Skip to content

Cancer Health Center

Font Size

Unusual Cancers of Childhood Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Thoracic Cancers

continued...

Prognosis

Bronchial tumors of all histologic types are associated with an excellent prognosis in children, even in the presence of local invasion.[29,30]

Clinical presentation and diagnostic evaluation

The presenting symptoms of a cough, recurrent pneumonitis, and hemoptysis are usually due to an incomplete bronchial obstruction. Because of difficulties in diagnosis, symptoms are frequently present for months, and, occasionally, children with wheezing have been treated for asthma with delays in diagnosis as long as 4 to 5 years.[31]

Metastatic lesions are reported in approximately 6% of carcinoid tumors, and recurrences are reported in 2% of cases. Atypical carcinoid tumors are rare but more aggressive with 50% of patients presenting with metastatic disease at diagnosis.[19,32] There is a single report of a child with a carcinoid tumor and metastatic disease who developed the classic carcinoid syndrome.[33] Octreotide nuclear scans may demonstrate uptake of radioactivity by the tumor or lymph nodes, suggesting metastatic spread.

Treatment

The management of bronchial tumors is somewhat controversial because bronchial tumors are usually visible endoscopically. Biopsy of these lesions may be hazardous because of hemorrhage, and endoscopic resection is not recommended. Bronchography or computed tomography scan may be helpful to determine the degree of bronchiectasis distal to the obstruction since the degree of pulmonary destruction may influence surgical therapy.[34]

Conservative pulmonary resection, including sleeve segmental resection, when feasible, with the removal of the involved lymphatics, is the treatment of choice.[35,36] Adenoid cystic carcinomas (cylindroma) have a tendency to spread submucosally, and late local recurrence or dissemination has been reported. In addition to en bloc resection with hilar lymphadenectomy, a frozen section examination of the bronchial margins should be performed in children with this lesion. Neither chemotherapy nor radiation therapy is indicated for bronchial tumors, unless evidence of metastasis is documented.

Pleuropulmonary Blastoma

Types of pleuropulmonary blastoma

Pleuropulmonary blastoma is a rare and highly aggressive pulmonary malignancy in children. Pleuropulmonary blastoma appears to progress through the following stages:

  • Type I: A purely lung cystic neoplasm with subtle malignant changes that typically occurs in the first 2 years of life and has a good prognosis. However, there have been reports of Type I transitioning directly to Type III.[37,38]
  • Type II: A cystic and solid neoplasm. Cerebral metastasis may occur in 11% of patients.[39]
  • Type III: A purely solid neoplasm.[40,41] Cerebral metastasis occurs in up to 50% of patients with Type III tumors.[39]
1|2|3|4|5|6|7|8
Next Article:

Today on WebMD

Colorectal cancer cells
A common one in both men and women.
Lung cancer xray
See it in pictures, plus read the facts.
 
sauteed cherry tomatoes
Fight cancer one plate at a time.
Ovarian cancer illustration
Do you know the symptoms?
 
Jennifer Goodman Linn self-portrait
Blog
what is your cancer risk
HEALTH CHECK
 
colorectal cancer treatment advances
Video
breast cancer overview slideshow
SLIDESHOW
 
prostate cancer overview
SLIDESHOW
lung cancer overview slideshow
SLIDESHOW
 
ovarian cancer overview slideshow
SLIDESHOW
Actor Michael Douglas
Article