Neuroendocrine Neoplasms of the Pancreas: A Multidisciplinary Approach

The correct answer is the second option; see the table below.

Overall Stage Primary Tumor (T) Nodal Metastasis (N) Distant Metastasis (M)
I T1 N0 M0
II T2 or T3 N0 M0
III Any T
T4
N1
  N0
M0
  M0
IV Any T Any N M1

What is the recommended follow-up for this patient?

Per NCCN guidelines (Neuroendocrine and Adrenal Tumors Version 3.2018), surveillance should include obtaining biomarkers and a multiphasic CT or MRI or chest CT (+/- contrast) as clinically indicated within 3-12 months following surgery, and then after one year post-op every 6-12 months for a maximum of 10 years. These recommendations also apply to patients for whom active surveillance is chosen instead of surgery, and testing may be done earlier if symptoms occur. Somatostatin receptor-based imaging and FDG-PET/CT scans are not recommended for routine surveillance.

Case 2

Patient 2 is a 65 year old man with no significant past medical history who presented with jaundice and pruritus. Labs revealed a total bilirubin of 11.5 (normal <1.4), AST 85 (normal range: 15-46), ALT 230 (normal range: 21-72), and total alkaline phosphatase of 375 (normal range: 38-126). Endoscopic retrograde cholangiopancreatography (ERCP) showed a stricture within the intrapancreatic portion of the common bile duct. A stent was placed in the common bile duct, and subsequent CT scans of the chest, abdomen and pelvis (figure 2A) showed a 9 mm enhancing lesion (yellow arrow) at the termination of the pancreatic duct, which was noted to be dilated to approximately 8 mm. A metal stent (red arrowhead) deployed during the ERCP is noted in the common bile duct. Pancreaticoduodenectomy (Whipple) was performed. Microscopic images are provided (figures 2B-2D).

Histology: Photomicrographs depict sheets of poorly-differentiated neoplastic cells with high nuclear to cytoplasmic ratio, nuclear irregularity, prominent nucleoli, numerous apoptotic figures and patchy necrosis. There were more than 20 mitoses per 10 high power fields. 60% of tumor nuclei were immunoreactive with MIB-1 (Ki67 K2 Leica). Similar to case 1, tumor cells were diffusely immunoreactive with synaptophysin (27G12, Leica) but only focally for chromogranin (LK2H10, Ventana) and keratin (AE1-AE3, Biocare). Tumor cells were negative for the other immunoperoxidase stains stated in Case 1.

What is most likely the diagnosis?

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