Solid Benign Neoplasms Flashcards
Liver Cell Adenoma found in
- young women (aged 20–40 years)
- steroid hormone use
- Oral contraceptive pill (OCP) use
- Anabolic Hormones
- Vascular liver diseases
- glycogenosis type 1A
- familial adenomatous polyposis.
molecular-pathologic classification
- β-catenin mutated adenoma
- HNF1A mutated adenoma
- inflammatory adenoma
- not otherwise specified adenoma
what is Liver adenomatosis
defined by the presence of more than 10 LCAs in the liver
highest risk of malignant transformation is observed in LCA with
β-catenin activation
LCA with β-catenin mutations can be further classified by the nature of the mutation
- exon-3 mutation have increased risk of hepatocellular carcinoma (HCC) degeneration
- mutation in exon 7/8 leads to only weak activation of β-catenin and no risk of malignant transformation.
Image features
> > intratumoral hemorrhage, the necrosis and fat component of LCA tends to be heterogeneous on CT
- On contrast-enhanced CT
» peripheral enhancement with centripetal progression. - MRI scans»_space; well-demarcated heterogeneous mass containing fat or hemorrhage
The two major risks of LCA are
rupture
malignant transformation.
Size for rupture ?
spontaneous rupture occurring in lesions 5 cm and larger
when to consider early intevention
Hepatic adenomas with β-catenin activation should be considered for early surgical intervention
Patients who present with acute hemorrhage ? Tx
- hepatic artery embolization»_space; temporizing maneuver.
- Once the patient is stabilized»_space; laparotomy and resection of the mass are required.
Patients with asymptomatic LCAs taking OCPs ? Tx
can be watched for regression after stopping of the OCPs
Pregnancy ?
Behavior of LCAs during pregnancy has been unpredictable
resection before a planned pregnancy is usually recommended
Margins needed ? and what is the Tx or Adenomatosis ?
- Margin status is not important in these resections
- Adenomatosis > large lesions should probably be resected because of the risk of rupture
Focal nodular hyperplasia (FNH)
- second most common benign tumor
- discovered in young women
- central fibrous scar with radiating septa
- The central scar often contains a large artery
- vascular malformation??
How to diagnose ?
- hypervascularity in the arterial phase of CT
- MRI with central nonenhancing scar.
The enhancement fades over time, and the lesion becomes isointense to the liver parenchyma in the portal and delayed phases.
When no central scar is seen ??
- differentiation from LCA or a malignant mass, especially fibrolamellar HCC, can sometimes be impossible.
- On occasion, histologic confirmation is necessary, and resection is recommended for definitive diagnosis.
- Fine-needle aspiration for the diagnosis of FNH has been recommended but is often unrevealing.
Tx of FNH ?
- Careful observation of symptomatic FNH with serial imaging
- Patients with persistent symptomatic FNH or an enlarging mass should be considered for resection.
Hemangioma
- most common benign tumor of the liver
What is Cavernous hemangiomas
Cavernous hemangiomas have been associated with FNH and are also theorized to be congenital vascular malformations
How does the hemangioma get enlarged ? and at what size it is called giant ?
- The enlargement of hemangiomas is by ectasia rather than by neoplasia.
- Lesions larger than 5 cm are arbitrarily called giant hemangiomas.
can it be difficult to differentiate from malignant tumors.??
Involution or thrombosis of hemangiomas can result in dense fibrotic masses that may be difficult to differentiate from malignant tumors.
What Syndrome might be associated with hemangioma ?
- Syndrome of thrombocytopenia and consumptive coagulopathy known as Kasabach-Merritt syndrome is rare but well described.
CT and MRI features ?
- typical peripheral nodular enhancement pattern is seen.
- Isotope-labeled red blood cell scans are an accurate test but are rarely necessary
- Percutaneous biopsy of a suspected hemangioma is potentially dangerous and inaccurate.
Indications for Hemangioma Resection
- Rupture
- significant change in size
- development of the Kasabach-Merritt syndrome
- for Definitive Diagnosis
Best approach in Surgery ?
The preferred approach to resection is enucleation with arterial inflow control, but anatomic resections may be necessary in some cases.
What about children with hemangioma
- Large hemangiomas in children can result in congestive heart failure secondary to arteriovenous shunting.
- Untreated symptomatic childhood hemangiomas are associated with high mortality.
- almost all small capillary hemangiomas resolve.
- Symptomatic childhood hemangiomas may be treated with therapeutic embolization AND medical therapy for congestive heart failure
- Resection if Symptomatic or Ruptured
Macro regenerative nodules, AKA : adenomatous hyperplasia
- circumscribed, bile-stained, bulging surface nodules
- in cirrhotic liver
- result from the hyperplastic response to chronic liver injury
- malignant potential
- difficult to distinguish from HCC
Nodular regenerative hyperplasia
- benign diffuse micronodular (usually <2 cm)
- associated with lymphoproliferative disorders, collagen vascular diseases, and the use of steroids or chemotherapy
- No malignant potential
Mesenchymal hamartomas
- rare solitary tumors of childhood
- usually large cystic masses found in the right liver
- Progressive and Painless
- Resection in case very Large
Fatty tumors of the liver
- lipomas
- myelolipomas (which contain hematopoietic tissue)
- angiolipomas (which contain blood vessels)
- angiomyolipomas (which contain smooth muscle).
