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