Head and Neck 4 Flashcards
Syndromes associated with Paragangliomas
- NF1
- MEN 2a + 2b
- vHL
- Carney-Stratakis
- Paragangliomas are the most strongly hereditary group of tumours. The most common genetic cause of hereditary paragangliomas are mutations in the succinate dehydrogenase (SDH) subunit (SDHB, SDHD, SDHA or SDHAF2) 2.
- They are also associated with four clinical syndromes:
- von Hippel-Lindau syndrome
- multiple endocrine neoplasia types 2A and 2B
- neurofibromatosis type 1
- Carney-Stratakis syndrome
- von Hippel-Lindau syndrome and neurofibromatosis type 1 are more commonly associated with phaeochromocytomas.
- SDH mutations are common in head and neck paragangliomas,
- except for SDHB, which is associated with sympathetic paragangliomas.
- SDHB also confers a higher risk of malignancy 2.
What is this disease?
- Graves disease
- is an autoimmune thyroid disease
- most common cause of thyrotoxicosis (up to 85%).
- There is a strong female predilection with an F:M ratio of at least 5:1. It typically presents in middle age.
- Patients are thyrotoxic. Extrathyroidal manifestations include:
- thyroid dermopathy (formerly called pretibial myxoedema): occurs in ~2% and almost always associated with thyroid ophthalmopathy
- thyroid acropachy: occurs in ~1% 7
- Graves ophthalmopathy (orbitopathy): affects 20-25% of cases
- encephalopathy associated with autoimmune thyroid disease (EAATD) 2,8
- much more commonly associated with Hashimoto thyroiditis
- The combination of exophthalmos, palpitations, and goitre is called the Merseburger (or Merseburg) triad
Retropharyngeal abscess
Causes
Organisms
Imaging features of Mycetoma
- Single sinus
- usually maxillary
- no internal enhancement, surrounding mucosal enhancement
- internal high denisty/calcifcation
- +/- chronic mucoperiosteal change and hyperostosis
Mucoperiosteal reaction of both maxillary, ethmoidal and left frontal sinuses with obliteration of both osteomeatal complexes. It exhibits variable signal intensity; on the left side the mucoperiosteal reaction displays low T1 bright T2 signal with marginal enhancement. On the right side there is low T1 yet loss of signal on T2 owing to paramagnetic effect of iron and manganese particles in fungal hyphae.
Case Discussion
T2 WI is helpful in fungal sinusitis as the presence of iron and manganese particles in mycetoma causes signal loss on T2 WI due to paramagnetic effect.
what size does duct obstruction occur at?
most common site of sialolithiasis?
risk factors 5?
- Sialolithiasis (Calculi)
- Calculi form as a result of deposition of calcium carbonate or calcium phosphate around an initial organic matrix consisting of glycoproteins, muccopolysaccharides, cellular debris, and possibly bacteria and other foreign substances such as food.
- Can be multiple, total duct obstruction is usually due to calculi >3 mm.
- Location:
- SMG, 80%
- Proposed factors accounting for the higher incidence of submandibular calculi are
- (1) more alkaline pH of SMG, which tends to precipitate salts;
- (2) thicker, more mucous submandibular saliva;
- (3) higher concentration of hydroxyapatite and phosphatase;
- (4) narrower Wharton orifice compared with main lumen; and
- (5) slight uphill course of salivary flow in Wharton duct when patient is in upright position.
- Proposed factors accounting for the higher incidence of submandibular calculi are
- Parotid, 20%
- rarely sublingual glands
- SMG, 80%
, the right arytenoid projects into the subglottic area (arrow). The right vocal cord is foreshortened and inferomedially rotated (*)
what innervates the vocal cord muscles?
Which nere is more frequently injured?
CT’s role in setting of laryngeal Trauma
- displaced fractures of the thyroid or ricoid cartilage
- arytenoid dislcoation
- a false passage
- displacement/injury of the epiglottis
Causes of Vocal cord paralysis
What are 8 different types of benign thyroid neoplasms?
