Pheochromocytoma and pancreatic adenocarcinoma

Case contributed by Frank Gaillard
Diagnosis certain

Presentation

Nausea and vomitting for investigation.

Patient Data

Age: 70 years
Gender: Female

A 2.5 x 1.9 cm ovoid hypodensity at the uncinate process of the pancreas is associated with mild common bile duct dilatation, measuring up to 12 mm. Mild intrahepatic ductal dilatation. Mild pancreatic duct prominence. No porta lymphadenopathy, or lymphadenopathy elsewhere. However, there is a large heterogeneous, right adrenal mass is without definite calcification, measuring up to 5.3 cm. The left adrenal gland is also moderately bulky. Incidental splenunculus. No free fluid. Past cholecystectomy. No suspicious focal bony lesion. Lung bases unremarkable.

Conclusion:

2.5cm pancreatic head lesion with mild biliary tree dilatation is suspicious for pancreatic tumor such as carcinoma. The large right adrenal mass is likely malignant, more likely a metastasis in this context although a second pathology is not excluded. Clinical/biochemical correlation would be helpful in the first instance.

Case Discussion

The patient went on to have a Whipples and adrenal mass resection.  

MICROSCOPIC DESCRIPTION:

Sections of pancreas show a 22mm moderately differentiated 
adenocarcinoma in the head and uncinate process of the pancreas. The tumor forms large irregular infiltrative and focally cribriform glands, containing some necrotic luminal debris, within a markedly fibrotic stroma. Tumor cell are large and cuboidal with abundant vacuolated eosinophilic cytoplasm. Some cells contain mucin. Tumor nuclei are large with irregular thickened nuclear membranes, fine 
vesicular chromatin and prominent nucleoli. Mitotic figures are frequent. Tumor abuts but does not invade the muscularis propria of the duodenum. Tumor is adjacent to nerves but no perineural invasion is seen. No lymphovascular invasion is present. Tumor invades extra pancreatic fat and is 1.5mm from the posterior margin (inferiorly) and 2mm from the anterior pancreatic surface (inferiorly). It is well clear of the pancreatic resection margin and all other pancreatic surfaces, bowel margins and bile duct margins. Tumor is present in 2 inferior pancreatic lymph nodes (one directly invaded; 0.5mm deposit in other, no extranodal extension); five other lymph nodes show no evidence of tumor.

Sections of adrenal show an expansile proliferation of cuboidal to slightly spindle shaped cells compressing adrenal cortex. Tumor cells have abundant cytoplasm containing amphophilic small granules and moderate to mildly pleomorphic nuclei containing coarsely speckled chromatin. Tumor cells are arranged in nests and ribbons within a highly vascular stroma. No mitotic figures are identified. Invasion of the adrenal capsule, extra adrenal tissue, nerves and lymphovascular vessels is not identified. The central tumor shows small foci of necrosis and some regions of hyalinised stroma populated by macrophages. The margins are clear.

DIAGNOSIS:

Whipple's tissue:  Moderately differentiated pancreatic ductal adenocarcinoma.

Right adrenalectomy: Pheochromocytoma. Completely excised.

Case courtesy of A.Prof Daminen Stella.

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