extremely rare in children and often presents with advanced stage disease LCH, particularly in children younger than year, can present with GI involvement manifested by formula intolerance or occult or overt lower GI blood loss Other signs and symptoms of LCH are usually present (see section on “Histiocytic Diseases”) GISTs tend to occur in older children and adolescents and often involve the stomach and upper GI tract This tends to be a slow-paced disease and may present with vague GI symptoms and/or evidence of upper GI bleeding Mesenteric adenopathy alone should not raise the suspicion of malignancy since a reactive process is far more likely than a malignancy in children Mesenteric adenopathy can be associated with most of the diseases above but there is usually evidence of other abnormality on imaging Massive adenopathy can also occur in three extremely rare nonmalignant conditions: sinus histiocytosis with massive lymphadenopathy (SHML), Castleman disease, and Kikuchi disease As above, severe GI bleeding or complete GI obstruction requires rapid assessment and intervention (see Chapters 33 Gastrointestinal Bleeding and 91 Gastrointestinal Emergencies ) In most patients, however, the evaluation can proceed at a more measured pace The history and physical examination should focus on the specific findings noted above that can lead to the suspicion of a GIbased malignancy Laboratory evaluation should include a CBC to look for evidence of blood loss, and baseline hepatic and renal function If initial evaluation suggests advanced lymphoma, a full metabolic assessment as in the section on “Leukemia” should be completed urgently Diagnostic imaging should be performed based on the findings and suspected diagnoses An abdominal x-ray may reveal abnormalities of the bowel gas pattern suggestive of ascites or a mass Ultrasound can be helpful to assess the likely organ of origin of a palpable abdominal mass but rarely is sufficient to establish a diagnosis Ultrasound findings consistent with lymphoma can include bowel wall thickening or intussusception If a primary GI malignancy is suspected, a CT scan with both intravenous and oral contrast should be performed after establishing that renal function is adequate for intravenous contrast In otherwise stable patients, this imaging can be performed subsequent to the ED evaluation Patients with evidence of high cell turnover on metabolic assessment and a suspected diagnosis of advanced Burkitt lymphoma must be admitted to a center capable of performing pediatric renal dialysis Other patients with a suspected GI malignancy should be admitted or referred to a center with pediatric oncology expertise NEUROBLASTOMA Goals of Treatment Neuroblastoma has a wide range of clinical presentations, depending on tumor location as well as duration of symptoms A high index of suspicion should be maintained in diagnosing these malignancies CLINICAL PEARL AND PITFALLS The periorbital ecchymoses of neuroblastoma and the subcutaneous pigmented lesions of stage 4S neuroblastoma seen in infants can both be mistaken for child abuse Current Evidence Neuroblastoma is derived from neural crest cells that exist within the adrenal medulla and along the sympathetic chain It is the most common solid tumor of childhood outside the CNS, accounting for 7% to 10% of pediatric tumors overall With an incidence of per 7,000 live births, neuroblastoma preferentially affects very young children; 50% of cases are diagnosed by age and 90% by age In approximately two-thirds of cases, the primary tumor is in the abdomen, specifically in the adrenal gland Clinical Considerations Clinical Recognition Neuroblastoma presentation can be clinically variable, depending on location An abdominal mass may be present, and depending on its size may be initially asymptomatic Larger masses may cause GI dysmotility, such as constipation or bowel obstruction, as well as inability to tolerate oral intake and secondary cachexia Bone pain may develop if the tumor involves osseous sites Marrow replacement can cause signs or symptoms of anemia, thrombocytopenia, or neutropenia Large masses of the chest or abdomen may impair pulmonary function and cause respiratory distress Potentially life-threatening or organ-threatening complications of neuroblastoma include the development of SVC syndrome from a mass in the posterior mediastinum that extends anteriorly or cord compression from tumor growing through the neural foramina into the spinal canal See sections on “Tumors of the Thorax” and “Tumors in and Around the Spinal Cord” for the approach to these complications However, at times neuroblastoma presents in a healthy, wellappearing child with an abdominal mass incidentally detected Other unique presentations include ipsilateral Horner syndrome (ptosis, miosis, and anhidrosis) from involvement of the cervical sympathetic ganglia, “raccoon eyes” from periorbital bone and soft tissue involvement causing proptosis and ecchymosis, and opsoclonus myoclonus This latter is a paraneoplastic syndrome characterized by “dancing eyes and dancing feet” and is associated with a favorable cancer prognosis but a poor neurocognitive outcome Neuroblastoma can secrete catecholamines that cause hypertension and vasoactive intestinal peptide (VIP) that causes secretory diarrhea Subcutaneous nodules can occur Clinical Assessment Initial assessment of a child with possible neuroblastoma should include an assessment of the patient’s airway, breathing, and circulation, followed by a complete history and physical examination that focuses on the potential signs and symptoms above Given the risk of cord compression from a retromediastinal or retroperitoneal tumor, all patients should have a thorough neurologic examination including percussing the vertebral bodies, with emergent imaging should neurologic deficits be detected The patient’s blood pressure should be measured and carefully matched against norms for age Signs and symptoms of pain should also be explored to localize potential tumor masses and pain should be treated as needed Laboratory evaluation should include CBC, liver function testing, renal function testing (BUN and creatinine), and urine catecholamines If neuroblastoma is suspected and the disease burden is high, TLS may develop (see “Leukemia” section) If the CBC shows evidence of marrow replacement, platelet and packed red blood cell transfusions may be needed (see Table 98.7 ) A plain film of the chest or abdomen may be useful for detecting calcifications Abdominal ultrasound may help define the location of a mass and its relationship to other structures CT scans should include the suspected site of the primary tumor, the surrounding lymph node groups, and the liver (a common site of metastasis) Management If a thorough evaluation and initial management finds no life-threatening or organthreatening problems, no uncontrolled pain, and no evidence of severe systemic illness, discharge to the care of a pediatric oncologist may be possible Otherwise admission is recommended TUMORS OF THE KIDNEY Goals of Treatment The most critical decision making and care in the ED is the differentiation of emergent from nonemergent Stable patients with newly diagnosed kidney tumors can be managed as outpatients with close subspecialty follow-up CLINICAL PEARL AND PITFALLS Most patients with Wilms tumor have an asymptomatic abdominal mass, but potentially life-threatening conditions include hypertension, gross hematuria, and an acute abdomen Current Evidence There are several different causes of renal tumors in children, some malignant and some benign Wilms tumor, the most common pediatric renal tumor, arises from embryonic renal blastemal cells (see Fig 98.2 ) Overgrowth syndromes such as Beckwith–Wiedemann, Soto syndrome, hemihypertrophy, and Denys–Drash syndrome predispose to embryonal tumors such as Wilms’ There are 500 cases of Wilms’ diagnosed annually in United States with peak incidence between the ages of and Eighty percent of cases are diagnosed by age ... chain It is the most common solid tumor of childhood outside the CNS, accounting for 7% to 10% of pediatric tumors overall With an incidence of per 7,000 live births, neuroblastoma preferentially... problems, no uncontrolled pain, and no evidence of severe systemic illness, discharge to the care of a pediatric oncologist may be possible Otherwise admission is recommended TUMORS OF THE KIDNEY Goals... causes of renal tumors in children, some malignant and some benign Wilms tumor, the most common pediatric renal tumor, arises from embryonic renal blastemal cells (see Fig 98.2 ) Overgrowth syndromes