A 58-year-old African American male presented to the Emergency Department by ambulance complaining of a constant, sharp, and worsening midepigastric pain that radiated posterior for approximately 30 minutes. The pain was perceived to be 10 out of 10 and was described as an inflated balloon that was squeezing from the inside out. The paramedics found the patient in his house hypotensive and lying in a right lateral decubitus position. The patient was given a bolus of normal saline and his blood pressure responded appropriately. Upon examination, the patient claimed to of had a two-year history of worsening mild midepigastric and left upper quadrant abdominal pain and was seen by his primary care provider whom prescribed him a proton pump inhibitor (PPI) which partially relieved some symptoms. In addition, the patient complained of being nauseated, feeling bloated after meals, having a history of bloody stools one week prior to admission, and increased pain after eating. He denied vomiting or early satiety. Vital signs on admission were stable.
Past medical history included gastroesophageal reflux disease (GERD), hyperlipidemia and impaired fasting glucose. His medications consisted of a PPI, H2-blocker and simvastatin. The only past surgical history was an appendectomy. The patient was a truck driver for several years. He denied alcohol, drug use, and smoked approximately ½ packs of cigarettes a day for the past 40 years. He reported his two nieces having an unspecified type of cancer but no other known family history of cancer or heart diseases. Review of systems was negative except for gastrointestinal complaints as described above. The patient denied weight loss, fatigue, fever, shortness of breath, and/or chest pain. On physical examination, the abdomen was hard, guarding was noted, and there was direct and rebound tenderness noted in all four quadrants but worse in the epigastric area.
On admission, the patient had a complete laboratory workup involving all systems. The abnormalities found mildly decreased were hemoglobin of 12.9 g/dl, hematocrit of 39.6%, albumin of 3.1 g/dl, and sodium of 132 mEq/l. The only abnormalities found mildly increased was the red blood distribution width of 16%, white blood cell count of 15,100 ul, corrected calcium of 11.32 mg/dl, glucose of 129 mg/dl, prothrombin time of 12.2 seconds, and the international ratio of 1.2 seconds. Stool guaiac was negative.
Computed tomography (CT) of the abdomen and pelvis on admission revealed a large 4.7 centimeter (cm) lobulated hypodense mass in the region of the porta hepatis probably arising from the liver with the possibility of adjacent morphologic tubular lymph nodes or satellite lesions (). Extending from the lobulated mass of the liver was a broad lobulated 10 cm band of density just deep to the rectus muscle extending from the upper abdomen to the level below the iliac crest, which possibly represented extraperitoneal infiltration. Additionally, an enhancing lesion was seen in the caudate lobe of the liver that was most likely metastasis. Blood was noted in both paracolic gutters, and there was a pancake-type density along the anterior abdominal wall, which was most likely blood that extended into the right pelvis representing omental caking. After review of the CT, serum measurements of Cancer antigen (CA) 19-9, Carcino embryonic antigen (CEA), and Alpha-fetoprotein (AFP) were found to be within normal limits. Serum Helicobacter pylori was positive. Colonoscopy and esophagastroduodenoscopy was recommended after review of the CT and findings were mild antral gastritis and a nearly obstructing mass in the descending colon. Recommendations were to rule out other malignancies and surgical intervention. An acute abdomen series with chest radiograph was consistent with the CT with additional findings of patchy and streaky infiltrate of the right and left lung bases and question of the mass in the left upper quadrant displacing the stomach medially. There was no evidence of acute obstruction or air under the diaphragm. Positron emission tomography scan was performed to evaluate for metastases and results showed increase uptake in the left upper quadrant, and about 4 cm of caudate lobe of the liver.
Computed tomography (CT) of the abdomen and pelvis. A large 4.7 centimeter (cm) lobulated hypodense mass is shown in the region of the porta hepatis likely secondary to the tumor mass.
Utilizing CT guidance, biopsy of the large mass in the left upper quadrant was performed by Interventional Radiology on the same day of admission. Pathology results two days later showed a well-differentiated neuroendocrine carcinoma. The architecture revealed insular and trabecular morphologies, and the Ki-67 index was approximately 30% positive (). Microscopic sections showed nested epithelial cells with moderately increased mitotic activity, nuclear atypia, irregular nuclear contours and hyperchromasia. Immunohistological stains were performed with the following results: the epithelial component was positive for cytokeratin AE1/AE3, neuroendocrine marker synaptophysin was positive, neuroendocrine marker chromogranin-A was positive, neuroendocrine marker neurone specific enolase was positive, and CA 19-9 was negative.
