Salivary Glands, Oral Cavity, Esophagel Disorders Flashcards

Question

Mucoepidermoid Carcinoma Overview

Answer 1

* **\< 4 cm, well-circumscribed, partially encapsulated mass w/ infiltrative margins** * _Most common malignant neoplasm_ of salivary gland * Age range: **15-80 y/o** * Overall **M:F 1:2** * Tongue & retromolar area M:F 1:7 * **54% in major salivary gland** * **Parotid** 60-70% * **46% in minor salivary gland**

Answer 2

_Four basic cell types:_ 1. **Squamous cell** 2. **Clear cell** 3. **Intermediate (basaloid) cell** 4. **Mucous cell**

Answer 3

* _Gross_: * **Cystic areas** filled w/ **clear mucus** * _Histology_: * **Large cysts** lined by **cuboidal and mucous cells** * Sheets of **intermediate cells** and abundant **mucous cells** * **No or rare squamous cells** * _Prognosis:_ * Recurrence: 10% * Metastasis: Rare * 5-year survival: 98%

Answer 4

* _Gross_: * **Pinkish to yellowish areas** * _Histology:_ * **No cysts** * Sheets of **intermediate cells** * **Prominent squamous cells** * **Almost no mucous cells** * **Necrosis**, cellular atypia and increased mitosis * _Prognosis:_ * Recurrence: 74% * Metastasis: 30% to regional LN * 5-year survival: 56%

Answer 5

* 10% of all malignant salivary gland tumors * Parotid: less common than Mucoepidermoid & Acinic Cell carcinomas * **Minor salivary gland: most common** * Age: **50-70 years** * **M:F ratio 1:1** * Identical to adnexal tumor of the skin called Cylindroma * **Cribriform vs Tubular vs Solid** morphology

Answer 6

* _Gross:_ * Small **poorly encapsulated** masses within salivary glands * Cut surface: **firm, white tumor** **w/o cysts or hemorrhagic areas** * Punched-out spaces * _Three architectural patterns:_ * **Cribriform** * **Tubular** * **Solid** (worst prognosis) * _Histology:_ * **Small cells** w/ **dark, compact nuclei** and **scant cytoplasm** * **PAS-****⊕****hyaline material** (excess BM) * **Prominent perineural invasion** ("sneaky" fashion)

Answer 7

Directly related of **completeness of excision** at 1^st surgery * _Recurrence at 5 years:_ * 59% for tubular tumors * 89% for cribriform tumors * 100% for solid tumors * _Metastases at 15 years:_ **34% overall** * _15-year survival:_ * 39% for tubular * 26% for cribriform * 5% for solid

Answer 8

* **3%** of all salivary gland tumors * Age range **20-70**, peak in **3^rd decade** * M:F ratio **3:1** * **Parotid** (81%), **minor salivary glands** (15%) * Can be **bilateral** & **multicentric** * Can arise in **intra-parotid lymph nodes**

Answer 9

* _Gross:_ * **Well-circumscribed** mass between **3-5 cm**. w/ **encapsulation** * Can have **cysts** * _Histology:_ * Cells resemble **normal serous cells** * Can have _several architectural patterns_ from **solid** to **papillary/cystic** and **tubular** * No prognostic significance * Usu. have a **prominent lymphoid infiltrate** in the stroma * **Anaplasia** of individual cells is important for prognosis

Answer 10

* **Low grade malignancy** * Recurrence 12% * Metastasis 8% * Death 6% * 5-year survival: 89% * 10-year survival: 68% * 20-year survival: 56% * _Bad prognostic features:_ * Focal necrosis * Neural invasion * Incomplete resection * Involvement of deep lobe * _Good prognostic features:_ * Location in minor salivary gland

Answer 11

**Leukoplakia** and **Erythroplakia** * Risk factors: **alcohol use, tobacco use, exposure to chronic irritants, HPV** * **2:1 male predominance**, seen in **adults** * Highest risk sites are _floor of mouth, ventral surface of tongue_ * Must biopsy any lesion that doesn’t respond to avoidance of tobacco/alcohol