- Hurthle cell
* Subset of follicular lesions
* composed of oval to polygonal cells with dense granular acidophilic cytoplasm and prominent macronucleolus
- Hurthle cell
- 2 Hyperplastic nodule
- 3 Colloid cysts/adenomas (27-60%)
- 4 Follicular Adenoma (26-40%)
- Encapsulated
- usually monoclonal
- 5 Hashimoto’s thyroiditis
- focal
- diffuse
- 4-5% of nodules
- 6 Fetal
- 7 Embryonal
- 8 Papillary adenoma
- does not even exist and any papillary architecture should be considered as papillary carcinoma and treated as such.
https://oncohemakey.com/differential-diagnosis-of-thyroid-nodules/
what is an onodi cell?
- posterior ehtmoid cell that surround the optic cancal and may border the carotid canal
- suspect if horizontal septation in sphenoid sinus in the foronal plare
- must be recofnised preoperatively to avodi inadvertent injury to the optic nerve
definition
causes
most common cause
- Sialosis
- Recurrent or chronic nonneoplastic, noninflammatory, nontender, enlargement of the parotid glands
- Usually bilateral and symmetric but can be asymmetric or unilateral
- Associations
- Endocrine diseases
- diabetes/pancreas
- abnormalities of ovaries
- thyroid glands
- acromegaly
- Nutritional states
- chronic alcoholism and
- alcoholic cirrhosis
- malnutrition states
- Endocrine diseases
- Medications
- Other conditions
NUC MED
What are the Nuclear medicine Studies for Graves disease?
- Nuclear medicine
- iodine-123: imaging performed at around 2-6 days; classically demonstrates homogeneously increased activity in an enlarged gland
- technetium-99m pertechnetate: homogeneously increased activity in an enlarged thyroid gland
- Scintigraphy
- Uniform distribution of increased activity by scintigraphy
- (Hashimoto thyroiditis can mimic this appearance, but patients are usually euthyroid)
- Elevated 131 I uptake: 50%–80%
Malignant Sinus Tumours
DDx list
11
Malignant sinus Tumors
- Most common:
- SCC, 80%
- Less common tumors:
- ACC
- Esthesioneuroblastoma
- Lymphoma
- Sinonasal undifferentiated CA
- Mucoepidermoid CA
- Mesenchymal tumors:
- fibrosarcoma,
- rhabdomyosarcoma,
- osteosarcoma,
- chondrosarcoma
- Metastases from lung, kidney, breast
Spinnaker sail sign
- the spinnaker sail sign is the primary finding of vocal cord paralysis
- Paramedian position of the affected VC
- ballooning of the ipsilateral laryngeal ventricle
- anteromedial rotation of the arytenoid cartilage
- medially displaced and thickened aryepiglottic foldenlarged ipsilateral pyriform sinus
what is this?
what is the usual bug?
who does it happen to?
symptoms?
- Mycetoma
- saprophytic fungal frowth in the sinus
- usually aspergillus fumigatus
- chronic noninvasive form of fungal sinus infection
- immunocompetent, non atopic, healthy patient
- mycetoma and allergic funal sinusistis are the most common forms of fungal sinusitis
- asymptomatic or mild pressure sensation of the sinuses
- can mimic chronic sinusitis
vocal cord polyp
- not true tumours
- most common benign lesions of the larynx
- represents a stromal reaction in response to vocal abuse
What is a glomus tumour??
benign or malignant
which cell type does it arise from?
vascular/not so vascular?
Characteristic sign
Associations
- AKA
- Glomus tumour/paraganglioma
- benign tumour
- Paragangliomas arise from neural crest cells, which can differentiate into cells of either the parasympathetic or sympathetic nervous system.
- In the head and neck, paragangliomas tend to be innervated by the parasympathetic system and do not secrete catecholamines and are thus termed nonchromaffin paragangliomas 10.
- ++ vascular, ++ enhancement
- Larger lesions have internal flow voids and a salt and pepper appearance
- Associations
- MEN 2
- VHL
- NF1
What is this?
?% of Laryngeal SCC?
Which structures does it arise from?
% with nodal mets at presentation?
Why?
Which structures need to be carefully evaluated?