Figure 2. Histological demonstration of the well-differentiated neuroendocrine carcinoma. Pathology slides from the tumourus mass showing a well-differentiated neuroendocrine carcinoma. The architecture is consistent with the insular and trabecular morphologies, (more ...)
On hospital day number six the surgery service performed a diagnostic laparoscopy, exploratory laparotomy with distal pancreatectomy, splenectomy, partial gastrectomy, left colectomy, and resection of the caudate lobe of the liver (). Pre and postoperative diagnoses were well-differentiated neuroendocrine tumor of the pancreatic tail with metastasis to the caudate lobe of the liver. After diagnostic laparoscopy revealed a moderate amount of blood around the peritoneal cavity and metastasis to the liver it was necessary to convert to exploratory laparotomy. Blood was aspirated from the abdominal cavity and sent to cytology, which later revealed numerous neutrophils admixed with red blood cells yet no malignant cells. General inspection of the peritoneal cavity revealed a tumor in the caudate lobe as well as a large 10 cm mass that was fixed in the left upper quadrant. The tumor itself was quite vascular and was surrounded by varices, which was most likely the cause for the blood throughout the abdomen as well as within the lesser sac. Additionally, a periaortic lymph node appeared grossly positive and was then dissected and sent for analysis. There was no evidence of metastases in the pelvis, small bowel, or omentum. The diaphragm was not involved or infiltrated by the tumor and neither was the left kidney or adrenal glands.
Exploratory laparotomy. Distal pancreatectomy, splenectomy, partial gastrectomy, left colectomy, and resection of the caudate lobe of the liver were performed during the surgical procedure.
Overall, the surgery was successful. At the end of the procedure two 10 ml Jackson-Pratt drains were inserted into the lesser sac and left upper quadrant.
Gross specimens were sent to pathology in three parts: frozen section of the periaortic lymph node measured 1.0 by 0.8 by 0.4 cm, the caudate lobe of the liver measured 4.0 by 3.5 by 2.5 cm, and the massive left upper quadrant tumor that consisted of an en bloc resection of the spleen, partial transverse colon, partial small bowel, pancreatic tail, partial distal stomach, adipose tissue, and lymph nodes. The spleen measured 12 by 7.5 by 2.5 cm, the colon measured 33 cm in length and 4 cm in diameter, the pancreatic tail measured 12 by 8 by 6 cm, a portion of the small bowel measured 15 cm in length and about 3 cm in diameter, and a small portion of the distal stomach measured 9 by 6.5 cm (). The massive tumor invaded into the serosa of the transverse colon, distal stomach, and small bowel. The tumor appeared to compress the capsule of the spleen, however, no direct invasion was identified (). After sectioning of all the involved organs it was noted that the tumor originated from the pancreas. Within the adjacent adipose tissue, multiple lymph nodes ranging in size from 0.3 to 1.0 cm were analyzed.
Gross specimen of the liver. One of the specimens removed from the body are shown here which presents the caudate lobe of the liver measured 4.0 by 3.5 by 2.5 cm.
Splenic tissue. This histological picture is showing the tumor compressing the capsule of the spleen without any direct invasion.
Microscopic sections were analyzed and found the perioarotic lymph node to be negative for metastatic carcinoma. Microscopic sections of the caudate lobe of the liver showed metastatic well-differentiated neuroendocrine carcinoma (). Sections of the massive left upper quadrant mass showed invasive well-differentiated neuroendocrine carcinoma characterized by cells with high nuclear to cytoplasmic ratio, irregular nuclear contours and hyperchromasia arranged in trabecular and insular patterns. There were 21 of 21 lymph nodes positive for metastatic carcinoma. Margins of resection were free of tumor. Tumor node metastasis (TNM) staging was T4, N1, M1.
Figures 6 & 7.
Microscopic sections of the caudate lobe of the liver. These pictures are demonstrating metastatic well-differentiated neuroendocrine carcinoma in different magnifications (exact numbers are mentioned in the figures).
Overall, the patient tolerated the procedure and hospital stay well. During the course of hospitalization his pain was well controlled with morphine, and his glucose was well controlled after being weaned off of an insulin drip and placed on a sliding scale. Vaccinations for encapsulated organisms including pneumococcal, Streptococcus pneumoniae, Haemophilus influenza and Neisseria meningitides were administered. Twenty days post-admission the patient was discharged from the hospital with instructions not to lift more than 15 pounds, and to follow-up with surgery in one week.