Head and Neck Flashcards
1
Q
Cause
Gender
Age
IF
A
Lichen Planus
- Autoimmune mucositis
- F>M
- Young adult – middle age at presentation
- Usually asymptomatic
- Reticular pattern of raised, white (hyperkeratotic) streaks on red background (Wickham’s striae)
- Sawtooth rete ridges
- Band-like subepithelial chronic inflammatory infiltrate
- IF: granular fibrinogen at basement membrane
2
Q
Cause
Age
Gender
Location
Prognosis
A
Squamous Cell Carcinoma Non-Keratinizing
- Oropharynx (base of tongue, tonsil)
- Related to HPV-infection (High Risk 16, 18, 31, 33)
- Men
- age 50s-60s
- cystic lymph node metastases
- Good prognosis
- p16 positive
3
Q
Cause
Age
Gender
Stains
A
Nasopharyngeal (Lymphoepithelial) Carcinoma
- High prevalence in: Hong Kong, China, Southeast Asia, North Africa, Arctic
- EBV exposure, nitrosamine ingestion
- M > F
- Age > 30
- Presentation: Epistaxis, Otitis media, Neck metastases
- Can be thought of as a variant of squamous cell carcinoma
- p63/p40+, HMWK+
- Keratinizing
- Not associated with EBV
- NON-keratinizing
- syncitial growth w/ inflammatory infiltrate
- USUALLY EBV+
- Somewhat more radiosensitive
4
Q
Mandible & Maxilla
- __ Related Osteonecrosis of Jaw
- Odontogenic Lesions
- Mesenchymal Neoplasms
A
Mandible & Maxilla
- Bisphosphonate Related Osteonecrosis of Jaw
- Odontogenic Lesions
- DC vs OKC
- Ameloblastoma
- Mesenchymal Neoplasms
- Osteoma
- Osteosarcoma
5
Q
A
Bisphosphonate Related Osteonecrosis of Jaw
- Tenderness and exposed bone in posterior mandible
- Triad of Acute osteomyelitis, Osteonecrosis, Oral flora colonization
- Secondary to bisphosphonate use (for tumor, osteoporosis):
- Alendronate (Fosamax®), Risedronate (Actonel®), Ibandronate (Boniva®), Pamidronate (Aredia®), Zolendronate (Zometa®) (Reclast®), Denosumab (Xgeva®) (Prolia®)
6
Q
Odontogenic Cysts around the crown of an unerupted tooth, DDx includes:
A
Odontogenic Cysts around the crown of an unerupted tooth, DDx includes:
- Dentigerous Cyst
- Odontogenic Keratocyst (OKC)
- Cystic Ameloblastoma
7
Q
A
Odontogenic Keratocyst
- Gorlin – AKA – Nevoid BCC Syndrome
- AD, Gene: PTCH, Chromosome: 9q22.3
- Odontogenic Keratocysts / “Gorlin cysts”
- Multiple BCCs (even on non-sun exposed skin)
- Medulloblastomas
- Palmar / plantar pits
- Skeletal anomalies
- Features of OKC squamous epithelium
- 5-7 cells thin
- Palisaded basal layer (fence)
- Corrugated surface (cardboard)
- May have daughter cysts in wall, leads to recurrence
8
Q
A
Dentigerous cyst
- Proliferative squamous lining
- Inflammation
- OKC can look like this but with only focal diagnostic lining – look carefully!
