A Rare Case of Sclerosing Variant of Pancreatic Neuroendocrine Tumor-Expressing Glucagon
Abstract Introduction Sclerosing variant of pancreatic neuroendocrine tumor (PanNET) is a rare variant of PanNET characterized by decreased tumor cellularity and prominent stromal fibrosis, and it is considered associated with serotonin expression. We herein present an unusual case of a sclerosing PanNET expressing glucagon. Methods The patient, a 75-year-old woman with refractory diabetes, scaly erythematous skin plaques in the eyelid, bilateral elbows and scalp, and episodes of nausea and poor appetite, presented to emergency department for acute abdominal pain. Abdominal computed tomography showed a 17-mm pancreatic head mass. Endoscopic ultrasound with fine-needle biopsy was performed for histopathological examination, followed by pancreaticoduodenectomy. Results Biopsy showed a background of predominantly blood and benign strips of pancreatic parenchyma and scanty fragments of fibrotic hyalinized stroma containing medium-sized atypical monotonous cells with round-to-ovoid nuclei, smooth borders, and inconspicuous nucleoli. These cells were difficult to characterize morphologically due to the scanty sampling. Immunohistochemical stains revealed the atypical cells were positive for AE1/AE2, synaptophysin, chromogranin, and glucagon and negative for insulin, serotonin, and pancreatic polypeptide. The lack of heterogeneous peptide cell composition supported a neoplastic proliferation of neuroendocrine cells. The pancreaticoduodenectomy specimen showed a 1.2-cm white firm mass in the pancreatic head. Histopathological study showed that, in a sclerotic background, the tumor cells grew mostly in trabecular patterns and occasionally in single cells, infiltrating into adjacent pancreatic parenchyma, and showed the same immunoprofile features as in the biopsy specimen. The proliferative index was <3%, supporting the diagnosis of low-grade sclerosing PanNET. The patient recovered well postsurgery. Moreover, the episodes of nausea and loss of appetite improved postsurgery. Patient did not show up for follow-up appointments in the dermatology clinic. Conclusion Sclerosing PanNET is challenging for diagnosis, especially on small biopsy specimens. Immunohistochemistry helps establish the diagnosis. Our case demonstrates a rare presentation of sclerosing PanNET expressing glucagon.