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

Chromogranin

Colon Biopsy

Chromogranin

Colon Biopsy Synatophysin

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

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

Classification of NE Cancers of the Colon

Classification of Neuroendocrine Cancers of the Colon I.Well-Differentiated Tumors – Carcinoid II.Well-Differentiated Endocrine Carcinoma – Malignant Carcinoid III.Poorly Differentiated Endocrine Carcinoma – our patient IV.Mixed Exocrine-Endocrine Carcinoma

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

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

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

Pathogenesis of Mr. L’s Disease

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

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

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

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