Pancreas and adrenal pathology Flashcards
Pancreatic endocrine neoplasm
- 2% of pancreatic tumors
- Occur anywhere in pancreas, sometimes peripancreatic or duodenal
- Solitary or multiple
- Can secrete any pancreatic hormone or non-functional
- Gross: circumscribed, solid
- Look like neuroendocrine carcinomas (carcinoids) elsewhere
- –> Round nuclei
- –> Coarse “salt and pepper” chromatin
- Some features suggest the type of hormone produced, but no need to determine pathologically –> diagnosis based on clinical symptoms and blood levels
Insulinoma
Confined to the pancreas
- Amyloid deposition is characteristic
Clinical:
- Episodic hypoglycemia causing confusion, stupor, loss of consciousness
- Precipitated by fasting or exercise
- Alleviated by food
- Mild or asymptomatic in 80%
May also see with islet cell hyperplasia –> congenital secondary to maternal diabetes
Exclude other causes of hypoglycemia
- Abnormal insulin sensitivity, liver disease, inherited glycogenoses, ectopic insulin production (rare tumors), self-injection
Gastrinoma
Zollinger Ellison syndrome
- islet cell tumor
- hypersecretion of gastric acid
- severe peptic ulcers
- often presents with diarrhea
Tumors occur in pancreas or duodenal wall
- more than half are invasive or metastatic at presentation
- Glucagonoma
- Somatostatinoma
- VIPomas
Glucagonoma
- mild DB mellitus
- skin rash
- anemia
Somatostatinoma
- DB mellitus (inhibits insulin)
- cholelithiasis
- steatorrhea
- hypochlorhydria
VIPomas
- watery diarrhea
- hypokalemia
- achlorhydria
Pancreatic endocrine tumors - clinical
- behavior may be benign or malignant, but difficult to predict based on pathologic features
- features diagnostic for malignancy –> mets, vascular invasion, local infiltration
- insulinomas = 90% benign
- all others = 60-90% malignant
Adrenal cortical hyperplasia
- Always bilateral
- May be diffuse or nodular
- Usually secondary to external stimulus (e.g. pituitary adenoma) –> especially if diffuse
- May produce one or multiple hormones
- Microscopic: Hyperplasia of fasciculata and reticularis zones
Adrenal cortical adenoma
- Most clinically silent, discovered incidentally
- May produce glucocorticoids or aldosterone, or rarely androgens
- Well-circumscribed, yellow to brown, usually Foamy cells are lipid-rich
- –> Eosinophilic are lipid-poor
- Mitoses are rare
- Cortisol-secreting adenomas and (surprisingly) aldosterone-secreting adenomas both look like zona fasciculata
Adrenal cortical neoplasms
Spirinolactone bodies –> laminated eosinophilic cytoplasmic inclusions seen in aldo-secreting adenomas
- due to spironolactone treatment for htn
Adrenal cortical carcinoma
Clinical
- Occur at any age, even children
- More commonly functional than adenomas
- Virilization common
Gross:
- Larger than adenoma (usually >200 g and >20 cm)
- Unencapsulated, infiltrative, often obliterates rest of adrenal
- Necrosis, hemorrhage, cystic changes common
Histology
- range from bland to anaplastic nuclear features
- mitoses common
Prognosis
- often invades adrenal vein, IVC and lymphatics
- distant mets common –> regional lymph nodes, lungs, other organs
- median survival ~2 years
Adrenal adenoma vs. carcinoma
Gross:
- Large size, hemorrhage, necrosis favor malignant
Microscopic:
- Atypia
- Mitotic activity
- Atypical mitoses
- Absence of clear cells
- Diffuse archtitecture
- Necrosis
- Capsular invasion
- Vascular invasion
- 4 usually malignant
Myelolipoma
- Benign tumor composed of fat and hematopoietic elements
- looks like normal bone marrow
- may also see this feature within adenomas
Pheo - clinical
Clinical
- Paroxysmal hypertension
- Tachycardia, palpitations, headache, sweating, tremor, and sense of apprehesion
- Confirm diagnosis by measuring urine catecholamines : VMA and metanephrines
Associated with several syndromes: MEN-2, NF1, VHL
Rule of 10%: 10% bilateral (higher if familial) 10% extra-adrenal 10% malignant 10% asymptomatic (no hypertension) 10% familial (this one is wrong, actually more like 25%)
Pheo - gross and histologic
- malignancy
Gross
- variable size
- arise in medulla
- yellow-tan
- hemorrhage, necrosis and cysts common
Histology:
- polygonal to spindles cells arranged in nests = zellballen
- surrounded by small spindles = “sustentacular cells”
- cytoplasm is granular
- nuclei round with coarse salt and pepper chromatic
- tumor cells stain for neuroendocrine markers = synaptophysin + chromogranin
- sustentacular cells stain for S-100
Malignancy
- cannot be predicted by histologic features
- even local and vascular invasion not reliable
- only diagnosed as malignant if mets
- mets are to lymph nodes, liver, lung and bone
Paragangliomas
- same as pheo, but arise outside adrenal
- most common in neck (carotid body) and para-aortic (ganglia of sympathetic nervous system
Neuroblastoma
- Tumor of young children
- 80% >4 years
- Rarely see in adults
Presentation:
- Abdomninal mass
- Watery diarrhea – elaborate VIP
- Arise in adrenal, sometimes paraganglia
Gross: large, soft, gray; hemorrhage, necrosis common
- Solitary; <10% multifocal
Microscopic = small blue cell tumor
- homer wright rosettes seen in 25%
Prognosis
- overall 3 year survival = 30%
- recurrences within 2 years
- local extension to kidney and spinal cord; distant mets to liver, bone and lymph nodes
