A 64-year-old Caucasian woman with a history of a resected gastric carcinoid 38 years ago presented to the Department of Gastroenterology at the University of Maryland with symptoms of early satiety and a progressive, 6-months weight loss of 20 pounds. The patient had no other complaints. Her past medical history was significant for trigeminal neuralgia which had been surgically treated. She did not take any medications and denied a family history of cancer. On exam the patient was a thin, well-appearing woman without remarkable abnormal physical findings. Laboratory values revealed a mild anemia with a hemoglobin of 10 mg/dL. Her electrolytes, liver function tests, and coagulation panel were within the normal ranges.
The patient underwent upper endoscopy and endoscopic ultrasound (EUS), which revealed the following findings: an area of nodular mucosa in the distal esophagus at the gastroesophageal junction, an erythematous patch in the stomach, a 2-cm gastric polyp, which was then resected, and an extragastric mass, consistent with a group of matted lymph nodes, located between the left lobe of the liver and the anterior wall of the stomach. The pathology results of the biopsied sites showed that the gastric polyp and nodular mucosa represented tubular adenomas, the latter containing focal intramucosal adenocarcinoma. The fine needle aspiration (FNA) of the gastric mass was consistent with metastatic NEC (malignant carcinoid), and the erythematous patch in the stomach at 40-cm showed invasive poorly differentiated/signet-ring cell carcinoma.
The patient underwent a staging computed tomography (CT) scan of the abdomen and pelvis that demonstrated two large enhancing masses between the left lateral lobe of the liver and the anterior wall of the stomach corresponding to the extragastric mass seen on endoscopic ultrasound. There was no evidence of distant metastases on CT scan.
The patient was then referred to the Department of Surgical Oncology, for evaluation for surgical resection. Intraoperatively, there was no evidence of distant metastases. A D2 total gastrectomy with omentectomy and regional lymph node dissection with a Roux-en-Y J-pouch esophagojejunostomy was performed. There were no palpable masses in the stomach. However, there were two palpable masses in the gastrohepatic ligament, consistent with metastatic lymph nodes. The spleen and distal pancreas were preserved.
Postoperative recovery was uneventful with the exception of one urinary tract infection. The patient was discharged to a rehabilitation facility on the fifteenth postoperative day.
The use of paraffin blocks for this study meets Institutional Review Board and Health Insurance Portability and Accountability Act requirements, and has been approved by the Institutional Review Board at the University of Maryland Protocol Number: H-29227.
The resected tissue was fixed in 10% buffered formalin and embedded in paraffin. The tissue was sectioned in 5 micron thick slices and stained with hematoxylin and eosin (H and E), mucicarmine and Periodic Acid-Schiff (PAS).
Immunohistochemical staining was performed using Ventana Enhanced DAB Detection Kit and Biotin-StreptAvidin (B-SA) amplified methodology (Ventana, Tucson, AZ) and commercially available prediluted monoclonal antibodies against the following antigens: neuron specific enolase (NSE), synaptophysin, chromogranin, E-cadherin, pancytokeratin, CAM 5.2, and Ki-67 (all Ventana, Tucson, AZ).
Fragments of formalin fixed gastric mucosa were also processed for evaluation by electron microscopy. Representative tissue samples (1-mm cubes) were fixed in 4F1G for 4 hours, postfixed in osmium tetroxide, dehydrated in graded alcohols, and embedded in epoxy resin. The sections were stained with uranyl acetate and lead citrate and examined on a JEM 1200 transmission electron microscope.
Pathologic findings: gross appearance
Macroscopically, the gastrectomy specimen contained a tan-white plaque-like mass measuring 3.5 × 3.4 × 0.5 cm located in the body of the stomach (lesser curvature), approximately 10.5 cm from the proximal margin and approximately 15.2 cm from the distal margin, which appeared to invade both the mucosa and the submucosa. The uninvolved gastric mucosa was tan with pinpoint hemorrhages, with absence of folds. Also present were multiple, well-circumscribed, firm, yellow nodules (ranging in size 0.4–0.7 cm in greatest diameter) scattered throughout the mucosa and submucosa of the cardia and body of the stomach. There were also two larger extragastric nodules (5.5 × 2.7 × 3.1 cm and 7.2 × 3.8 × 2.9 cm respectively) consistent with matted lymph nodes that were tan, firm, and well-circumscribed and grossly abutting the wall of the lesser curvature. These two nodules were located 9.1 cm from the proximal margin and 13.2 cm from the distal margin respectively. The cut surfaces of the extragastric nodules were firm, pale tan to pink, and hemorrhagic. The attached adipose tissue contained multiple lymph nodes, ranging from less than 0.1 × <0.1 cm × <0.1 cm to 1.1 × 0.4 × 0.2 cm. Ten resected lymph nodes, in addition to the two large collections of matted lymph nodes, were submitted.