Pulmonary Carcinoid Tumors and Other Primary Lung Tumors

Question 1

Prevalence of pulmonary carcinoid tumors

Answer 1

0.4-3.0% of resected lung cancers

Lungs 2nd most common site of origin for primary carcinoid tumors

Question 2

WHO classification of carcinoid tumors

Answer 2

Typical (< 2 mitotic figures/hpf, no necrosis)

Atypical (2-10 mitotic figures/hpf, tissue necrosis, or architectural disruption)

Question 3

Prevelence of typical vs atypical carcinoid tumors

Answer 3

Typical: 75-80% of tumors

Atypical: 15-20% of tumors

Question 4

According to WHO carcinoid tumors are part of the histologic spectrum of neuroendocrine tumors, including…

Answer 4

• Typical carcinoid (low grade)
• Atypical carcinoid (intermediate grade)
• Large cell neuroendcrine (high grade)
• SCLC (high grade)

Question 5

Demographic characteristics of carcinoid tumor presentation

Answer 5

Bimodal age distribution (peaks at 35 and 55 years of age)

Question 6

Patients older than ___ years have a higher incidence of atypical carcinoid tumors?

Answer 6

age = 50 years old

risk 25% vs 10% (50 years vs. <30 years)

Question 7

Presentation of pulmonary carcinoid

Answer 7

• Asymptomatic (1/3 pts)
• recurrent PNA
• cough
• hemoptysis

Question 8

Ectopic hormones secreted by carcinoid tumors

Answer 8

• 5-hydroxytryptophan
• ACTH
• vasopressin
• insulin

Question 9

% pts wtih Cushings syndrome with localized disease

Answer 9

5%

Question 10

Anatomic characteristics of carcinoid tumors

Answer 10

• Central location
  
  • Typical (70%)
  • Atypical (50%)
• Lobar or sublobar bronchus (75%)

Question 11

CT findings of carcinoid tumors

Answer 11

• Peripheral tumors:
  
  • smooth, round, homogenous appearance
• Central tumors:
  
  • obvious airway component, post-obstructive atelectasis or pneumonitis

Question 12

Other diagnostic studies used in w/u of carcinoid

Answer 12

• PET scan
• Octreotide scan
• Bronchoscopy (with biopsy)
  
  • central tumors
• CT guided FNA biopsy:
  
  • peripheral tumors

Question 13

Success of preoperative diagnosis of carcinoid

Answer 13

70-80%

Question 14

Most peripheral and central tumors without evidence of nodal disease

Answer 14

Typical carcinoid tumors

(no further eval needed prior to resection)

Question 15

Distribution of atypical carcinoid tumors with central location and cN1 or cN2 disease

Answer 15

Cenral location (50%)

Peripheral location (50-70%)

*need thourough staging prior to resection (including mediastinoscopy)

Question 16

Surgical treatment principles for carcinoid

Answer 16

• R0 resection (limited resection is acceptable)
• Medistinoscopy with LN bx shoud be performed:
  
  • Clinical nodal diseaes
  • Atypical carcinoid histology

Question 17

Indication for neoadjuvant/adjuvant chemotherapy for carcinoid

Answer 17

N2 disease

Question 18

Surgical option for carcinoid tumors in patients unable to tolerate parenchymal resection

Answer 18

Endobronchial resection

(negative margin important >> high chance of local recurrence)

Question 19

Definiton and characteristics of carcinoid tumorlets

Answer 19

Carcinoid tumors < 5 mm in diameter

• 10% of patients with other primary carcinoid tumor or other types of NSCLC
• should no be staged as metastatic disease
• treatment should be based on primary lesion

Question 20

TOC for metastaic or unresectable carcinoid

Answer 20

Chemotherapy

Reponse rates 20-40%

30% will progress while receiving chemotherapy

Question 21

Duration of postoperative followup for carcinoid

Answer 21

20 years due to indolent nature of disease

Question 22

Tumor of trachea or mainstem bronchus usually detected when symptomatic with significant obstructive component

Answer 22

Adenoid cystic carcinoma

Question 23

Submucosal extension beyond gross intraluminal tumor characteristic of these tumors

Answer 23

Adenoid cystic carcinoma

Intraoperative frozen section thus essential for R0 resection

Question 24

Other salivary gland-type tumors

Answer 24

Mucoepidermoid

Acinic cell carcinoma

Question 25

Most common benign lung tumors

Answer 25

• Hamartoma
• Inflammatory pseudotumor
• Sclerosing hemangioma
• Bronchogenic cyst
• Leiomyoma
• Adenoma
• Papilloma and soft tissue tumors