Pediatric emergency medicine trisk 1029

FIGURE 124.14 This 2-month-old girl was well until 4 days before admission. She developed
congestion and an apparent upper respiratory tract infection. She slowly developed increasing
dyspnea and was admitted in acute respiratory distress. A chest radiograph revealed a high left
diaphragmatic eventration with a significant mediastinal shift to the right.

Current Evidence
Tumors of the chest wall are rare in children and may occur at any age from
infancy to late adolescence. More than half of these lesions are malignant, but
there are a host of benign causes as well. Types of benign tumors include


lipoblastoma, mesenchymoma, mesenchymal hamartoma, aneurysmal bone cysts,
chondroma, lipoid histiocytosis, osteochondroma, osteoid chondroma,
lymphangioma or hemangioma, and infectious processes such as tuberculosis and
actinomycosis. Malignant tumors are comprised of a variety of histologic types
and may be either primary or secondary. The most common are chondrosarcoma,
Ewing sarcoma/primitive neuroectodermal tumors (PNETs), fibrosarcoma,
osteosarcoma, and rhabdomyoscarcoma. Many malignant tumors may be present
at birth and have been identified early in the first year of life.

Goals of Treatment
Although tumors of the chest wall rarely present with symptoms requiring truly
emergent intervention, large masses or those with associated effusion may cause
respiratory symptoms or significant pain. Timely diagnosis of a chest wall mass is
of great value as a significant percentage of these tumors are malignant. Patients
in whom such a lesion is discovered in the ED will benefit from prompt
characterization of the mass and involvement of appropriate subspecialty
services, such as the oncology and pediatric surgery specialists.

Clinical Considerations
Clinical Recognition

Benign tumors of the chest wall are usually asymptomatic until trauma or fracture
brings them to attention. Malignancy may be signaled by a rapid increase in size,
pain, tenderness, or local inflammation. Pleural or pericardial effusions may be
present, causing dyspnea and tamponade, respectively, if sufficiently large.
Physical examination may reveal chest wall fullness or a mass, and large lesions
or effusions may cause diminished breath sounds on the affected side. Chest
radiographs may show pleural effusion and a peripheral mass, the depth and
extent of which is better demonstrated by CT scan.


FIGURE 124.15 A: A 13-year-old girl developed first right-sided and then left-sided epigastric
pain with retching but little or no vomitus. She had grunting respirations. A radiograph revealed
a large air- and fluid-filled mass in the left side of the lower chest. B: As shown in the diagram,
a nasogastric tube would not pass into the stomach.

FIGURE 124.16 Malignant chest wall tumors in children. Most common lesions and their
usual sites of origin are shown.


The site of the lesion may suggest certain diagnoses (Fig. 124.16 ). Ewing
tumor typically involves the lateral aspects of the ribs. Chondrosarcoma typically
involves the costal cartilages between the sternum and the distal rib end. The
sternum is a favored site for anaplastic sarcomas. These last two tumors may
extend into the thoracic cavity, as well as outside the bony thorax.
Management
If the clinical and radiologic picture clearly indicates a benign, self-limited
process, observation may be appropriate. However, if there is concern that the
lesion is not benign, even a small chest mass in a child should be considered
malignant and biopsy is appropriate.
Initial management of patients presenting with respiratory distress includes

supplemental oxygen administration, evaluation for pleural and pericardial
effusions with aspiration or tube thoracostomy drainage if present, and pain
management if clinically indicated. Radiographic evaluation should include a CT
scan of the pertinent area and a metastatic bone survey. If a malignant process is
suspected, oncology and surgical consultations are warranted.
Multimodal, coordinated treatment is frequently required involving surgical
resection, chemotherapy, and radiotherapy. Initial biopsy should be done using a
core needle technique or a limited open approach, with care to place and orient
the incision so as not to compromise the subsequent resection and chest wall
reconstruction. Preoperative chemotherapy and radiotherapy may be useful to
shrink selected lesions. Resection of the tumor and of subsequent recurrences
have resulted in disease-free survivals of 15 years or more. Extensive chest wall
resections may result in thoracic instability and paradoxical chest wall motion.
Technical advances have included the use of rigid materials such as mesh and
methylmethacrylate, and together with improvements in surgical technique and
postoperative care, significant resections including sternectomy or vertebrectomy
can be done safely with excellent preservation of chest contour and respiratory
function.
Suggested Readings and Key References
General
Coran AG, Adzick NS, Krummel TM, et al., eds. Thorax section. In: Pediatric
Surgery. 7th ed. Philadelphia, PA: Elsevier Saunders; 2012;771–960.
Tracheal Obstruction