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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
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