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Presented by Ralph Hruban, M.D. and prepared by Maryam Farinola M.D.
Case 5: This middle-aged woman developed abdominal pain that radiated to her back.
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Week 219: Case 5
This middle-aged woman developed abdominal pain that radiated to her back. A large mass in the head of the pancreas was identified on CT scan.images/large cell ne carcinoma 3.jpg
images/large cell ne carcinoma 4.jpg
images/large cell ne carcinoma CD56.jpg
images/large cell ne carcinoma NSE.jpg
images/large cell ne carcinoma AE1.jpgCorrect
Answer: Large cell neuroendocrine carcinoma
Histology: This high-grade neoplasm has large areas of geographic necrosis, a very high mitotic rate, and does not form glands. Immunolabeling for endocrine markers (NSE, CD56, Leu-7, etc.) are positive.
Discussion: Given the immunohistolabeling profile of this neoplasm, the differential diagnosis includes a well-differentiated pancreatic endocrine neoplasm (PEN), a small cell carcinoma, and a large cell neuroendocrine carcinoma. In this case the geographic necrosis and extremely high mitotic rate are not consistent with a well-differentiated PEN. Poorly differentially endocrine carcinomas are characterized by abundant necrosis, a very high mitotic rate (typically >50 per 10 hpf) and extensive invasion. The cells of the large cell type have a moderate amount of amphophylic cystoplasm and the nuclei are round to oval with coarsely clumped chromatin and prominent nucleoli. The nuclear features are those of a large cell carcinoma, and not those of a small cell carcinoma. Poorly differentiated endocrine carcinomas of the pancreas are rare, comprising only 2-3% of all pancreatic endocrine neoplasms. Because primary poorly differentiated endocrine carcinoma of the pancreas are so rare, the possibility of a metastasis of an extra-pancreatic primary should always be kept in mind. Prognosis, as expected, is extremely poor.
Incorrect
Answer: Large cell neuroendocrine carcinoma
Histology: This high-grade neoplasm has large areas of geographic necrosis, a very high mitotic rate, and does not form glands. Immunolabeling for endocrine markers (NSE, CD56, Leu-7, etc.) are positive.
Discussion: Given the immunohistolabeling profile of this neoplasm, the differential diagnosis includes a well-differentiated pancreatic endocrine neoplasm (PEN), a small cell carcinoma, and a large cell neuroendocrine carcinoma. In this case the geographic necrosis and extremely high mitotic rate are not consistent with a well-differentiated PEN. Poorly differentially endocrine carcinomas are characterized by abundant necrosis, a very high mitotic rate (typically >50 per 10 hpf) and extensive invasion. The cells of the large cell type have a moderate amount of amphophylic cystoplasm and the nuclei are round to oval with coarsely clumped chromatin and prominent nucleoli. The nuclear features are those of a large cell carcinoma, and not those of a small cell carcinoma. Poorly differentiated endocrine carcinomas of the pancreas are rare, comprising only 2-3% of all pancreatic endocrine neoplasms. Because primary poorly differentiated endocrine carcinoma of the pancreas are so rare, the possibility of a metastasis of an extra-pancreatic primary should always be kept in mind. Prognosis, as expected, is extremely poor.
