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Abstract
The case of a malignant pancreatic endocrine neoplasm with an unusual signet ring cell appearance is reported. The tumor was resected from a 30-year-old man with a 4.0-cm tumor in the body of the pancreas diagnosed by computerized tomographic (CT) scan. The resected tumor had a unique morphology characterized by numerous mucin-negative, signet ring cells, which were argyrophilic and immunoreactive for cyto-keratin (CAM 5.2), chromogranin, synaptophysin, neuron specific enolase, and gastrin. Dense-core neurosecretory-type granules and numerous cytoplasmic lamellar inclusions were identified by electron microscopy. These inclusion bodies consisted of multilayered concentric osmiophilic lamellae (myelin figures), which most likely represent an abnormal accumulation of degenerating organelles. Two years later, the patient developed an abdominal recurrence of the tumor, confirming its malignant behavior. This case expands the spectrum of pancreatic endocrine tumors to include an aggressive signet ring cell tumor with a novel ultrastructural basis.