Clinical Recognition Intraorbital tumors may involve any of the tissues contained by the orbit including bone, muscle, soft tissue, and the globe itself Masses in these regions have a wide differential diagnosis, including infections (periorbital and orbital cellulitis), orbital myositis, benign germ cell tumors, or cystic lesions such as a dermoid cyst Retinoblastoma is the most common intraocular malignancy in children It occurs in in 23,000 births and is usually diagnosed by age Two-thirds of patients with retinoblastoma present with a white pupil (leukocoria) noted by parents This is the tumor as seen through the vitreous The most common malignancies affecting the bony orbit are LCH and neuroblastoma Presenting symptoms usually include proptosis and strabismus Vascular tumors including capillary hemangiomas of the orbit may present with red or purple nodular lid lesions or proptosis Masses in the aerodigestive tract may be benign, infectious, or reactive in etiology Regardless of the tissue of origin, these masses usually present with symptoms related to their anatomic position Oropharyngeal tumors such as with Burkitt lymphoma can cause snoring and obstructive sleep apnea as well as chronic otitis media and unilateral tonsillar hypertrophy Gingival hypertrophy may be a sign of a monocytic leukemia LCH or Burkitt lymphoma of the mandible can present with loose teeth Rhabdomyosarcoma of the salivary or parotid gland often presents with pain or a facial mass Malignant tumors of the nose, nasopharynx, and sinuses can present with purulent or bloody rhinorrhea, epistaxis, or sinusitis Nasopharyngeal carcinoma tends to have a long duration of symptoms before diagnosis because symptoms are rarely specific Malignant tumors of the sinuses and base of the skull can present with cranial neuropathies such as deviation of the eyes due to compression of the cranial nerves by the tumor Rhabdomyosarcoma of the middle ear can present with persistent otitis, pain, or cranial neuropathy The external ear canal can be affected by LCH leading to otorrhea and otitis externa Neck masses due to benign congenital anomalies such as branchial cleft cysts or cystic hygromas may grow suddenly as a result of infection or bleeding Lymphadenopathy in children is common and usually benign It is most commonly either reactive or infectious in etiology Bilateral nodes may be associated with viral infections such as EBV or cytomegalovirus (CMV) Unilateral lymphadenopathy or lymphadenitis, especially in infants and young children, may be associated with Staphylococcus aureus or group A streptococcus infections Even lymph node enlargement with a chronic time course is still most likely infectious (e.g., mycobacteria, cat-scratch disease, toxoplasma) Lymph nodes can appear large even without infection or malignancy, as observed in Castlemans, Kikuchis, and Rosai– Dorfman syndromes Enlarged lymph nodes in the neck due to malignancy can be from lymphoma or leukemia or can be because of metastasis from an adjacent solid tumor such as rhabdomyosarcoma, neuroblastoma, or nasopharyngeal carcinoma It can be difficult to distinguish the primary tumor mass from a lymph node in these circumstances Clinical Assessment Children with neck tumors, regardless of etiology, must be evaluated for impact on the airway, breathing, and circulation Clinicians should explicitly consider the following: Does the mass compress the airway? Does the patient experience respiratory distress or is there a compromise to breathing? Does the mass interfere with circulation of the head and neck leading to SVC syndrome (see “Tumors of the Thorax” section)? Does the tumor threaten to compress the cervical spine (see “Tumors in and Around the Spinal Cord” section)? Following this assessment, a careful history should address the duration the mass has been present and its rate of growth, any recent infectious illnesses, the patient’s immunization status, cat exposure, medications, and the presence of systemic systems Physical examination should assess for other masses or lymphadenopathy in the body In evaluating nodes of the neck, reactive nodes are often small, mobile, and soft, or while infected nodes may be enlarged, red, and tender Characteristics that make malignancy more likely include nontender masses, very firm/hard texture, diameter more than cm, adherence to other structures, irregular margins, and absence of signs or symptoms of infection Management Often radiographic imaging and laboratory evaluation is not needed However, in the case of suspected leukemia or lymphoma, laboratory studies should be obtained to assess for hyperleukocytosis, cytopenias, and TLS (see preceding discussion on leukemia) Radiographic imaging should be pursued if more information is needed about the tumor’s position in relationship to the patient’s airway and other vital structures of the head and neck When indicated, this is best accomplished with a CT scan In addition, a chest x-ray should be obtained to explore whether the disease could include a mass in the anterior mediastinum Laboratory evaluation should include a CBC with differential, ESR and LDH (which may be elevated in certain lymphomas), testing for any relevant infectious etiologies, and consideration of thyroid function testing Children with leukocoria should have an urgent ophthalmologic examination to help differentiate retinoblastoma from other possible etiologies such as congenital cataract, coloboma, idiopathic retinal detachment, and