Neoplastic Pancreas Flashcards
Etiologies of Von Hippel-Lindau Syndrome
Autosomal dominant
Etiologies of Polycystic Kidney Disease
Autosomal dominant
Characteristics of pt’s with Von Hippel-Lindau Syndrome
- 75% have pancreatic cysts
- Neuroendocrine tumors - related to, or involving, the interaction between the nervous system and the hormones of the endocrine glands
- Renal cell carcinoma
- Eye tumors
- Brain/spinal cord lesions
- Pheochromocytomas
Clinical signs of Von Hippel-Lindau Syndrome
- Asymptomatic
- Pain/pressure/jaundice if obstruction of the bile ducts occurs
- HTN w/ pheochromocytomas
Sonographic appearance of Von Hippel-Lindau Syndrome
- Single or multiple of various sizes
- Size and number of cysts can increase with age
- 72% found in patient at autopsy
- 25% found in patients with ultrasound
What percentage of pancreatic cysts does neoplastic cysts make up?
10-15%
What are the 2 types of neoplastic cysts?
- Microcystic adenoma (serous)
- Macrocystic adenocarcinoma (mucinous)
Where is Microcystic Adenoma Found?
- Rare
- Benign
- Elderly women
- No potential for malignency
- Seen in patients with Von Hippel-Lindau Syndrome
Where is Macrocystic Adenocarcinoma Found?
- Rare
- Significant malignancy potential
- Slow growing
- 35-60% found in body and tail, only 5% found in head
Clinical Signs of Microcystic Adenoma (Cystadenoma)
- Asymptomatic
- More frequent in females
- People over 60yo
Labs associated with microcystic adenoma (cystadenoma)
- None specific
- Increase in amylase
Sonographic appearance of microcystic adenoma (cystadenoma)
- Large, well circumscribed lesion with multiple tiny cysts
- Cyst size varies from 1-20mm
Clinical signs of macrocystic adenocarcinoma (cystadenoma)
- Non-specific abd. epigastic pain
- Palpable mass
- Concurrent disease, such as diabetes, calculous disease of biliary tree, arterial hypertension
Sonographic appearance of macrocystic adenocarcinoma (cystadenoma)
- Well circumscribed
- Thin or thick walled
- Uni- or multi-loculared
- > 20mm in size
Etiology of Adenocarcinoma
- Most common primary tumor of the pancreas
- 60-70% occur in the head of pancreas
- Involves the EXOCRINE!!! portion of gland
- > 90% of all malignant tumors of the pancreas
- 4th leading cause of cancer death, behind lung, breast, & colon
- Rare before 40yo. 2/3 present after 60.
- More common in males!!!
- Poor prognosis. Mean average survival time is 2-3 months
Clinical signs of Adenocarcinoma
- Symptoms appear late in disease
- Pain radiating to back, dull ache in mid epigastrium
- Painless jaundice
- N/V
- Weight loss!!!!!
- Clay colored stools
Labs associated with Adenocarcinoma
-Increased bilirubin (direct or indirect???)
Etiology of Islet Cell Tumors
- Endocrine!!!
- Neuroendocrine tumors
- Of, or relating too, or involving the interaction between the nervous system and the hormones of the endocrine glands
Characteristics of Non-Functioning Islet Cell Tumors
- Make up 1/3 of all islet cell tumors
- 92% of these are malignant
- Slow growth rate
- Typically no distant metastasis
Characteristics of Functional Islet Cell Tumors
- Typically benign
- Produce symptoms
Types of Functional Islet Cell Tumors
- Insulinoma
- Gastrinoma
- Glucagonoma (highly malignant)
- Vipoma (highly malignant)
- Somtostinoma
- Last 3 are rare
Clinical signs of Insulinoma
- Most common islet cell tumor (60%)
- 10% will be malignant
- Produces too much insulin
- Pt will present with hypoglycemia
Labs associated with insulinoma
-Low glucose
Sonographic appearance of insulinoma
-Well defined, small, hypoechoic mass
Clinical signs of gastrinoma
- 2nd most common functional tumor (18%)
- Higher malignancy rate of 25-60%
- Produces a syndrome known as Zollinger-Ellison syndrome
- Pt presents with diarrhea and peptic ulcer disease (PUD)
- Mean age at presentation is 50yo
What is cystic fibrosis?
- Common hereditary disease which affects the entire body causing progressive disability and often early death.
- Name refers to the characteristic scarring (fibrosis) and cyst formation within the pancreas, first recognized in the 30s
Sonographic appearance of cystic fibrosis
- Increased echogenicity - caused by fibrosis and fatty replacement
- Cysts are very tiny and unresolvable for US