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Presented by Peter Illei, M.D. and prepared by Safia Salaria, M.B.B.S.
Case 1: This 63-year-old woman initially presented a few months ago with an unintentional 20 pounds weight loss over the past 3 months.
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1. Question
Week 492: Case 1
This 63-year-old woman initially presented a few months ago with an unintentional 20 pounds weight loss over the past 3 months, decreased appetite, and low energy. A CT scan of the abdomen and pelvis showed multiple lesions in the liver consistent with metastatic disease. Subsequent PET scan showed several FDG-avid lesions in the hemi-liver with the largest in the right hemi-liver.She has no other evidence of hypermetabolic activity in the rest of her body.images/1Alex/080811Case1Qimage1.jpg
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images/1Alex/080811Case1Qimage5.jpgCorrect
Answer: Metastatic well-differentiated neuroendocrine neoplasm (carcinoid tumor)
Histology: The tumor has a papillary architecture where the tumor cells are arranged surrounding dilated thin walled blood vessels.The nuclei are oval to round with little pleomorphism and have finely dispersed chromatin.Mitoses are not readily identified.
Discussion: The tumor cells are positive for cytokeratin (AE1/AE3 – image 6),chromogranin,synaptophysin and are negative for CK7,CK20,CK17,Heppar-1 and TTF-1.This staining pattern supports the diagnosis of metastatic well differentiated neuroendocrine neoplasm (carcinoid tumor).In order to determine possible origin and biologic behavior immunostains were performed for CDX-2 (gastrointestinal origin),PAX-8(pancreatic origin) and Ki67.The tumor cells are negative for PAX8(image 7)and positive for CDX-2(image 8).This staining pattern favors a gastrointestinal origin. The Ki67 proliferation index is <2%(image 9)consistent with a low grade neuroendocrine neoplasm.
Follow up clinical inquiry revealed that patient has had symptoms of profuse diarrhea intermittently. She also complains of some flushing and mild hot flashes, particularly in her head and neck area. She does not have any headaches, light headedness, dizziness, or episodes of syncope. She has never had any shortness of breath or pain.
Incorrect
Answer: Metastatic well-differentiated neuroendocrine neoplasm (carcinoid tumor)
Histology: The tumor has a papillary architecture where the tumor cells are arranged surrounding dilated thin walled blood vessels.The nuclei are oval to round with little pleomorphism and have finely dispersed chromatin.Mitoses are not readily identified.
Discussion: The tumor cells are positive for cytokeratin (AE1/AE3 – image 6),chromogranin,synaptophysin and are negative for CK7,CK20,CK17,Heppar-1 and TTF-1.This staining pattern supports the diagnosis of metastatic well differentiated neuroendocrine neoplasm (carcinoid tumor).In order to determine possible origin and biologic behavior immunostains were performed for CDX-2 (gastrointestinal origin),PAX-8(pancreatic origin) and Ki67.The tumor cells are negative for PAX8(image 7)and positive for CDX-2(image 8).This staining pattern favors a gastrointestinal origin. The Ki67 proliferation index is <2%(image 9)consistent with a low grade neuroendocrine neoplasm.
Follow up clinical inquiry revealed that patient has had symptoms of profuse diarrhea intermittently. She also complains of some flushing and mild hot flashes, particularly in her head and neck area. She does not have any headaches, light headedness, dizziness, or episodes of syncope. She has never had any shortness of breath or pain.
