GI Tract Pathology - Salivary Glands and Esophagus

Which salivary gland is the largest, has the longest duct, and has the majority of issues

Parotid

What are 2 processes that can cause inflammation in salivary glands

Viral infection

Obstruction

What is the most common viral cause of inflammation in the salivary glands (parotid)

mumps

Causes bilateral inflammation of the parotid gland

mumps

What in the serum is characteristically elevated in a mumps infection

Amylase

Other than the parotid, what organs can be affected by mumps

Testis - orchitis

Pancreatitis

If a patient is infected with mumps, what in the serum would tell you the pancreas is also affected? Explain.

Parotid acini destruction will result in elevated amylase. The pancreas also secretes amylase, so this doesn't help. One thing the pancreas secretes that the parotid does not secrete is LIPASE. If the pancreas is also involved, serum LIPASE will be elevated as well.

What is the danger of mumps in teens?

Orchitis - seminiferous tubules are inflamed

This can cause permanent damage to sperm production in male teens and result in STERILITY

When you think about mumps, what are the 3 structures you should worry about

parotid glands

pancreas

testes in males

What is Sialadenitis

inflammation of the parotid

Silo = saliva = inflammation of salivary gland

What is Sialolithiasis

silo = saliva. lith = stone

Inflammation of parotid gland due to a stone

What salivary gland is most at risk for sialolithiasis

Parotid because it has the longest duct

-this typically occurs unilaterally

What is the most common infection due to obstruction of salivary gland (Sialolithiasis)

S. Aureus

What are the 2 types of benign tumors that occur in the major salivary glands? Which of the 3 major salivary glands do the majority of these tumors occur in?

Pleomorphic Adenoma

Warthin Tumor

Most occur in the parotid

Do the 2 types of benign tumors that occur in major salivary glands often turn into a malignancy?

Pleomorphic Adenoma

Warthin Tumor

Rarely turn into malignancy

What change usually accompanies P. adenomas and W. Tumors

facial nerve damage - these tumors push on the facial nerve and cause pathology

What is the most common tumor of major salivary glands

pleomorphic adenoma

-typically affects the parotid gland

What is the presentation of a pleomorphic adenoma in the parotid gland

presents as a mobile, painless mass near the angle of the jaw

The following describes what

-slow-growing tumor

-well encapsulated

-composed of chondromyxoid stromal cells (connective tissue), myoepithelial cells, and glandular cells

Pleomorphic adenoma

-most common benign tumor in the major salivary glands

-most commonly in parotid

What dose it mean that pleomorphic adenomas are biphasic

made of multiple tissues

-stroma (connective tissue)

-epithelium (glandular cells)

Do pleomorphic adenomas typically damage the facial nerve?

No

What is the rate of reoccurrence of a pleomorphic adenoma. Explain

Has a HIGH rate of reoccurrence because the tumor has an atypical shape. Because of this, it is difficult to fully remove and if it is not fully removed it will grow back

Transformation into a malignancy is uncommon

Know that P. adenoma has a HIGH rate of reoccurrence

The following describes what

-benign cystic tumor with abundant lymphoid follicles

-usually arises in the parotid

Warthin tumor

Warthin tumors have a strong association with

smoking - KNOW THIS

What tissue is found in Warthin tumors

lymphoid tissue and cystic tissue

What is the difference between Benign and Malignant tumors

metastasis

What is the most common malignant tumor of the salivary glands

Mucoepidermoid carcinoma

Muco = mucous

Epiderm = epidermis

When do Mucoepidermoid carcinomas occur and what sex is it more common in

Typically occurs 30-50 years old and has a slight female predominance

What is a Mucoepidermoid carcinoma composed of (what tissues)

squamous cells (epidermoid)

mucous secreting cells (muco)

Malignant growth of a Mucoepidermoid carcinoma commonly affects what structure and causes what

facial nerve and causes face paralysis

The majority of Mucoepidermoid carcinomas are characterized by a translocation that leads to the formation of the fusion gene _______, which disrupts normal transcription

MAML2

What is the treatment for Mucoepidermoid carcinomas

surgical resection

Prognosis is good with (low or high grade) Mucoepidermoid carcinomas

Good with low grade tumors = has well differentiated cells

Bad with high grade = less differentiated cells = poor prognosis

-nucleus:cytoplasm ratio is high (blue is bad)