Answer 12

* **White plaque** on **mucous membranes** * Clinical definition: **pathology can range from hyperplasia to dysplasia to carcinoma in situ** * **Cannot remove by scraping** * Cannot classify clinically or microscopically as something else * **5-6%** chance of transforming to **carcinoma**

Answer 13

* **Red, velvety lesion of oral cavity** * Often has associated **dysplasia** * **50%** chance of **malignant transformation**

Answer 14

* **\> 95%** of these are **squamous cell carcinoma** * Often discovered late * 50% fatal overall * _Risk factors_: **tobacco, alcohol, betel nuts** * _Sites_: **floor of mouth, tongue, hard palate, base of tongue** * Best prognosis for lip * Worst prognosis for floor of mouth, base of tongue * _Early_: **raised firm plaques** w/ **irregular roughening** * _Later_: **protruding mass** w/ **central necrosis** forming an **ulcer w/ raised borders** * Can be **superimposed on leukoplakia or erythroplakia** * Begins as **in-situ** and then see range of differentiation * Usually see **extensive local infiltration** before **metastases**, _slow growing_

Answer 15

* Many similarities to oral cavity carcinoma * Mostly **squamous cell carcinoma** * Same risk factors: **tobacco, alcohol, betel nuts** * **Hyperplasia** **→** **dysplasia** **→** **carcinoma** sequence * Generally presents as **hoarseness** * 60% are confined to larynx at presentation * Treat w/ surgery, radiation or combination

Answer 16

* _Structure:_ * Muscular tube 23-25 cm in length * Pharynx → GE junction * _Function:_ * Conduit for food and fluids * Prevention of reflux * **Upper esophageal sphincter (UES)** ⇒ anatomic structure * 3 cm segment formed by _cricopharyngeus muscle_ and portions of prox. _esophageal circular and inferior pharyngeal constrictor muscles_ * **Lower esophageal sphincter (LES)** ⇒ physiologically defined * 2-4 cm segment just proximal to anatomic GEJ

Answer 17

* **Agenesis**: _absence_ of formation (rare) * **Atresia**: thin non-canalized segment ⇒ obstruction * Usu. in association w/ TE fistula * Associated w/ _hydramnios in 3^rd trimester_ * **Tracheoesophageal Fistula**: communication between trachea and esophagus * Incidence of 1/800 live births

Answer 18

Uncomfortable sensation which sits in the pit of your stomach or right in the epigastric region.

Answer 19

Difficulty swallowing History is key! * **Oropharyngeal dysphagia** * **Difficultly getting food to back of mouth** * “Choking” or coughing when eating * Food getting into nose * Likely hx of stroke or any disease that effects striated muscle * **Esophageal dysphagia** * _Solids alone_ ⇒ **mechanical causes** (Ex. Schatzki’s ring) * _Solids & liquids_ ⇒ **motility “nerve” causes** (Ex. achalasia) * Continuous or intermittent? * Continuous ⇒ something “fixed or unchanging” * Associated GERD? * Weight loss?

Answer 20

* _Esophageal Dysmotility_ * **Nutcracker Esophagus** (distal contractions) * **Diffuse Esophageal Spasm** * **LES Dysfunction** * W/o reduced peristaltic contractions * That would be seen in Achalasia) * Dysmotility can lead to development of **diverticuli** * **Ephiphrenic** – just above the LES * **Zenker** – just above the UES

Answer 21

**Outpouching of wall which includes all layers** * **Zenker diverticulum** (pharyngo-esophageal): _above the UES_ * D/t UES dysfunction (premature relaxation) * Pulsion diverticulum * Most common esophageal diverticulum * See **regurgitation** w/o dysphagia, aspiration, neck mass * **Traction diverticulum**: _mid esophageal_ * **Epiphrenic diverticulum**: _above LES_ * D/t discoordination of peristalsis and LES relaxation

Answer 22

**Narrowing of the lumen** Mechanical obstruction * Congenital or acquired * Usu. d/t acquired **submucosal thickening** from: * Chronic GERD, radiation, tumor, systemic sclerosis, caustic injury or extrinsic compression