9
Q
A
Ameloblastoma
- Can be cystic or solid
- Solid: Large epithelial nests
- Recapitulates tooth development
- Peripheral reverse polarity (nuclei away from basement membrane)
- Central stellate reticulum
- Locally destructive, requires hemimandibulectomy
10
Q
A
Odontoma
- Gardner AKA variant of FAP
- APC gene Chr 5q21
- Colonic polyposis / carcinoma
- Osteomas of the skull
- Desmoid tumors (fibromatosis)
- Thyroid cancer
- Epidermoid cysts
- Sebaceous cysts
- Osteomas in general
- comprised of compact cortical bone with little stroma
- Most frequent in frontal / ethmoid sinus
- 70-90% of patients with MULTIPLE osteomas have Gardner syndrome
11
Q
Nose & Paranasal Sinuses & Nasopharynx
A
Nose & Paranasal Sinuses & Nasopharynx
- Sinusitis
- Fungal: Mycetoma, Allergic Sinusitis, Invasive Sinusitis, Rhinosporidiosis (vs. Rhinoscleroma)
- Granulomatosis with polyangiitis
- Tumors & Tumor-like lesions
- Inflammatory Polyp
- Respiratory Epithelial Adenomatoid Hamartoma
- Schneiderian Papilloma
- Nasopharyngeal Angiofibroma
- Sinonasal Adenocarcinoma
- High Grade Tumor Differential (SNUC, etc)
12
Q
A
Granulomatosis with Polyangiitis
Systemic necrotizing vasculitis
Respiratory tract +/- glomerulonephritis
c-ANCA+ in 50-90%
Treatment with immunosuppression
Histology
Granulomas
Vasculitis
Neutrophilic microabscesses
13
Q
Sinonasal Tumors
- Most common site is:
- Followed by:
A
Sinonasal Tumors
- Most common site is:
- Maxillary sinus 60%
- Followed by:
- Nasal cavity 20-30%
- Ethmoid sinus 10-15%
- Sphenoid sinus 1%
14
Q
A
Inflammatory Polyp
- NON-neoplastic
- Chronic sinusitis
- Cystic fibrosis in kids
- Lateral nasal wall masses
- Thick basement membrane
- Myxoid stroma with Eos
- Atypical stromal fibroblasts
- Cystically dilated glands
15
Q
A
Respiratory Epithelial Adenomatoid Hamartoma
- Benign NON-neoplastic hamartoma
- Adult Males
- Posterior nasal septum
- Proliferation of respiratory glands w/ goblet cells
- Surface invaginations
- Stromal hyalinization / thick basement membrane
16
Q
Schneiderian Papilloma subtypes
- Gender
- Age
- Location
- Histology
- Cancer %
A
Schneiderian Papilloma subtypes
- Classic Inverted Type
- M > F
- Age: 40-70
- Location: lateral nasal wall
- Histology: Predominantly endophytic growth, Neutrophilic microabscesses in epithelium
- Cancer: 30%
- Oncocytic Type (“cylindrical cell”)
- M = F
- Age: > 50
- Location: lateral nasal wall
- Histology: Endophytic and exophytic growth, microcysts in epithelium
- Cancer: 20%
- Exophytic Type (“fungiform”)
- M > F
- Age: < 50
- Location: nasal septum
- Histology: Predominantly exophytic growth
- Cancer: rare
17
Q
A
Inverted Schneiderian Papilloma
18
Q
A
Oncocytic Schneiderian Papilloma
19
Q
A
20
Q
2 Subtypes
- Gender
- Risk factors
- Type/Location
- IHC
- Histology
A
Sinonasal Adenocarcinoma
Non-intestinal Type (Non-ITAC)
- Gender: M > F
- Risk factors: none
- Type/Location:
- Low grade: ethmoid sinus
- High grade: maxillary sinus
- IHC: CK20-, CDX2-, CK7+
- Small back to back glands, Round to oval nuclei
Intestinal Type (ITAC)
- Gender: M > F
- Occupational: wood and leather dust exposure
- Type/Location:
- Most common type is colonic (int grade)
- Most common location is ethmoid sinus
- IHC: CK20+, CDX2+, CK7 var
- Large dilated glands, Dark elongated nuclei
21
Q
A
Nasopharyngeal Angiofibroma
- Adolescent Males
- FAP patients
- Slit-like blood vessels
- Clinically bloody
- Surrounded by dense eosinophilic fibrous stroma with bland spindle cells
- AR+ / Beta-catenin +
- Surgery +/- Embolization
22
Q
A
HPC-like tumor of sinonasal cavity
- Mean age 60s
- Monotonous spindled to oval cells
