A 17-Year-Old Male with a Small Bowel Neuroendocrine Tumor (NET): Flushing Differential Diagnosis
Monday, March 7, 2016
South Exhibit Hall H (Convention Center)
Maria Alejandra Forero Molina, Elizabeth Garcia-Gomez, MD, Deyanira Gonzalez-Devia, MD, Rafael García Duperly, MD, Alonso Vera Torres, MD
Rationale: NETs are heterogeneous neoplasms originated from cells with a secretory function, extremely rare in children. Small bowel NETs are related to serotonin hypersecretion causing flushing, diarrhea, abdominal pain, bronchoconstriction and heart involvement (carcinoid syndrome); unusual symptoms in kids. We present a 17-year-old male with this pathology. 

Methods: Clinical, 5-hydroxyindoleacetic acid (5-HIIA), CT-scan, biopsies

Results: The patient was remitted to the allergist because he presented evanescent non-pruriginous erythematous lesions after eating. He described other symptoms: conjunctival injection, warmth and diaphoresis after the lesions disappeared. Other circumstances like strong emotions, standing and valsalva triggered the symptoms. He denied abdominal pain, diarrhea, cough or wheezing. We triggered the symptoms by asking him to eat; after 9minutes he presented an evanescent flushing in the face, trunk and extremities for 12minutes. Given the characteristics of the lesions, we discarded the diagnosis of urticaria or food allergy. We asked for 24hours urinary 5-HIIA: 42,6mg (<10mg/24hours). The CT showed thickening of the distal ileum and multiple lesions on both hepatic lobules. Colonoscopy revealed a prominent ileocecal valve with a mammillated and eroded lesion. Hepatic and intestinal biopsies: well-differentiated grade 2 primary NET of the ileocecal valve with hepatic metastasis. He started ocreotide and underwent a wide hepatectomy and right hemicolectomy.

Conclusions: This is one of the few reports of carcinoid syndrome in children associated to high 5-HIIA levels. Additionally the patient had an atypical presentation: flushing involved the extremities, very advanced disease and was very young. It shows a differential diagnosis of flushing that should be considered by allergists.