Answer 23

* _Ledge-like protrusions of mucosa_ in _upper esophagus_ * Mechanical obstruction * **Plummer Vinson syndrome**: iron deficiency anemia, glossitis, cheilosis (corners of the mouth become inflamed)

Answer 24

* _Ledge-like protrusions of mucosa_ in _lower esophagus_ * Mechanical obstruction * **Submucosal ring at squamo-columnar junction** * Type A ⇒ both sides lined by squamous mucosa * Type B ⇒ one side by squamous and the other by columnar mucosa * Symptomatic when lumen ≤ 12 mm * **Intermittent dysphagia to solids** * Dilate with 18-20 mm dilator

Answer 25

* **Aperistasis of esophagus** * Incomplete relaxation of LES w/ swallowing * Increased resting tone of LES * _Pathogenesis:_ * **Degeneration of inhibitory neurons** (ganglion cells) in distal esophagus * Normally release nitric oxide and VIP ⇒ lets LES relax during swallowing Incidence 0.4-1 in 100k; M=F Peak age of 70 with smaller peak 20-40s

Answer 26

* _Primary_: **unknown** (most common) * Idiopathic? Viral? Autoimmune? * _Secondary_: * Western Hemisphere: **Chagas disease** (*trypanosoma cruzi*) * Genetic: **Allgrove’s disease** * Neoplastic: **direct invasion or paraneoplastic** * Infiltrative diseases: **sarcoid** * **Neuropathies** * Special cases: **Pregnancy, obesity, Parkinson’s**

Answer 27

* **Dysphagia to solids and liquids** (100% of pts) * Chest pain * Weight loss – generally only in severe cases * Heartburn * Secondary achalasia has a shorter duration of sx and greater percentage of weight loss (90%) * _Complications:_ * Regurgitation and aspiration * Infection * ↑ Risk of Squamous cell carcinoma (2-4x) * Treat w/ **balloon dilatation, myotomy**

Answer 28

* **Esophageal Manometry** – 100% accurate * **Barium study** * Dilated esophagus above the constricted area * Absence of myenteric plexus from the dilated wall * **“Bird’s beak appearance** * EGD showing Candida

Answer 29

**Protrusion of stomach above the diaphragm** so that it is _in the chest_ Allows reflux to freely flow back into the esophagus Can be sliding or paraesophageal or mixed: * **Sliding hernia** (95%) * Upward displacement of _both esophagus and stomach_ through esophageal hiatus * **Paraesophageal** (5%) * Upward displacement of _stomach_ through hiatus alongside a _fixed esophagus_ * Incidence: 1-20% adults, but only 9% are symptomatic * Complications: ulceration, bleeding perforation, strangulation, contributes to reflux

Answer 30

* **Longitudinal irregular linear tear** of _EG junction or proximal stomach_, variable depth * Associated w/ excessive vomiting and refluxing (most common in alcoholics) * Accounts for 5-10% of UGI bleeding

Answer 31

Esophageal rupture (often fatal)

Answer 32

**Dilation of submucosal and serosal venous plexuses** * _Etiology_: **portal hypertension** (in 90% cirrhotic pts) * _Most common causes:_ * Cirrhosis (seen in 50% of cirrhotic pts, 25-40% w/ cirrhosis have variceal bleeding) * Schistosomiasis * _Clinical presentation_: * Varices are silent until they bleed * Bleeding is an emergency * _Outcome:_ * 30% die w/ 1st bleed * \> 50% rebleed within 1 year * Next episode has a similar mortality rate

Answer 33

* **Reflux esophagitis** * **Infection** (candida, HSV, CMV, bacteria, etc.) * **Ingestion or caustic substance** (acid, alkali) * **Treatment effect** (chemotherapy, radiation, pills) * **Mechanical** (intubation) * **Systemic, Metabolic or Skin Disease** (e.g. Crohn’s disease, Uremia, Pemphigoid, Graft versus Host disease)