Supra glottic SCC
- 30% of laryngeal scc
- tumour arises from
- false cords
- ventricles
- the laryngeal surface of epiglottis and
- aryepiglottic folds
- The supraglottis is vascular and rich in lymphatics
- 35% have nodal mets at presentation
- Evaluate extension to adjacent spaces
- Thyroid cartilage invasion
- Inner cortex only T3
- though and through invasion (T4a)
- pre-epiglottic and paraglottic space involvement
- T3
- Extralaryngeal extention
- T4
- Thyroid cartilage invasion
5 DDx of Salivary gland cysts
-
Cystic Salivary Lesions
-
1. Mucous retention cyst:
- true cyst with epithelial lining
-
2. Mucocele
- extravasation cyst:
- results from ductal rupture and mucus extravasation.
- Not a true cyst;
- pseudocyst composed of fibrous and granulation tissue
-
3. Ranula:
- retention cyst
- simple
- or extravasation cyst
- diving
- from sublingual glands in floor of mouth or beyond (diving)
- retention cyst
-
4. Sialocele:
- focal collection of saliva secondary to leak from ductal system from previous obstruction or inflammation;
- may not be distinguishable from first branchial cleft cyst on imaging (aspiration for distinction)
-
5. Benign lymphoepithelial cysts
- BLCs
- HIV-positive patients (early),
- a precursor to autoimmune deficiency syndrome (AIDS)
- Associated adenopathy and lymphoid (tonsils, adenoids) hyperplasia may be clues to HIV seropositivity
- Typically present as bilateral parotid cysts, superficial in location, in LNs
- May not be distinguishable from Sjögren disease (lesions are parenchymal)
-
1. Mucous retention cyst:
what is this condition?
AKA
what are the serum levels?
- Pseudohypoparathyroidism (PHP) is a condition where there is end-organ resistance to parathyroid hormone (PTH).
- Epidemiology
- Pseudohypoparathyroidism has an estimated prevalence of 1.1 per 100,000 people 6.
- Clinical Presentation
- Hypocalcaemia and tetany
- Short stature
- Developmental delay
- Pathology
- Subtypes
- There are several recognised subtypes which include:
- type I: abnormal cAMP response to PTH stimulation
- type Ia (Albright hereditary osteodystrophy (AHO)): has characteristic phenotypical features
- type Ib: lacks phenotypical features
- type II: normal cAMP response to PTH stimulation
- type I: abnormal cAMP response to PTH stimulation
- There are several recognised subtypes which include:
- Markers
- parathyroid hormone level: high
- serum phosphate level: high
- serum calcium level: low
- Radiographic features
- Musculoskeletal manifestations
- short stature and obesity
- brachydactyly
- short metacarpals (inclusive of short 4th/5th metacarpals)
- short metatarsals
- soft tissue calcification
- exostoses: short metaphyseal or more central and perpendicular to long axis of a bone
- broad bones with coned epiphyses
- CNS / head and neck manifestations
- basal ganglia calcification
- sclerochoroidal calcification 4
- deep white matter calcification
- Musculoskeletal manifestations
Where can Extralaryngeal spread occur?
- through the cricothryroid ligament,
- thryoarytenoid space
- route of spread between paraglottic space and pyriform sinus apex
- or invasion of the Thyroid Cartilage
Clinical findings of Graves disease
- Graves disease consists of one or more of the following:
- Thyrotoxicosis
- Goiter
- Ophthalmopathy
- Dermopathy: pretibial myxedema: accumulation of glycosaminoglycan in pretibial skin
- Rare findings:
- Subperiosteal bone formation (osteopathy of phalanges)
- Clubbing (thyroid acropathy)
- Onycholysis = separation of the nail from its bed
- Gynecomastia in males
- Splenomegaly, 10%
- Lymphadenopathy
Different types of neck dissection
- Selective neck dissection
- resection of knonw or potential nodal lvs with preservation of functional nonlymphatic structures
- Modified (radical) neck dissection
- resecrtion of all nodes in levels I-V, with preservation of one or more of the following nonlymphatic structures
- Ipsilateral IJV
- Spinal accessory nerve
- SCM
- SMG
- resecrtion of all nodes in levels I-V, with preservation of one or more of the following nonlymphatic structures
- Radical Neck Dissection
- en bloc resection of ipsilateral nodes from mandible to the clavicle (levels I-V)
- SMG
- Spinal Accessory nerve
- IJV
- SCM
- en bloc resection of ipsilateral nodes from mandible to the clavicle (levels I-V)
- Extended radiacl neck dissection
- radiacal neck dissection
- removal of additional lymphatic and nonlymphatic structures
- retropharyngeal node
- Carotid artery etc
- Myocutaneous flaps used for repair.
