FIGURE 124.14 This 2-month-old girl was well until 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 ... 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... 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