Answer 34

* **Early age onset** * **Male** predominant * Environmental or food allergen * GERD likely worsens condition * **Intermittent dysphagia to solids** * _Clinical presentation_ * Feeding difficulties – age 2-3 * GERD/vomiting – age 5 * Abdominal pain – age 9 * Dysphagia – age 11 * Food impactions and esophageal strictures – age \>12 * _Diagnosis_: EGD with biopsy

Answer 35

* GERD * Hypereosinophilic Syndrome * Parasitic Diseases * Allergic Vasculitis * Esophageal Leiomyomatosis * Periarteritis * Inflammatory Bowel Disease

Answer 36

* PPI therapy * Symptom diary * Allergy testing * Swallowed steroid * Allergy medication

Answer 37

**Intermittent motility issues** * Can be triggered by drinking cold liquid or stress * ± **Severe CP** and difficulty swallowing solids and liquids (at the time of spasm) * X-ray shows a **“cork-screw esophagus”** * Tx targeted towards alleviating the pain * PPI if GERD is the cause * TCA’s at low dose to numb GI hypersensitivity

Answer 38

Calcinosis; Raynaud’s phenomena; Sclerodactyly; Telangiectasia; Esophageal dysmotility * **Esophageal dysmotility** * Aperistalsis of esophagus * Low pressure LES * Scleroderma esophagus * _Pathophysiology_ * **Smooth muscle intimal fibrosis** ⇒ LES being fibrosed open * Results in **severe, unrelenting reflux** * _Treatment_ * Small non-fatty meals * Aggressive PPI therapy * Dilation if acid related strictures develop * Must be careful performing a fundoplication ⇒ can cause further dysphagia

Answer 39

**Reflux of gastric contents** **into esophagus** Acid, bile or non-acid material * Most common cause of esophagitis * _Anatomic causes_ * **Defective LES barrier** * **Transient relaxation** most important cause of reflux * Mediated by vagal pathways * Triggered by gastric distension by gas or food, after swallowing, or ↑ intraabdominal pressure * Diaphragmatic weakness * Crucal disruption * **Hiatal hernia**: no clear cause and effect relationship but most pts w/ severe esophagitis have HH * _Gastric factors_: * **L****oss of normal anatomic barrier** allows acid to reflux up into esophagus * Acid/pepsin, delayed emptying * _Dietary factors_: Tomato based foods, Chocolate, Caffeine, Spicy foods, citrus, garlic, mint, onions * _Lifestyle factors_: pregnancy, obesity, eating and lying down, smoking, alcohol

Answer 40

* _Typical symptoms_ * Heartburn * Regurgitation (can be acid or food) * Sour taste in mouth * _Atypical symptoms_ * Laryngitis * Cough * Refractory asthma

Answer 41

* **Esophagitis** * Eosinophils seen in GERD & motor d/o of esophagus * **Basal zone hyperplasia** ⇒ \> 20% of epithelial thickness

Answer 42

* _Acid suppression medications_ * **Proton Pump inhibitors** * Bind to _active proton pumps_ (predominantly in the body of stomach) * _Irreversibly_ bind with regeneration of pumps every 48 hrs * **Best effect is to take PPI 30 mins before meal**s * Immediate and delayed release forms * Ex: omeprazole, esomeprazole, pantoprazole, lansoprazole * **Histamine blockers** * Bind to the _histamine receptor_ of the Proton Pump * Tachyplaxis (building up tolerance) – can develop in 2/3s * **Better to take as needed** * Works on demand * Ex: ranitidine, famotidine * _Diet and lifestyle changes_ * **Avoid certain foods** * Tomato based, spicy, citrus, onion, garlic, mints, chocolate, caffeine * **Don’t eat and recline for at least 2 hours** * 4 hours if gastroparesis or slow emptying stomach * Wedge under mattress to elevate chest while sleeping * **Stop tobacco** * **Weight loss**

Answer 43

* **Erosive esophagitis** * Ulcer * Stricture * **Pneumonitis** * **Barretts esophagus** * **Adenocarcinoma** of the esophagus