Where does the frontal sinus drain to?
- frontal sinus
- frontal-ethmoidal recess
- middle meatus
- (joins flow from ipsilateral maxillary sinus)
PATHOLOGY SPECIMEN
-
Follicular thyroid adenoma
- commonly found benign neoplasm of the thyroid consisting of differentiated follicular cells.
- It cannot be differentiated from follicular carcinoma on cytologic, sonographic or clinical features alone.
- Epidemiology
- Follicular thyroid adenoma is more commonly found in women,
- increases in incidence with
- increasing age
- in regions in which the diet is iodine deficient.
- 5 times more frequent than follicular carcinomas.
- Pathology
- Macroscopically
- round to oval,
- fibrous capsule that is usually regular and thin.
- between 1 and 3 cm,
- cystic degeneration,
- haemorrhage,
ossification, - calcification and
- fibrosis
- Macroscopically
- Cytology
- Functioning follicular adenomas occur as a result of a monoclonal expansion of thyroid follicular cells with a high prevalence of activating mutations in the gene for the TSH receptor 1.
- Genetics
- N-RAS and K-RAS mutations may be present in patients with follicular adenoma and have been implicated in the evolution of follicular adenoma to follicular carcinoma 4.
- Subtypes
- macrofollicular thyroid adenoma
- microfollicular thyroid adenoma
- Radiographic features
- Ultrasound
- Ultrasound features of follicular adenomas share many features with follicular carcinomas. In general follicular thyroid adenomas:
- thin peripheral halo
- predominantly cystic or mixed cystic and solid lesions
- isoechoic or predominantly anechoic
- can be homogenous or heterogeneous
- absence of internal flow or predominantly peripheral flow indicates is associated with reduced probability of thyroid follicular malignancy
- Ultrasound features of follicular adenomas share many features with follicular carcinomas. In general follicular thyroid adenomas:
- Ultrasound
Laryngeal amyloidosis
Intraoperative photo demonstrating left well-defined submucosal lesion arising from the left laryngeal ventricle, clear of vocal fold and anterior commissure.
Case Discussion
The patient underwent cold steel and shaver excision of the laryngeal lesion under general anaesthesia.
Histology: numerous deposits of homogenous eosinophilic material which stained salmon pink on congo red and showed apple-green birefringence, confirming amyloidosis.
Case discussion
Amyloidosis is very rare and represents a heterogeneous group of disorders marked by abnormal protein formation and deposition which can cause organ failure and death. Up to 20% involve the head and neck, the larynx being the most common site of disease, representing 0.2-1.2% of all benign laryngeal tumours. Symptoms depend on the size and specific location of the deposit and may include dysphonia, dysphagia or exertional dyspnoea.
Mucosal Head and Neck cancers Nodal staging
AJCC
NPC and Non-NPC staging.
- distribution and impact of nodal spread from NPC is different from other mucosal head and neck cancers and therefore a different N classificaiton is used.
- American Joint Committee on Cancer system applies for epithelial cancers (usually SCC) of most regions of the head and neck, including:
- those of the skin,
- maxillary sinus,
- nasal cavity and
- ethmoid sinus,
- oral cavity,
- oropharynx,
- major salivary glands,
- hypopharynx,
- larynx, and
- unknown primary.
-
Notable exclusions of head and neck cancers:
- are HPV-related oropharynx cancer,
- nasopharynx cancer,
- mucosal melanoma, and
- thyroid cancers
- differentiated,
- medullary, or
- anaplastic
-
NASOPHARYNGEAL CANCER (NPC)
- N1:
- unilateral nodal mets
- above supraclav fossa <6cm
- +/- unilateral or bilateral retropharyngeal nodes <6cm
- N2:
- bilateral nodal mets above supraclav fossa <6cm
- N3a
- nodal mets >6cm
- N3b
- nodal mets extension to supraclav fossa
- N1:
- OTHER SITES (Oral cancer, oropharynx, hypopharynx, larynx):
- N1:
- met in a single ipsilateral LN <3cm
- N2
- met in a single ipsilat LN >3 but <6cm
- or in multiple ipsilateral LNs <6cm
- or in bilateral or contralateral LNs <6
- N2a
- mets in a single ipsi LN >3 but <6cm
- N2b
- mets in multiple ipsi LNS <6
- N2c
- Mets in bila or contrala LN <6
- N3
- mets in a LN >6cm
- N0: no regional LN mets
- Nx: LN status cannot be assessed.
