PMC6588116_01
Physical findings consistent with Cushing's syndrome:both new and progressive for the 6 months before diagnosis. (A) Easy bruisability. (B) Buffalo hump.
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On admission, physical examination showed classic stigmata of Cushing's syndrome, including easy bruisability, moon facies, a buffalo hump, and abdominal striae (Fig. 1).
(A) Endoscopic ultrasound of pancreatic mass. (B) CT abdomen of the pancreatic mass, also demonstrating a concerning perihepatic lymph node that was proven positive on final pathology. (C) PET-CT demonstrating mild PET avidity of the pancreatic mass. CT, computed tomography; PET-CT, positron emission technology-CT.
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ACTH concentration gradient, leading to a diagnosis of ectopic ACTH syndrome. Abdominal imaging, including ultrasonography, computed tomography (CT), and ultimately positron emission tomography (PET)-CT, identified a 3.4 x 2.9 cm hypermetabolic mass in the pancreatic tail with abnormal peripancreatic lymph nodes and a hepatic lesion concerning for metastatic disease (Fig. 2).
Sections contain a circumscribed high-grade neuroendocrine neoplasm with variable morphology, including areas of small monotonous cells with abundant eosinophilic or clear cytoplasm arranged in nests, cords, and trabeculae (A, magnification 400 x ) and areas of monotonous cells with a higher nuclear:cytoplasmic ratio growing in sheets (B, magnification 400 x ). The cells have round to oval nuclei with dispersed chromatin. There is no nuclear molding or large cells with abundant cytoplasm. Mitotic figures are frequent (B, arrows) with mitotic count of at least 27 mitoses per 10 high-power fields. Small patches of necrosis are present (<5% of tumor). Immunohistochemical stain for Ki-67 demonstrates a proliferative index of 21% (C, magnification 400 x ). The overall features are those of a high-grade neuroendocrine carcinoma, but not those of a typical small cell carcinoma or large cell neuroendocrine carcinoma, which usually exhibit unique morphological features, as well as abundant necrosis and very high Ki-67 proliferative index. Based on AJCC TNM system eighth edition this tumor fits the criteria for a "well-differentiated neuroendocrine tumor grade 3."
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Histological examination revealed a high-grade neuroendocrine carcinoma without features of small or large cell involving pancreas, liver, and 6 of 16 lymph nodes (Fig. 3).