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Intestinal Malignant Tumors in Children

Intestinal Malignant Tumors in Children. Juan I. Camps MD, MBA Pediatric Surgeon Palmetto Health Children’s Hospital Columbia, SC. Disclosure and Learning Objectives. Discuss Clinical features, surgical options and prognosis of intestinal malignant tumors in children.

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Intestinal Malignant Tumors in Children

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  1. Intestinal Malignant Tumorsin Children Juan I. Camps MD, MBA Pediatric Surgeon Palmetto Health Children’s Hospital Columbia, SC

  2. Disclosure and Learning Objectives Discuss Clinical features, surgical options and prognosis of intestinal malignant tumors in children. Dr. Camps has nothing to disclose with regards to commercial support.

  3. Adenocarcinoma Incidence: 1,3 -2 cases per million lives Location: Colon and Rectum Histology: Adenocarcinoma Genetics: APC gene, NF-1, Cr 18. Timing: second decade of live (15-19y/o)

  4. Predisposing factors Diet high in fat and low in fiber Familial Polyposis syndrome Hereditary non Polyposis syndromes Inflammatory bowel disease

  5. Predisposing factors Urinary diversion: ureterosigmoidostomy Chronic parasitic infection Radiation therapy Exposure to environmental contamination

  6. Genetics ADENOMATOUS POLYPOSIS FAP: APC gene mutation Lynch Syndrome: MSH2, MLH1, MSH6 Li-Fraumeni Syndrome: TP53 Turcot Syndrome: APC, MLH1

  7. Genetics HAMARTOMATOUS POLYPS Cowden syndrome: PTEN gene Juvenile Polyposis Syndrome: BMPR1A Peutz-Jeghers Syndrome: STK11

  8. Prevalence Sporadic form: 75% Predisposing factor: 25% Polyposis syndromes Non-polyposis Ulcerative colitis Crohn’s Disease

  9. Polyposis Disease Incidence: 1% Timing: preschool and school aged children Juvenile Polyps: 80% Lymphoid Polyps: 15% Adenomatous polyps: 3%

  10. Polyposis Disease Benign lesions Mucosa: Hamartomas Submucosa: Lymphoid hyperplasia Adenomatous polyps: potential malignant mucosal transformation

  11. Polyposis Disease Located only Intestines: Juvenile Lymphoid Familial adenomatous Polyps Syndromes affecting Intestines: Peutz-Jegher’s syndrome Gardner’s syndrome Turcot’s syndrome

  12. Isolated Juvenile Polyps Most common: 1% preschool 3-5 years old Polyps Hamartomas 80% Normal colonic tissue with abnormal structure most likely related to inflammation Solitary 50% of cases Distribution location 50% recto sigmoid Presentation bleeding Treatment; >5 polyps= juvenile polyposis Colonoscopy if more 5 polyps all removed

  13. Juvenile PolyposisSyndrome Diffuse Juvenile polyposis of the infancy Bad prognosis Diffuse juvenile polyposis Found in stomach, distal colon an rectum Diffuse presentation, excision Endoscopic resection p\or partial bowel resection Juvenile polyposis coli >50-100 polyps Located distal rectum High incidence of malignancy

  14. Familial AdenomatousPolyposis Polyps >100 adenomatous polyps Incidence 1/6000-12000 Mutation in the apc gene chr 5q Presentation of polyps in the stomach 50 % High incidence of malignancy

  15. Syndromes Peutz-Jeghers Syndrome Skin color changes Hamartomas polyps Gardner’s Syndrome: Adenomatous polyps Soft tissue and bone tumors Turcot’s Syndrome: Familial adenomatous polyps Central nervous system tumors

  16. Peutz-JeghersSyndrome Association Intestinal polyps Pigmented spots mouth, hands, feet Polyps found everywhere small intestine 50% Stomach duodenum 30% Distal colon rectum 15% Hystologyhamartomas and adenomas Treatment colonoscopy polyp <1.5 remove >1.5 cm surgical resection

  17. Radiation Cancer treated with radiation increase incidence of secondary colorectal cancer Might induce a p53 gene mutation

  18. Ulcerative colitis 20 times higher normal population After 10 year incidence increase 1-2 % per year Occur at younger age, Multi locations

  19. Crohn’sDisease Incidence 20 percent higher than that in the normal population. Random biopsies and surveillance

  20. Hereditary Non-PolyposisColon Cancer Autosomal dominant inheritance 5 x more prevalence than familial polyposis-related colon cancer Kindred with this type of colon cancer commonly have proximal colon lesions and developed cancer at a younger age.

  21. Symptoms Abdominal pain Nausea and vomiting 40-70 % Change bowel habits Constipation Slow growth

  22. Physical Findings Abdominal distension Tenderness. Mass. Blood in the stool >50% Weight loss 20-30%

  23. Diagnosis Surgical abdomen Bowel obstruction. Barium enema Colonoscopy Abdominal Scan

  24. Pathologic FeaturesCompared to Adults Dukes’ stage C-D 80% Mucinous type adenocarcinoma > 50% Adult 5 % mucinous Early metastasis and more aggressive, worst prognosis because delayed Dx, CEA levels poor indicative of treatment response Location most common right side

  25. Treatment Surveillance Peutz-Jeghers Juvenile polyposis Colectomy Familial polyposis Gardner’s syndrome Turcot’s syndrome

  26. Treatment Surgical oncology resection: Colon resection mesenteric and lymphatic dissection Ovaries and omentum frequently involved

  27. Summary Biology of colon carcinoma is different compared to adult intestinal cancer. Presentation, histology type, stage, prognosis are different.. Molecular genetics of colon carcinoma in children should lead to better understanding and better outcomes.

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