- N1:
- Node size in classificaiton refers to greastest dimension.
Features favoring benign thyroid nodules:
- Features favoring benign nodules:
-
Purely cystic or spongiform nodules
- have a very low risk for malignancy
-
Increased echogenicity
- of solid component relative to thyroid parenchyma favors benign nodules
-
Smooth
- uninterrupted, well-defined, and curvilinear edge
-
Halo
- dark rim around nodule periphery
- A uniform complete halo has been suggested to favor a benign nodule, but a complete or incomplete halo may nonetheless be seen in 10%–24% of thyroid CAs.
-
Comet-tail artifacts
- are seen in colloid cysts.
-
Macrocalcifications
- (large enough to result in posterior acoustic shadowing)
- Depending on pattern may favor benign but can also have an association with malignancy
- (large enough to result in posterior acoustic shadowing)
- “Eggshell” calcification favors benign nodule
-
Purely cystic or spongiform nodules
- Antrochoanal Polyp
- Most common type of solitary sinonasal polyp
- polypoid mass extending from maxillary sinus to nasal cavity via an enlarged maxillary ostium or accessory ostium
- Epid
- Most commonly seen in teenagers and young adults
- Radiographic Features
- Well-defined dumbbell-shaped mass extending from maxillary sinus into nasal cavity
- Usually low/mucoid central density (may have higher density depending on inspissated component and fungal colonization)
- Bone remodeling rather than destroyed
- Peripheral enhancement of surrounding mucosa, no central enhancement (unlike inverted papilloma and tumors)
- Case courtesy of Dr Roberto Schubert, Radiopaedia.org, rID: 15098
Determination of Thyroid Cartilage invasion in laryngeal SCC
three most important signs
on MRI what
- Can be challenging bc of variable ossificaiton
- nonossified parts can have attenuation similar to tumour making interpretation challenging
- most reliable criteria used for eval of TC invasion on CT is
- erosion
- lysis
- extralaryngeal spread (tumour on both sides)
- Sclerosis is a reactive phenomenon and does not necessarily indicate acutal cartilage invasion by tumour
- MRI
- can be used to supplement CT.
- better at distinguishing nonossified cartilage from tumour
- however there is propensity for false positives due to reative edema
- invaded cartilage should follow tumour signal on all sequences.
- Helps distinguish ture invasion from edema
- Dual energy CT may imporve evaluation of TC invasion
Parathyroid gland locations
- Locations:
- Upper glands:
- posterior to upper-mid pole of thyroid,
- occasionally extend posterior to pharynx or esophagus
- (CT useful for evaluation of deep locations)
- Lower glands:
- posterior and/or lateral to lower pole of thyroid
- Ectopic locations:
- from angle of mandible
- to lower anterior mediastinum;
- near hyoid,
- arotid sheath,
- intrathyroid, prevascular space in mediastinum
- the inferior glands follow the descent of the thymus
- Upper glands:
What are the types of hypoparathyroidism?
- HypoPT
- low calcium
- high phosphate
- low parathyroid hormone
- surgical removal most common
- Pseudohypoparathyroidism
- low calcium
- high phosphate
- high parathyroid hormone
- End-organ resistance to PTH (hereditary)
- Pseudo-pseudohypoparathyroidism
- Only skeletal abnormalities (Albright hereditary osteodystrophy)
- normal bloods
- skeltal manifesations of Pseudohypoparathyroidism
FIG. 1. Typical features of Albright hereditary osteodystrophy. (A) A young woman with characteristic features of AHO; note the short stature, disproportionate shortening of the limbs, obesity, and round face. (B) Radiograph of the AHO patient showing marked shortening of fourth and fifth metacarpals. (C) Archibald sign, the replacement of “knuckles” with “dimples” due to the marked shortening of the metacarpal bones. (D) Brachydactyly of the hand; note thumb sign (Murderer’s thumb or potter’s thumb) and shortening of the fourth and fifth digits.
