Aciel A. Shaheen, MD, Inayat Gill, DO, Ahmed I. Edhi, MD, Mitual B. Amin, MD, Mitchell S. Cappell, MD, PhD; William Beaumont Hospital, Royal Oak, MI
Introduction: Pancreatic ganglioneuroma is a relatively rare tumor that almost always occurs in children, with a few cases reported in patients in their 20s and no cases reported in patients >60 years old. A case is reported of pancreatic ganglioneuroma in an octogenarian, and the clinical consequences of this new finding are discussed.
Methods: An 84-year-old female with a past medical history of advanced multiple myeloma presented with right upper quadrant and epigastric pain for two days. Vital signs were stable except for a respiratory rate of 24 breaths/minute with normal O₂ saturation. Physical exam revealed a soft abdomen with mild epigastric tenderness, normoactive bowel sounds, and no hepatosplenomegaly. Routine laboratory tests were remarkable for hemoglobin of 8.7 g/dL (normal 12.1-15 g/dL), normal liver function tests, and normal serum lipase. Quantitative serum immunoglobulins and serum and urine protein electrophoresis revealed characteristic findings of multiple myeloma, with IgA lambda monoclonal gammopathy. Abdominal ultrasound and abdominopelvic CT with IV and oral contrast revealed dilated intra-and extra- hepatic bile ducts, ≥5 hepatic cysts, and a 1.2 x 0.8 cm hypodense pancreatic mass (Figure-1). Endoscopic ultrasound showed the hypoechoic pancreatic mass had mildly irregular but well-defined borders with no evident invasion of nearby structures. FNA during EUS revealed pancreatic ganglioneuroma by H & E stain (Figure-2), with positive immunohistochemistry for S100, SOX-10, and synaptophysin (Figure-3), findings diagnostic of pancreatic ganglioneuroma. The tumor was not excised because of her advanced multiple myeloma. The patient experienced symptomatic improvement one month after discharge from rehabilitation therapy. Discussion: This work reports the new finding of pancreatic ganglioneuroma occurring in an octogenarian. This report has clinical implications because pancreatic ganglioneuroma generally has non-specific imagining findings, requires tissue for diagnosis, and generally requires complete excision because it can rarely metastasize. The differential of small hypoechoic pancreatic lesions should include pancreatic ganglioneuroma and such lesions may require excision even when asymptomatic.
Figure-1. Axial section of abdominal computerized tomograph at the level of T3 showing hypodense 12 x 8 mm pancreatic mass (horizontal arrow), dilated common bile duct (vertical arrow), and multiple small hypodense hepatic cysts.
Figure-2. Medium power photomicrograph of hematoxylin & eosin stained section of the pancreatic tumor shows an admixture of small-sized, prominent, spindle shaped Schwann cells (*), mostly present on the left, and clusters of large ganglion cells (arrows), mostly present on the right.
Figure-3. Immunohistochemistry for synaptophysin shows positivity (brown color) in the cytoplasm of the ganglion cells (arrows). There is faint, barely perceptible, granular staining in the cytoplasm of the Schwann cell component.
Disclosures: Aciel Shaheen indicated no relevant financial relationships. Inayat Gill indicated no relevant financial relationships. Ahmed Edhi indicated no relevant financial relationships. Mitual Amin indicated no relevant financial relationships. Mitchell Cappell: FDA – Consultant.