma with classical clinical and imaging findings. These highly vascular tumors are prone to significant haemorrhage and biopsy is contraindicated due to risk of exsanguination.
- Acute bacterial Tonsilitis
- presents as acute pharyngitis
- inflammatory changes presence or absence of rim enhancing low collection helps differentiate inflammation alone with phlegmon from abscess (may be treated surgically)
- There can be spread of inflammation and edema to adjacent spaces including retropharyngeal space and danger space in severe cases
Oropharyngeal SCC
Cause?
which type of OPSCC has a better prognosis?
-
Oropharyngeal SCC
- OPSCC
- Oropharyngeal squamous cell carcinoma, commonly known as throat cancer or tonsil cancer, is a type of head and neck cancer that refers to the
- cancer of the base and posterior one-third of the tongue, the tonsils, soft palate, and posterior and lateral pharyngeal walls.
- aeitiology
- majority HPV +ve in the US/Aust
- HPV negative a/w smoking/tobacco/etoh (less common form)
- majority are HPV 16+
- HPV +ve has a better prognosis
Typical findings on imaging of OPSCC?
is there a difference in appearance of primary tumour in HPV positive vs negative?
- enhancing mass at different oropharyngeal subsites
- there is no difference in appearance of primary tumour in HPV positive vs negative
- extension to adjacent sites and outside of the oropharynx important for staging and needs to be determined thoroughly
- Primary tumour may also be occult/not seen on Ct/MRI
- PET/CT may be helpful for identification of unknown primary site
- but normal tonsillar tissue is FDG avid and small primaries may also be difficult to detect on PET. Look for assymmetric uptake
- HPV +VE nodes are frequently cystic and therefor potential for false negative on PET
oropharyngeal Diffuse large B cell lymphoma
Huge symptomatic oropharyngeal mass with airway compromise and advanced nodal involvement.
An fibroscopic biopsy was performed.
Oropharyngeal biopsy: Diffuse large B cell lymphoma (CD20,CD79a,BCl 6 positive, CD5,CD10,CD56 and cyclin D1 negative)
Another example of an extreme head and neck space pathology. It continues to amaze me how late many patients present with pathology.
Case courtesy of Dr Ian Bickle, Radiopaedia.org, rID: 59187
Postradiation mucositis
Postradiation mucositis
- history of recent radiation therapy
- characteristic postradiation changes
- soft tissue edema, nonmass like enhancement
- Diffuse mucosal enhancment may help in differentiating mucositis from recurrent neoplasm
- focal
- irregular
- nodular
- masslike enhancment
- enhancment deep to the mucosa
- Beningn pleomorphic adenoma of minor salivary gland of palate
- AKA benign mixed tumour
- Pleomorphic adenoma can be defined as a benign mixed tumor composed of epithelial and myoepithelial cells arranged with various morphological patterns, demarcated from surrounding tissues by fibrous capsule.
- It is one of the salivary gland tumors affecting both major and minor salivary glands.
- Parotid gland is the most commonly affected of the major group, and palate is the most common site intraorally.
- We report a case of palatal pleomorphic adenoma in a 35 year-old adult from South India
- Vallecular cyst
- unilocular benign cystic lesion. located in vallecular
- no invasive features or solidy enhancing component.
- Laryngeal cysts are rare laryngeal lesions. It is estimated that 10.5% of laryngeal cysts occur in the vallecular space. Vallecular cysts (VC) have been reported in literature under other names, such as mucus retention cyst, pre‐epiglottic cyst, epiglottic cyst, base of the tongue cyst, and ductal cyst. This is probably due to the confusion surrounding the pathogenesis and aetiology of this lesion. Adult patients with a VC generally have a benign course and commonly present with symptoms of hoarseness, foreign body sensation and dysphagia. This is 30 year old male patient with hoarseness.
what is the foramen caecum?
what pathology does it relate to?
Thyroglossal duct cysts (TGDC) are the most common type of congenital neck cysts and paediatric neck masses. They are typically located in the midline and are the most common midline neck massin young patients. They can be diagnosed with multiple imaging modalities including ultrasound, CT and MRI.
Epidemiology
Thyroglossal duct cysts typically present during childhood (90% before the age of 10 years) as a gradually growing painless fluctuant cervical mass or remain asymptomatic until they become infected, in which case they can present at any time.
Thyroglossal duct cysts account for 70% of all congenital neck anomalies, and are the second most common benign neck mass after lymphadenopathy.
Associations
ectopic thyroid: ~40%
Clinical presentation
The presentation is typically either as a painless rounded midline anterior neck swelling or if infected, as a red warm painful lump. It may move with swallowing and classically elevates on tongue protrusion.
Pathology
Thyroglossal duct cysts are epithelial-lined cysts. They result from failure of normal developmental obliteration of the thyroglossal duct during development (8th - 10th gestational week) and can thus occur anywhere along the course of the duct.
The epithelial lining of the cyst varies with location. Those that form near the tongue are lined by stratified squamous epithelium. The more common cysts located in the neck are lined with cells similar to thyroidal acinar epithelium.
Three main sites of Hypopharyngeal SCC?
prognosis?
% of all proximal aerodigestive tract malignancies?
- Pyriform sinus 75%
- posterior wall
- postcricoid region
Squamous cell carcinoma (SCC) of the hypopharynx is relatively uncommon, carries the worst prognosis of any head and neck squamous cell carcinoma (HNSCC), and is a challenge to diagnose and treat.
Hypopharyngeal carcinoma is relatively uncommon representing only 10% of all proximal aerodigestive tract malignancies.
Squamous cell carcinomas account for ~95% of all primary tumours of the hypopharynx.
How are laryngeal SCC’s characterised?
- Supraglottic
- Glottic
- Subglottic
- Transglottic
- if tumour crosses these sites
- Laryngeal tumors may extend into the hypopharynx or vice versa
- Determine the centre of the mass to help with correct lesion localisation as there are differences in staging classification according to the site of origin.
- T2 hyper-intense enhancing mass involves the right aryepiglottic fold, vocal cord and paraglottic fat
- Likely involvement of right thyroid cartilage. There is no abnormal signal/enhancement in the tissue overlying the thyroid lamina to suggest cartilage invasion.
- Abnormal enhancement at anterior commissure and left cord suggests contra-lateral involvement. There is evidence of subglottic extension to the level of the inferior border of cricoid.
- No post cricoid or hypopharynx posterior wall involvement.
- Jugular chain lymph nodes are increased in number but not enlarged.