-multiple nucleoli

-abnormally shaped nuclei

What is the main difference between Mucoepidermoid carcinomas and Pleomorphic adenomas/Warthin tumors

Mucoepidermoid carcinomas are malignant = metastasize

Pleomorphic adenomas/Warthin tumors are benign = their growth spreads locally

How could you tell the difference between Pleomorphic adenomas/Warthin tumors or Mucoepidermoid carcinoma based on patient symptom presentations alone

Mucoepidermoid carcinoma can invade the facial nerve and cause facial paralysis

Pleomorphic adenomas/Warthin tumors can push on the facial nerve, but won't invade it. Therefore they will not cause facial nerve paralysis, however they could possibly cause mild symptoms due to pushing on facial nerve, but it won't be major

What are esophageal webs

Thin, MUCOSAL shelf-like projections that DO NOT surround the circumference of the esophagus (only made of mucosal layer)

Typically formed in the UPPER esophagus

The following describes what

-more common in postmenopausal women and are often associated with bullous disease

-mucosal shelf in the upper 1/2 of the esophagus

-does not surround the circumference of the esophagus

Esophageal Web

What is Plummer-Vinson syndrome

combination of

-esophageal web

-iron deficiency anemia

-glossitis (tongue inflammation)

Patient will present with dysphagia and halitosis (bad breath)

Esophageal webs increase risk of squamous cell carcinoma of the esophagus

The following describes what

-Patient has dysphagia and halitosis

-Pt. has iron deficiency anemia

-Pt. has glossitis

-Pt. has esophageal web

Plummer-Vinson syndrome = a combo of

-esophageal web

-iron deficiency anemia

-glossitis (tongue inflammation)

What are Shatzki rings

esophageal rings formed as a response to a chronic acid reflux disease

formed in the LOWER esophagus

these are COMPLETE RINGS

include both MUCOSA and SUBMUCOSA

The following describes what

-complete rings found in the lower part of the esophagus

-includes mucosa and submucosa

Shatzki rings

What are the main differences between Shatzi rings and esophageal webs

Esophageal webs

-typically upper esophagus

-make a shelf and do not line the circumference of the espoh

-made of the mucosal layer only

Shatzi

-typically lower esophagus

-complete rings around the esohpagus

-include both mucosa and submucosa

What is Mallory-Weiss Syndrome

associated with mucosal longitudinal lacerations caused by vomiting

The following describes what

-mucosal longitudinal lacerations

-caused by vomiting

-pt. presents with painful hematemesis

Mallory-Weiss Syndrome

What is Boerhaave syndrome and what is a predisposition for its development

Boerhaave syndrome = perforation of the esophagus

-leads to air in the mediastinum and subcutaneous emphysema

Predispotition = Mallory-Weiss Syndrome

Boerhaave syndrome is most commonly seen in what people

binge drinkers/alcoholics

-caused by vomiting --> happens a lot in people who binge drink

Also seen in patients who had endoscopy

The following describes what

-a complication to Mallory-Weiss syndrome where the esophagus is perforated leading to air in the mediastinum and subcutaneous emphysema

Boerhaave syndrome

What causes esophageal varices

-result from liver cirrhosis and consecutive portal hypertension

-increased pressure in the portal vein causes blood backup in the left gastric vein

-L gastric vein drain lesser curve of stomach and lower esophagus

-results in a formation of submucosal esophageal varices, which are anastomoses between lower and upper esoph veins that drain into the azygous veins bypassing the portal system

What is a danger of esophageal varices

They are typically asymptomatic

if they rupture they will cause painless hematemesis and can be fatal if not treated

What is the most common cause of death in cirrhosis patients

Esophageal varices

What is the main difference between esophageal varices and Mallory-Weiss syndrome

Esophageal varices = painless hematemesis

Mallory-Weiss = painful hematemesis

What are the most common causes of eosinophilic esophagitis

Allergic response

-asthma

-food allergies

The following is a sign of what

-numerous mucosal rings and furrows that can be seen on EGD

Eosinophilic esophagitis

Does eosinophilic esophagitis cause an increase in caner risk?

