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extremely rare in children and often presents with advanced stage disease. LCH,
particularly in children younger than 1 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