others Direct extension via the optic nerve into the meninges and spinal fluid is a possible but unlikely complication of the tumor Presentations are usually local and therefore cured by enucleation, but systemic chemotherapy, intra-arterial (ophthalmic artery) chemotherapy, cryotherapy, laser therapy, and insertion of radioactive plaques are all being explored to preserve vision Management of retinoblastoma hinges on the probability of useful vision in the affected eye Ophthalmology should be consulted early to determine if the patient’s visual acuity has already been affected by the mass and to plan the urgency of examination under anesthesia Management of intraorbital tumors may be possible on an outpatient basis, in conjunction with an experienced pediatric ophthalmologist, if the mass is unlikely to affect vision quickly or if vision is already profoundly impaired in the affected eye Management of other head and neck tumors can sometimes occur on an outpatient basis, in conjunction with a pediatric oncologist and a specialist, such as an oral surgeon or otorhinolaryngologist with expertise in the anatomic region of the tumor However, specific symptoms such as uncontrolled pain, difficulty maintaining hydration, TLS, or any evolving threat to the airway require inpatient management TUMORS OF THE THORAX Goals of Treatment The most critical decision making and care in the ED is the differentiation of emergent from nonemergent tumors of the thorax This difference is frequently driven by tumor location (see Fig 124.11 in Thoracic Emergencies chapter) CLINICAL PEARL AND PITFALLS Children with anterior mediastinal mass must be managed with the utmost caution Prevention of respiratory failure is critical, as these masses may be located below the carina, rendering even intubation ineffective Current Evidence Thoracic tumors can be caused by a number of childhood cancers While hematologic malignancies are common, embryonal neoplasms such as neuroblastoma, sarcomas such as primitive neuroectodermal tumor (PNET), and carcinomas can also present in the chest In general, there are no specific predisposing conditions or factors The anterior mediastinum is the most common location of a thoracic tumor in children The “4 Ts” of the anterior mediastinal tumors include “terrible lymphoma,” “teratoma,” “thymoma,” and “thyroid carcinoma.” The latter three are rare Nonmalignant conditions with AMM include adenopathy associated with infection, sarcoid, and normal thymus Common lymphomas ( Table 98.5 ) in the anterior mediastinum include T-cell lymphoblastic lymphoma (or T-cell ALL with an associated AMM), Hodgkin lymphoma, and diffuse B-cell large cell lymphoma Lymphoma can also occur in the middle mediastinum, which can also be the site of masses associated with pulmonary sequestration and other developmental anomalies “Teratoma” of the mediastinum includes benign and malignant germ cell tumors Posterior mediastinal masses include neuroblastoma and other neurogenic tumors such as malignant peripheral nerve sheath tumors (especially in patients with neurofibromatosis, type 1), or benign lesions such as schwannoma Primary lung tumors are vanishingly rare in childhood but presentation of lung metastasis at diagnosis or relapse is not uncommon Many pediatric sarcomas, some lymphomas, germ cell tumors, Wilms tumor, and rarely neuroblastoma can present or recur with lung metastasis These typically involve multiple small or large lung nodules in the pulmonary parenchyma or are pleural based Askin tumor is a unique PNET chest wall tumor that occurs in children and young adults Clinical Considerations Clinical Recognition Tumors in the anterior and middle mediastinum often present with respiratory symptoms ranging from mild cough to severe respiratory distress ( Fig 98.1 ) These tumors can compress the great vessels and cause SVC syndrome When asymptomatic they may be identified during evaluation for nonspecific systemic symptoms or even discovered on a chest radiograph performed for another reason In contrast, posterior mediastinal masses are frequently identified on a chest radiograph performed for another reason They may, however, cause local pain from nerve root involvement and/or cord compression (see “Tumors in and Around the Spinal Cord” section) The initial complaints associated with pulmonary metastasis may include respiratory insufficiency, postobstructive infection, foreign body–type symptoms, or hemoptysis Pulmonary nodules may be discovered on a chest radiograph Pulmonary effusions can be the presenting sign of childhood cancer Effusions can be caused by malignant cells in the pleural space or from obstruction of lymphatic drainage Effusions are common with AMM due to leukemia or lymphoma and can also occur with posterior mediastinal neuroblastoma, lung metastasis, and chest wall tumors They may be symptomatic or asymptomatic The ... intraorbital tumors may be possible on an outpatient basis, in conjunction with an experienced pediatric ophthalmologist, if the mass is unlikely to affect vision quickly or if vision is already... of other head and neck tumors can sometimes occur on an outpatient basis, in conjunction with a pediatric oncologist and a specialist, such as an oral surgeon or otorhinolaryngologist with expertise... rare in childhood but presentation of lung metastasis at diagnosis or relapse is not uncommon Many pediatric sarcomas, some lymphomas, germ cell tumors, Wilms tumor, and rarely neuroblastoma can