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Defending Diagnoses

Defending Diagnoses. Carcinoid Tumor (11): Jack Mbabuike Colon Adenocarcinoma (3): Joshua Gordon Basal Cell Carcinoma (1): Owen Dubowy Hepatocellular Carcinoma (1): Amer Assal Other Diagnoses: Pheochromocytoma VIPoma Gastric Cancer Gastrinoma. Liver Biopsy.

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Defending Diagnoses

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  1. Defending Diagnoses • Carcinoid Tumor (11): Jack Mbabuike • Colon Adenocarcinoma (3): Joshua Gordon • Basal Cell Carcinoma (1): Owen Dubowy • Hepatocellular Carcinoma (1): Amer Assal • Other Diagnoses: Pheochromocytoma VIPoma Gastric Cancer Gastrinoma

  2. Liver Biopsy

  3. Liver Biopsy Chromogranin

  4. Colon Biopsy

  5. Colon Biopsy Chromogranin

  6. Colon Biopsy Synatophysin

  7. Final Diagnosis Carcinoid syndrome secondary to poorly differentiated neuroendocrine carcinoma of the colon with liver metastasis

  8. Neuroendocrine Cancer • Neuroendocrine cells are widely distributed throughout the body • GI tract and pancreas have the largest component of neuroendocrine cells than any other organ system • Nomenclature of GI neuroendocrine tumors is confusing – WHO standardized in 2005

  9. Classification of NE Cancers of the Colon

  10. Classification of Neuroendocrine Cancers of the Colon • Well-Differentiated Tumors – Carcinoid • Well-Differentiated Endocrine Carcinoma – Malignant Carcinoid • Poorly Differentiated Endocrine Carcinoma – our patient • Mixed Exocrine-Endocrine Carcinoma

  11. Definition of Carcinoid Syndrome • Constellation of symptoms produced by the actions of neuroendocrine tumor secretory products

  12. Prevalence of Colon Neuroendocrine Tumors • Likely underestimated due to need for special additional staining • Large retrospective series of resected colorectal tumors found: - 4% of tumors had partial neuroendocrine differentiation - 1% complete neuroendocrine differentiation

  13. Pathophysiology of Colon Neuroendocrine Tumors • Poorly understood, risk factors are not known • Some suggestion of hereditary component • Some overlap with the genetic model of tumorigenesis of colonic adenocarcinoma

  14. Pathogenesis of Mr. L’s Disease

  15. abdominal pain change in bowel habits melena/hematochezia anemia, weakness, weight loss symptoms of carcinoid syndrome rare Clinical Presentation of Colon Neuroendocrine Cancer

  16. Diagnosis of Neuroendocrine Colon Cancer • Colonoscopy with biopsy • Immunohistochemical stains for chromogranin and synaptophysin

  17. Treatment of Neuroendocrine Colon Carcinoma • Surgery if local disease – curative • Prognosis is poor for metastatic disease • Chemo is similar to small cell lung cancer - Cisplatin and Etoposide - Irenotecan • Treatment of symptoms - Sandostatin

  18. Follow-up • Patient had progression of disease after 4 cycles of cisplatin and etoposide, bone mets developed • Irenotectan initiated with continued progression • Sandostatin initiated for worsening diarrhea and flushing • Patient transferred to Bronx VA for palliative radiation therapy • He passed away last week

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