- Rich vasculature – Staghorn-like with prominent perivascular hyalinization
- SMA+, CD34-
- Surgical excision
23
Q
A
Low-grade Sinonasal Sarcoma With Neural and Myogenic Features
- Monotonous, cellular spindle cell neoplasm
- Infiltrative
- S-100 +
- SMA +
- t(2;4) in 2 tested cases
- Locally aggressive but no metastases
24
Q
A
25
Q
A
26
Q
A
27
Q
A
NUT Midline Carcinoma
- Head & Neck (35%) and Mediastinum
- High grade carcinoma with squamoid features
- May be keratin / p63 positive
- Primitive, monotonous cells with:
- Round nuclei, nucleoli
- Amphophilic to clear cytoplasm
- Occasional abrupt squamous differentiation
- NUT;BRD4 or 3 translocation (IHC/FISH)
- Median survival < 1 year
28
Q
A
29
Q
Larynx
- Most common benign tumor
- Most common malignant tumor
- Most common neuroendocrine tumor
- Most common sarcoma
A
Larynx
- Most common benign tumor – Papilloma
- Most common malignant tumor – SCC
- Most common neuroendocrine tumor – Atypical carcinoid
- Most common sarcoma – Chondrosarcoma
30
Q
A
Squamous Papilloma
- Low Risk HPV 6/11
- Single
- Adult Males
- Multiple
- Children
- “Recurrent respiratory papillomatosis”
- Acquired in birth canal of mother
- Multiple throughout larynx, tracheobronchial tree
- Occasionally lead to SCC, more so with radiation/smoking
31
Q
Epidemiology of laryngeal SCC
- Gender
- Age
- Risk factors
- Most common site
- Prognosis
A
Epidemiology of laryngeal SCC
- M > F
- Age 50s-60s
- Tobacco, Alcohol
- Most common site:
- Glottis (in US)
- Supraglottis (in most of western Europe)
- Prognosis: Stage-dependent
- Cartilage invasion / bulky tumour volume → poor response to chemoradiotherapy
- Histologic grade has limited impact
32
Q
A
Neuroendocrine Tumors of Larynx
- Atypical carcinoid is most common
- M>F
- a/w smoking
- Most common in supraglottis
- 50% nodal and distant mets
- Surgery (chemo/radio insensitive)
- Keratin +, Synap +, CMG +
- May have: Calcitonin +, mCEA +, TTF-1 RARE
- Small cell carcinoma is second most common
- Rare: paragangliomas, typical carcinoids
34
Q
Laryngeal Sarcomas
A
Laryngeal Sarcomas
- Chondrosarcoma is most common
- Rare but more common than benign chondroma
- Most common in cricoid cartilage
- Age 40+
- Usually low-int grade, low metastatic rate, curable by excision with clean margins
36
Q
A
37
Q
A
Necrotizing Sialometaplasia
- Most common in palate
- Frequent ulceration
- ? Ischemia
- following surgery, radiation
- Lobular configuration maintained
- Ductal structures
- With lumina, +/- mucinous cells, intraluminal necrosis
40
Q
A
41
Q
A
42
Q
A
Mucoepidermoid Carcinoma
43
Q
A
44
Q
A
Adenoid cystic
45
Q
A
Mammary Analog Secretory CA
- Resembles secretory carcinoma of breast:
- Stains with Mammaglobin and S-100
- Carries same translocation (>90%):
- ETV6-NTRK3 -> tyrosine kinase fusion protein (absent in Acinic Cell)
- Can undergo high grade transformation
47
Q
A
Polymorphous Low Grade Adenocarcinoma
- Epidemiology:
- Most common location is palate
- But most common tumor in palate is NOT PLGA (adenoid cystic)
- Histology:
- Single cell type
- Pale oval nuclei (compare to AdCC)
- Various architectural patterns (polymorphous)
- Lobular, papillary, cystic, cribriform, trabecular, ductal, single file, whorling
- Infiltrative growth pattern
- Single cell type
- S-100+
- PRKD1 mutation
48
Q
A
Low Grade Cribriform Cystadenocarcinoma
- Resembles low grade ductal lesions of breast (ADH, low grade DCIS)
- Formerly thought to be the low grade counterpart of salivary duct carcinoma
- Not been proven
- No Her-2-neu amplification
- Few cases, Excellent prognosis
- S-100+
49
Q
A
Salivary Duct Carcinoma
- Epidemiology:
