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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
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Liver BiopsyChromogranin
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Colon Biopsy
Chromogranin
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Colon Biopsy
Synatophysin
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Final Diagnosis
Carcinoid syndrome secondary to poorly differentiated
neuroendocrine carcinoma of the colon with liver metastasis
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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
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Classification of NE Cancers of the Colon
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Classification of Neuroendocrine Cancers of the
ColonI. Well-Differentiated Tumors – Carcinoid
II. Well-Differentiated Endocrine Carcinoma – Malignant Carcinoid
III. Poorly Differentiated Endocrine Carcinoma – our patient
IV. Mixed Exocrine-Endocrine Carcinoma
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Definition of Carcinoid Syndrome
• Constellation of symptoms produced by the actions of neuroendocrine tumor secretory products
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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
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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
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Pathogenesis of Mr. L’s Disease
Environmental Factors
Sequential Genetic Mutations
Colonic Neuroendocrine Cell Malignant Transformation
Tissue Invasion with Metastasis
Systemic Release of Neurohormonal
Products
Appetite, Weight Loss, and Fatigue
Hypovolemia
Hypokalemia and Alkalosis Diarrhea
Near Syncope Bronchospasm and
Cough Palpitations and Flushing
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Clinical Presentation of Colon Neuroendocrine Cancer
• abdominal pain• change in bowel habits• melena/hematochezia• anemia, weakness, weight loss• symptoms of carcinoid syndrome rare
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Diagnosis of Neuroendocrine Colon Cancer
• Colonoscopy with biopsy
• Immunohistochemical stains for chromogranin and synaptophysin
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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
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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