- Tracheobronchopathia osteochondroplastica
- very rare idiopathic non-neoplastic tracheobronchial abnormality.
- Epidemiology
- The estimated prevalence on routine bronchoscopy can be up to 0.7%.
- It typically affects those in the 5th to 6th decades
- male predilection 4.
- Clinical presentation
- Most patients are asymptomatic.
- Those who have symptoms may present with cough, shortness of breath on exertion, wheezing or recurrent respiratory infection.
- Haemoptysis can occasionally result from an ulceration of a nodule or an acute infection.
- Pathology
- There is development of osseous or cartilaginous 1-8 mm 2,3 nodules or both in the submucosa of the trachea and bronchial walls.
- They may be either focal or diffuse.
- There is characteristic sparing of the posterior membranous portion of the trachea 6.
- There are two possible theories of pathogenesis
- ecchondrosis and exostosis from cartilage rings
- cartilaginous and osseous metaplasia of the elastic tissue in the internal elastic fibrous membrane
- Differential diagnosis
- tracheobronchial amyloidosis
- unlike TO, classically circumferential involvement
- may appear as focal or diffuse narrowing
- relapsing polychondritis
- like TO, spares posterior membranous tracheal wall
- more likely smooth, hyperdense mural thickening 10
- patients usually have other clinical findings of cartilaginous inflammation 10
- tracheobronchial amyloidosis
Different types of 2nd branchial cleft cysts
- Type 1:
- anterior to SCM
- deep to platusma
- Type 2
- most common
- anterior to SCM
- posterior to SMG
- Lateral to the carotid sheath
- Type 3:
- extends between ICA and ECA
- may extend to lateral pharyngeal wall
- or superior to skull base
- Type 4
- adjacent to lateral pharyngeal wall
Laryngeal Chondrosarcoma
Clinical/Path
- Hyaline cartilage origin, not elastic, path grades I-III
- 0.5% of laryngeal malignancies, mean 64, M>F 3.6:1
- Airway compromise
- Most low grade, but prognosis good despite grade (except for rare myxoid)
- Surgical resection of chondroid lesion usually curative whether benign or malignant
What are Sinonasal Polyps?
mimic
arises from which type of mucosa?
- not true tumour
- sequela of inflammatory rhinosinusitis
- expansion of fluids in the deeper lamina propria of the SCHNEIDERIAN mucosa in the nasal cavity and paranasal sinuses.
- They are the most common expansile lesion in the sinonasal cavity.
- Fibrosis and neovascularisation of nasal polyps may occur and can result in a a vascularised polyp that mimics an angiofribroma on pathology
Radiographic Features of Hashimotos Thyroititis
- Hashimoto thyroiditis:
- Findings vary depending on stage of disease and extent of involvement (diffuse vs. focal)
- Acute:
- Focal nodules against normal or altered background of thyroid parenchyma
- Diffuse hypoechoic and heterogeneous micronodular pattern involving entire gland
- Variable vascularity
- this appearance may be described as pseudonodular or a giraffe pattern.
- Chronic:
- Enlarged hypoechoic micronodular gland
- Hypervascular if patient is hypothyroid (hypertrophic action of thyroid-stimulating hormone [TSH]); following treatment and normalization of TSH hypervascularity decreases
- Atrophic/end-stage:
- Small hypoechoic gland with heterogeneous echo pattern
- Hypovascular
Laryngeal chondrometaplasia
Symptomatic laryngeal chondrometaplasia is a rare entity. Metaplasia is the transformation of one differentiated tissue into another as a response to injury, but the exact etiology of the laryngeal chondrometaplasia is still unknown. The symptomatic laryngeal lesion is usually first discovered by fiber-optic laryngoscopy and subsequently diagnosed by biopsy. Cross-sectional imaging has been increasingly used to assess the exact origin and extent of the lesion.
What are the types of and causes of Hypoparathyroidism?
Hypoparathyroidism
- Primary
- Idiopathic
- autoimmune
- absence of parathyroid glands
- Rare; associated with
- cataracts,
- mental retardation,
- dental hypoplasia,
- obesity,
- dwarfism
- Rare; associated with
- Secondary
- Surgical removal (most common)
- radiation
- Rarely other causes such as autoimmune disease or other