- De novo or Ca-ex-PA
- Aggressive
- Histology:
- Resembles high grade ductal carcinoma of breast: large nests with peripheral cribriforming and central necrosis
- PNI and LVI are common
- Androgen Receptor +
- Although ER/PR neg
- Genetics:
- Her-2-neu amplification in some
50
Q
A
Hyalinizing Clear Cell Carcinoma
- Epidemiology:
- Intraoral minor salivary glands
- Histology:
- Monomorphous, clear cells with wrinkled nuclei
- Pink hyaline stroma: thin septae or dense bands
- IHC:
- Pos: Keratin, HMWK, p63/p40
- Neg for other myoep markers: S-100, GFAP, SMA, calponin
- Genetics:
- EWSR-ATF1
- Identical to clear cell odontogenic carcinoma
52
Q
A
Cholesteatoma
- Squamous epithelium from external canal migrates onto eardrum
- Destructive invasion into drum / mastoid
- Related to chronic infections
- Morbidity if deep into skull
53
Q
A
Jugulotympanic Paraganglioma
- Most common tumor of middle ear
- NE markers +
- Keratin –
- S-100 + sustentacular cells
- May be locally infiltrative
54
Q
A
55
Q
A
Papillary Endolymphatic Sac Tumor
- Inner ear (temporal bone)
- Associated with VHL
- Hemangioblastomas, renal cysts and tumors, pancreatic cysts and tumors, pheochromocytomas, epididymal / ovarian cystadenomas
- Hearing loss
- Local invasion
- No metastasis
56
Q
Arising in ovary
A
Papillary Thyroid Carcinoma Arising in Struma Ovarii
- rare, no current consensus as to how to treat
- Struma ovarii is a specialized monodermal teratoma that is composed predominately of thyroid tissue
- hyperthyroidism in 5% to 8%
- 5% to 10% are malignant (ID’d post surgery)
- PTC most common carcinoma
- same BRAF mutation
57
Q
A
tall cell variant of papillary carcinoma
59
Q
Thyroid, diagnosis?
A
Radioactive iodine treatment
- Nuclear changes occur in thyroid follicular epithelial cells following administration of radioactive iodine.
- Propylthiouracil, potassium iodide, and β-blockers do not usually produce nuclear abnormalities.
- Nuclear abnormalities may be found in Hashimoto thyroiditis.
- Histologic features of Hashimoto thyroiditis, such as oxyphil metaplasia, fibrosis, and significant/destructive chronic inflammation, are not characteristic of radioactive iodine treatment.
- Optically clear nuclei, which characterize papillary thyroid carcinoma, are not a feature of radioactive iodine treatment.
66
Q
A
Hashimoto thyroiditis
67
Q
Several studies have demonstrated __ deposition within the context of Hashimoto thyroiditis.
- Laboratory work-up of patients with Hashimoto thyroiditis may reveal __ antibodies.
- These autoantibodies likely mediate thyrocyte dysfunction as follows: deposition of__ on the __ of follicular epithelial cells leads to __ and thyrocyte necrosis.
- Hashimoto thyroiditis is associated with several human leukocyte antigen (HLA) alleles including __.
- The incidence of Hashimoto thyroiditis is increased among individuals with __.
A
Hashimoto thyroiditis
- Several studies have demonstrated immune complex deposition within the context of Hashimoto thyroiditis.
- Laboratory work-up of patients with Hashimoto thyroiditis may reveal antithyroglobulin, antithyroid peroxidase, and antimicrosomal antibodies.
- These autoantibodies likely mediate thyrocyte dysfunction as follows: deposition of immune complexes (as immune complexes) on the basement membranes of follicular epithelial cells leads to complement activation and thyrocyte necrosis.
- Hashimoto thyroiditis is associated with several human leukocyte antigen (HLA) alleles including HLA-DR3, HLA-DR4, and HLA-DR5.
- The incidence of Hashimoto thyroiditis is increased among individuals with Turner syndrome and Down syndrome.