Answer 44

* **Inflammation of the esophagus** seen during _endoscopy_ * Appears as **“red streaks”** or **ulcers** * In 10% of patients with true GERD * Pts w/ esophagitis usually need PPI therapy * Graded various ways

Answer 45

* **Esophageal metaplasia** * Normal esophageal columnar epithelium → **intestinal epithelium with goblet cells** * EGD: red, velvety tongue-like area of columnar mucosa w/ goblet cells * Incidence ↑ * Up to 10% w/ GERD sx * Most common in white men 40-60 * Higher risk for **dysplasia** in _longer segment Barretts_ (\> 3 cm) * Can lead to adenocarcinoma * Need surveillance to look for dysplasia and carcinoma

Answer 46

* **Neoplastic epithelium that remains confined within the basement membrane** * Lesion can be **flat** or **raised** (including polypoid) * Graded-two tiers: * Low grade * High grade * Precursor lesion to **Adenocarcinoma** * Presence of dysplasia @ initial clinical presentation important predictor of outcome * Controversial whether dysplasia regresses * Dysplasia & risk of adenocarcinoma **does not respond to surgical or medical therapy** * _Clinical strategies:_ * Prevention of development of BE (treat GERD) * Biopsy surveillance of BE (unclear if improves pt survival) * If **multifocal high-grade dysplasia**, **Carcinoma in situ**, or **invasive adenocarcinoma** found ⇒ intervene

Answer 47

**Abnormal tightening or narrowing of the esophagus** * **Dysphagia to solids** w/ strictures ≤ 12mm * True location of the stricture is _where the patient points or below that area_ * Aim of **dilation**: ↑ luminal diameter to ≥ 12–14 mm * Results in immediate improvement/resolution of dysphagia in most * Methods of dilation: * **Bougie** – Exerts radial and axial forces as it traverses the stricture (proximal to distal) * **Balloon** – Exerts mainly radial forces equally throughout the stricture

Answer 48

* 1% of cancers in the US * 6% of cancers of the GI tract * _Worldwide_: **Squamous Cell CA (SCCA)** ⇒ 90% of esophageal cancers * _In US_: 50% SCCA, 50% AdenoCA * D/t rapid rise in AdenoCA in last 2 decades * Cancer types other than SCC and AdenoCa are very rare

Answer 49

* **\> 75% distal esophagus** * _Histology:_ * **Mucin-producing glands** * Less often, **signet ring cells** * _Gross appearance:_ * Flat patch * Raised patch * Nodular mass * Deep ulcerative

Answer 50

* **Silent** until advanced disease has developed * Manifests w/ **difficulty/painful swallowing** * _Moves from solid to liquid foods_ * **5-year-survival overall \< 25%** * If found early w/ adenoCA only in mucosa or submucosa, can have 5-year survival of 80%

Answer 51

* Most pts **\> 45 y/o** * **Men \> women** (4:1 in US) * Blacks \> Whites (6:1 in US) * _High incidence areas:_ * Northern Iran, Central Asia, Northern China, Puerto Rico, South Africa, Eastern Europe

Answer 52

* Exposure to **dietary/environmental carcinogens** is causal * In US and Europe: * **Alcohol** and **tobacco** * In high incidence countries: * **F****ungus**or**nitrosamine-****containing foodstuffs** * **HPV** may also play a role * **Nutritional deficiencies** potentiate * Genetic factors less important * **Previous esophageal damage** also a factor

Answer 53

* **50% in middle** **⅓** **of esophagus, 20% in upper** **⅓** * **Squamous dysplasia** **→** **CIS** * **Exophytic** ⇒ protrude into the lumen * **Infiltrative** ⇒ grow deeply into the wall * Can invade surrounding structures * Mostly **moderate-well differentiated** * When symptomatic ⇒ tumor usu. large and invasive * Pt moves from _solid to liquid foods_

Answer 54

_5 yr survival:_ * 75% w/ superficial disease (rarely diagnosed at this stage) * 25% in pts w/ curative resection, advanced disease * 9% in all pts

Salivary Glands, Oral Cavity, Esophagel Disorders Flashcards

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