There is no increased risk of cancer

No association with GERD (won't respond to GERD treatment)

No metaplastic changes

GERD and Barrett's esophagus are caused by

reflex

The following describes what

-results from reduced LES tone and presents as heartburn and regurgitation

GERD

What are some risk factors for GERD

EtOH - bolded in notes

Obesity

Tobacco

What is Barrett's esophagus and what causes it

Barrett's = type of metaplasia in which the normal strat epi is replaced with simple columnar + GOBLET CELLS (intestinal epi)

This is caused by prolonged exposure to acid from acid reflux (GERD)

Barrett's esophagus increases the risk of

esophageal adenocarcinoma 10-50x

The following describes what when seen on endoscopy

-Salmon patches

Barrett's esophagus

In Barrett's esophagus, does the epithelium become

simple columnar WITH goblet cells

What are the 2 types of esophageal cancer?

squamous cell carcinoma

adenocarcinoma

How does esophageal cancer present? What is the prognosis?

it presents LATE with PROGRESSIVE DYSPHAGIA (first solids, and then liquids become hard to swallow)

Typically has a poor prognosis

Adenocarcinoma is related to what disorder of the esophagus while squamous cell carcinoma is not

GERD and Barrett's

What are the most common mutations involved in esophageal cancer? Are they oncogenes or tumor suppressors

Oncogenes

-EGFR

-KRAS

-HER2

Tumor suppressors

-TP53

-CDKN2A (decrease P16)

Where does squamous cell carcinoma typically develop in the esophagus? Is it related to reflux disease?

SCC typically develops in the upper esophagus

NOT related to reflux disease

What are the most common causes of squamous cell carcinoma of the esophagus

alcohol

tobacco

hot food or drink

esophageal chemical injury (tide pods)

What increases the risk of squamous cell carcinoma of the esophagus

Plummer Vinson Syndrome

Esophageal webs

Achalasia

The following describes what

-presence of keratin pearls

-prominent desmosomes (intracellular bridges)

Squamous cell carcinoma

Adenocarcinoma is derived from what type of tissue? Where does it develop in the esophagus? What pre-existing condition causes it to develop?

Derived from glandular tissue

Develops in the lower 1/3 of the esophagus

Barrett's esophagus is a pre-existing condition that disposes a person for it

What is the most common type of esophageal cancer in the West

Adenocarcinoma

Conditions of the muscularis externa typically present as

dysphagia

Diagnosis of conditions affecting the muscularis externa of the esophagus are based on

manometry findings of LES pressure

imaging

What is esophageal achalasia

failure of the LES to relax due to damage of AUERBACH's plexus (found between inner circular and outer longitudinal layer)

Neuronal loss, resulting in esophageal achalasia can be due to (2 things)

Chagas disease

-trypanosoma cruzi transmitted by the kissing bug

Paraneoplastic tumor effect

-i.e. lung cancer

LES has ________ peristalsis and LES pressure is _________

absent, high

What is the typical presentation of achalasia

solids and liquids dysphagia

halitosis (bad breath)

On a barium swallow, what would be the diagnostic sign of achalasia

"bird beak" sign - buzz word

Achalasia increases the risk of what cancer

squamous cell carcinoma of the esophagus

What is diffuse esophageal spasm (stenosis)

uncoordinated spontaneous contraction of the esophageal musculature

On a barium swallow, what would be a diagnostic sign of diffuse esophageal spasm

esophagus looks like a "corkscrew"

LES pressure is ______ in diffuse esophageal spasm

low/normal

Patients typically present with _______ and ________ in diffuse esophageal spasm

dysphagia and angina-like chest pain

What is sclerodermal esophageal dysmotility

due to smooth muscle atrophy of the middle and lower 1/3 of the esophagus.

LES pressure is ______ in sclerodermal esophageal dysmotility

low

What can cause sclerodermal esophageal dysmotility

it can be part of CREST syndrome caused by antibodies to chromosomal centromeres

CREST

-systemic sclerosis

-Reynaud's phenomenon

-Sclerodactyly

-Telangiectasia

What are the differences in LES pressure of the following disorders

-Sclerodermal esophageal dysmotility

-Diffuse esophageal spasm (stenosis)

-Esophageal achalasia

-Sclerodermal esophageal dysmotility = low

-Diffuse esophageal spasm (stenosis) = low

-Esophageal achalasia = high