Abstract
Imaging of a 53-year-old Japanese man revealed two tumors in the liver and a tumor in the head of the pancreas with a swelling lymph node. A needle biopsy for the liver tumors was performed, revealing a neuroendocrine tumor. Enucleation, lymphadenectomy, and partial hepatectomy were performed. The microscopic examination identified many tumor cells with intracytoplasmic inclusions arranged in a nested, cord, or tubular fashion. The intracytoplasmic inclusions displayed densely eosinophilic globules and displaced the nuclei toward the periphery, which constitutes “rhabdoid” features. The tumor cells were positive for synaptophysin and weakly positive for NCAM, but negative for chromogranin A. Epithelial markers (AE1/AE3 and CAM5.2) accentuated intracytoplasmic globules. Pancreatic neuroendocrine tumors with rhabdoid features are very rare. Generally, rhabdoid features are aggressive and dedifferentiated characteristics of various types of tumor. Pancreatic neuroendocrine tumors containing rhabdoid cells tend to display extrapancreatic spread at the time of presentation, although some of these tumors with rhabdoid features are not always associated with aggressive behavior.
Highlights
Neuroendocrine tumors of the pancreas are rare neoplasms that account for 1–2% of all pancreatic tumors [1]
Electron microscopy studies have shown that the rhabdoid inclusions of pancreatic endocrine tumors are composed of whorls of intermediate filaments that entrapped variable numbers of neurosecretory granules and that in some inclusions, the intermediate filaments and neurosecretory granules are accompanied by rough and smooth endoplasmic reticulum profiles [8]
The eosinophilic inclusions were revealed to be composed of whorls of intermediate filaments that displaced cytoplasmic organelles and the nuclei to the periphery [13]
Summary
Neuroendocrine tumors of the pancreas are rare neoplasms that account for 1–2% of all pancreatic tumors [1]. A hypervascular mass had been detected in the left lobule of the liver by computer tomography (CT) for other disease. He had a history of hypertension, but no history of hereditary disease such as Von Hippel-Lindau disease nor family history of the patients. Atypical cells were positive for CAM5.2, synaptophysin, chromogranin A, and glucagon and negative for CK7, CK20, AFP, vimentin, CD34, desmin, c-kit, insulin, gastrin, and somatostatin. These results were suggestive of a metastatic neuroendocrine tumor. The patient is alive 4.5 years after surgery without evidence of recurrence or metastasis
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