68
Q
5 Salivary gland cribriform lesions?
A
Basal cell adenocarcinoma
- Basal cell adenocarcinoma (which represents the correct diagnosis in this case, based on the information provided as well as other findings) sometimes shows cribriform architecture.
- Adenoid cystic carcinoma characteristically shows cribriform architecture. The pseudocysts within cribriform areas of adenoid cystic carcinoma show either mucoid basophilic material (glycosaminoglycans), or hyaline eosinophilic material (reduplicated basal lamina).
- Low-grade salivary duct carcinoma is alternatively named low-grade cribriform carcinoma.
- Mammary analogue secretory carcinoma characteristically shows lobular cribriform or microcystic architecture, with mucin in the microcysts/pseudocysts.
- Benign lesions such as basal cell adenoma and pleomorphic adenoma may also display cribriform patterns.
69
Q
Dx?
Cause?
Result?
HLA?
Causes of primary hyperthyroidism?
Causes of secondary hyperthyroidism?
A
- Graves disease
- antithyroid-stimulating hormone receptor (anti-TSHR) antibodies, which cause constitutive activation of follicular epithelial cells
- overproduction of triiodothyronine (T3) and thyroxine (T4)
- Histologic features
- papillary hyperplasia
- “scalloping” of colloid
- follicles containing little or no colloid
- columnar morphologic appearance
- HLA-B8 has been associated with an increased risk for development of Graves disease
- HLA-Bw35 has been associated with an increased risk for development of subacute thyroiditis.
- Causes of primary hyperthyroidism
- Graves disease, “toxic” goiter, and “toxic” adenoma
- Causes of secondary hyperthyroidism
- pituitary adenoma and gestational thyrotoxicosis
70
Q
Dx?
Age?
Gender?
Prognosis?
Staining?
Genetics?
A
Salivary duct carcinoma
- > 50 yo
- M > W
- most aggressive salivary gland malignancies
- perineural invasion and lymph node involvement occur in the majority
- occurs de novo and/or within preexisting pleomorphic adenoma (i.e., as a manifestation of carcinoma ex pleomorphic adenoma)
- positive nuclear immunohistochemical staining for androgen receptor, and positive membranous staining for Her-2/neu
- 17q21.1 amplification, involving the gene ERBB2, 40% of salivary duct carcinoma
71
Q
A
Follicular neoplasm of thyroid
• The photomicrograph shows a highly cellular specimen containing follicular cells arranged in a microfollicular pattern. This appearance combined with finding scanty or no colloid is suggestive of a follicular neoplasm. Rosette-like microfollicles may not appear as well formed as those in the photomicrograph. Clusters or small, tight aggregates of follicular cells may be seen.
82
Q
A
THYROID GLAND: Hyalinizing Trabecular Adenoma
- Paraganglioma-like adenoma of the thyroid (PLAT)
- Rare, “benign” neoplasm
- May be mistaken for PTC or MTC
- Gross: 3-4 mm, yellow-tan, circumscribed
- Prominent trabeculae in abundant hyaline stroma
- INCI, nuclear grooves and Psammoma bodies
- Metachromatic basement membrane-like material between cells *
- 2-5 micron, yellow, spherical, cytoplasmic bodies with clear halo of retracted cytoplasm and nuclear molding (representing giant lysosomes)
- Thyroglobulin +
- ? Variant of PTC due to RET/PTC rearrangements
- May be related to PTC through common pathway for abnormal production of basement membrane
- Hyalinizing trabecular carcinoma (vascular/capsular invasion)
85
Q
A
Tall Cell Variant of PTC
86
Q
A
Columnar Cell Variant of PTC
88
Q
A
THYROID GLAND: Medullary Carcinoma
Tumor of parafollicular cells
Calcitonin – serum levels
Isolated, inherited familial, autosomal dominant pattern
MEN type II
Medullary Ca. and pheochromocytoma = Sipple syndrome
VHL
NF
90
Q
A
THYROID GLAND: Anaplastic/Undifferentiated CA
- One of the most aggressive human malignancies
- Survival in months
- Rare
- Elderly patients
- Women
- Large bulky mass – “bull neck appearance”
- P53 mutation
