Benign Neoplasms & Congenital Lung Diseases

Question 1

nasal polyposis

Answer 1

*a finding associated with chronic rhinosinusitis
*polyps may be visible only with nasal endoscopy or CT scan
*present along with other conditions in some patients (asthma, aspirin-exacerbated respiratory disease [AERD])
*usually bilateral; if unilateral:
-look for allergic fungal rhinosinusitis, other sinonasal neoplasms, cystic fibrosis, ciliary dyskinesia

Question 2

conditions commonly associated with nasal polyposis

Answer 2

1. asthma
   -often strongly type 2-inflammation-high
2. aspirin-exacerbated respiratory disease (AERD)
   -triad: 1) nasal polyposis; 2) asthma; 3) NSAID sensitivity

Question 3

vocal cord polyps

Answer 3

*can be due to:
1) vocal overuse (very talkative people, singers, workers in a loud environment)
2) irritation (acid reflux, tobacco use)
3) trauma (long-term intubation)
4) infection (HPV)
*for all, symptoms include:
-reduced vocal endurance
-sensation of increased effort when talking or singing
*direct inspection is needed to diagnose

Question 4

respiratory papillomatosis

Answer 4

*due to infection with human papillomavirus serotypes 6 and 11
*can transform into squamous cell carcinoma
*easily transmissible when aerosolized
*can be aggressive, requiring repeat surgical removal

Question 5

which 2 HPV serotypes are associated with respiratory papillomatosis

Answer 5

HPV serotypes 6 and 11 ***

Question 6

hamartoma

Answer 6

*most common benign pulmonary lesion in adults who undergo surgical resection
*found in lung parenchyma and distal to trachea
*contain at least 2 benign mesenchymal elements (cartilage, adipose tissue, connective tissue, smooth muscle) with entrapped benign respiratory epithelium
*usually asymptomatic and found on imaging done for another reason
*endobronchial hamartoma is rare, and can cause obstructive pneumonia and cough
*FAT DENSITY ON CT IS VERY SPECIFIC FOR HAMARTOMA

Question 7

bronchial carcinoid

Answer 7

*a neuroendocrine neoplasm
*tend to be very round and spherical
*90% are “typical”; 10% are “atypical”:
-atypical has more mitotic figures and behaves a bit more aggressively
*good prognosis compared to large cell neuroendocrine carcinoma or small cell carcinoma
*can arise centrally and grow into the lumen of a large airway
*smooth, lobulated border and may bleed extensively when biopsied

Question 8

bronchial carcinoid histology

Answer 8

*“salt-and-pepper” finely granular chromatin, abundant cytoplasm

Question 9

tracheoesophageal fistula

Answer 9

*abnormal connection between the trachea and esophagus
*can be congenital or acquired
*presentation (congenital): newborn with respiratory distress and choking and/or vomiting after feeding
*treatment: surgery (congenital) or stenting (erosive)
*part of VACTERL syndrome (can occur by itself though)

Question 10

bronchogenic cyst

Answer 10

*abnormal budding of the foregut during early development
*commonly found in the anterior mediastinum or along the tracheobronchial tree
*unilocular, lined with ciliated columnar (respiratory) epithelium
*may connect to the tracheobronchial tree, but usually do not
*radiographically similar to an esophageal duplication cyst, which can also occur in the mediastinum and is lined with alimentary mucosa
*presentation: frequently an incidental radiological findings; sometimes presents with infection
*treatment: often not necessary; surgery if large or infected

Question 11

pulmonary sequestration

Answer 11

*congenital abnormality with lung tissue that both:
1. does not communicate with the tracheobronchial tree AND
2. has a SYSTEMIC, rather than pulmonary, arterial supply
*can be extralobar or intralobar

Question 12

extralobar pulmonary sequestration

Answer 12

*located outside pleura of lung (“accessory lobe”)
*commonly found at left lung base
*easier to resect than intralobar because it has its own pleura
*gross features: pyramidal structure
*age at diagnosis: 60% < 6 months
*arterial supply: systemic
*origin: congenital anomaly

Question 13

intralobar pulmonary sequestration

Answer 13

*located within the pleura of lung lobe
*commonly found in a lower lobe
*gross features: congested, hemorrhagic segment(s) of lobe
*age at diagnosis: 50% > 20 years
*arterial supply: systemic
*origin: congenital anomaly

Question 14

congenital pulmonary adenomatous malformation (CPAM)

Answer 14

*rare masses of MALDEVELOPED LUNG TISSUE (abnormal tissue types)
*often cystic, sometimes solid
*presentation: detected in utero or on routine chest imaging
*treatment: observation (in utero may resolve), surgery if large/enlarging

Question 15

immotile cilia syndrome

Answer 15

*rare disorder that frequently presents in childhood
*partial or complete PARALYSIS OF THE CILIA on the respiratory epithelium (upper and lower airways) due to DYNEIN OR MICROTUBULE DEFECTS
*chronic rhinosinusitis, otitis media with effusion
*daily, year-round productive cough due to impaired mucus flow
*chronic infection is common and bronchiectasis can occur
*often FERTILITY IMPAIRMENT
*sometimes, SITUS INVERSUS TOTALIS is present (Kartagener syndrome)
*treatment: supportive care, airway clearance techniques, preventive antibiotics

Question 16

congenital diaphragmatic hernia (CDH)

Answer 16

*failure of PLEUROPERITONEAL MEMBRANE TO FUSE
*division that should be present between thorax and abdomen is not fully formed, → herniation of abdominal organs into thorax
*results in pulmonary hypoplasia because compression of the lungs limits their growth
*more common on left side
*can be fatal