Organ-Specific Flashcards
What are the 3 subsets of gliomas their predominance?
ASTROCYTOMA
- 80% of primary tumors in adults
- graded from diffuse, to anaplastic to glioblastoma
- pilocytic are relatively benign, typically in children, found in NF1
OLIGODENDROMA
- 5-15% of gliomas
- common in 40’s and 50’s
- better prognosis, than astrocytomas
EPENDYOMA
- arise next to ventricular system
- 5-10% of brain tumors in first 2 decades of life
- commonly in spinal cord
- common in NF2
- commonly present with posterior fossa symptomatology and obstruction
What are the subtypes of Neuronal tumors and their key features?
GANGLIOMAS
- slow growing
- usually well controlled with surgery
- BRAF mutation common
DYSEMBRYOPLASTIC NEUROEPITHELIAL
- rare tumor of childhood usually presents as seizure disorder
CENTRAL NEUROCYTOMA
- found in ventricular system, similar to oligodendroglioma in cell morphology
What are some of the features of medulloblastoma?
- WHO IV
- central locations in children, lateral locations in adults
- poorly differentiated
- 20% of brain tumors in children
- associated with Li-Fraumeni syndrome from mutations in Tp53
- rosette formation as histological features
- radiosensitive
What are some of the features of meningiomas?
- usually slow growing
- patients often present with poorly localised findings
- can have psammoma bodies
- prior radiotherapy to head and neck is a predisposing factor
- associated with NF2
What is fat embolism syndrome?
pulmonary insufficiency, neurologic symptoms, anaemia and thrombocytopaenia
combination of mechanical obstruction and toxic intermediaries due to
release of FFA from fat globules causing local toxic injury to endothelium
What are some distinct features of both MEN syndromes that set the m apart from sporadic endocrine cancers?
- YOUNG age of onset
- MULTIPLE endocrine organs either synchronous or metachronous
- often MULTIFOCAL within the one organ
- often preceded by asymptomatic HYPERPLASIA
- more AGGRESSIVE and higher recurrence rate
What are the abnormalities associated with MEN-1?
the 3 P’s
- PARATHYROID: hyperplasia, usually the initial manifestation
- PANCREAS: edocrine tumors, leading cause of morbidity/mortality
- PITUITARY tumors, frequently prolactinomas
+
- gastrinomas, often in duodenum
- adrenocortical tumors
- thyroid tumors
- lipomas
What are the subtypes of MEN-2 and the abnormalities associated with them?
MEN2A - phaeochromocytoma - medullary carcinoma of thyroid (100%) - parathyroid hyperplasia caused by germline gain-offunction mutations in the RET protooncogene on chromosome 10q11.2
MEN2B
- medullary carcinoma of thyroid
- phaeochromocytomas
- NO primary hyperparathyroidism
- neuromas and gangliomas
Familial Medullary Thyroid Cancer
- variation of MEN2A
- strong predispositoin of medullary thyroid cancer but not the other associated PT hyperplasia or phaeochromocytoma
What are the hallmark appearances of osteoporotic bone compared to normal bone?
Microfractures
Thickened vertical trabeculae, reduced horizontal trabeculae
Macroscopically enlarged bone with thick, coarsened cortices
What is the pathogenesis of Paget’s disease of bone?
- activating mutations in RANK, inactivating mutations in OPG
- initial osteolytic phase
- mixed osteoclastic-osteoblastic phase
- burned out, quiescent osteosclerotic phase
What is Von-Hippel Lindau syndrome?
associated with cerebellum and retina hemangioblastomas, renal cysts and RCC
Which cancer has a strong association with polycythaemia?
Renal adenocarcinoma
In which zone of the prostate is prostate adenocarcinoma usually found?
70% peripheral zones, classically posterior
How do we classify testicular neoplasms and what are some of the key facts about each?
GERM CELL (95%)
- seminomas: most common, don’t contain AFP or HCG, extremely radiosensitive
- embryonal
- choriocarcinoma: highly malignant
- teratomas
- non-seminomas are likely to present with advanced clinical disease
NON-GERMINAL (5%)
- leydig
- sertoli
- mostly benign
Which cancers have an increased risk in association with the OCP?
The OCP is protective against which cancers?
INCREASED RISK
breast Ca, cervical Ca and hepatic adenoma
DECREASED RISK
ovarian Ca, endometrial Ca and colorectal Ca
What is Monckeberg’s sclerosis? What are the characteristics that differentiate it from typical arteriolosclerosis?
Calcific deposits in muscular arteris, visible on radiographs and palpable but DO NOT encroach on vessel lumen. Age related degenerative process and an eample of dystrophic calcification.
Arteriolosclerosis (hyaline and hyperplastic) are associated with thickening of vessel walls with luminal narrowing
What are the key differences between dystrophic and metastatic calcification?
DYSTROPHIC
- occurs in dying tissues
- not associated with normal serum calcium
- eg. atherosclerosis, valvular disease
METASTATIC CALFIFICATION
- occurs in normal tissues
- associated with hypercalcaemia
What are the 3 stereotypical reactions at a cellular level to injury of the blood vessel wall?
smooth muscle cell proliferation, extracellular matrix deposition, and intimal expansion.
What are the histological appearances and causes of Hyaline vs Hyperplastic arteriolosclerosis?
HYALINE
Arteriolar wall is thickened with increased protein deposition (hyalinized), and the lumen is markedly narrowed
- due to age, hypertension and diabetes
HYPERPLASTIC
Concentric, laminated (“onion-skin”) thickening of the walls with luminal narrowing
- hypertension
Describe the pathological sequence of atherosclerosis in 7 steps.
- ENDOTHELIAL injury causing increased permeability and endothelial activation
- ACCUMULATION of lipoproteins in vessel wall
- MONOCYTE adhesion to endothelium and migration of macrophages and foam cells
- PLATELET adhesion
- FACTOR release inducing smooth muscle recruitment
- SMOOTH MUSCLE cell proliferation, ECM production and T cell recruitment
- LIPID accumulation in macrophages and smooth muscle cells
Comparing lesinos of the left and right coronary systems, what are some of the differences?
LEFT
- short
- LAD and circumflex usually proximal
RIGHT
- diffuse and distal
- PIVA usually spared
What are some pathological features of atherosclerotic plaques that are prone to rupture?
- large areas of foam cells and extracellular lipid
- thin fibrous caps or few smooth muscle cells
- clusters of inflammatory cells
What is syndrome X in reference to coronary disease?
Angina in the absence of occlusive lesions. Thought to be due to microvasculature lesions
What are the key sequelae of severe burns? (>20% surface area)
- hypovolaemic shock: increased local interstitial osmotic pressure from release of osmotic actives, rapidly shift body fluids into interstitium
- injury to airways and lungs: inhalation of toxic components
- seconcdary infection: most common in pseudomonas
- ↓lymphocytic and phagocytic function
- hypermetabolic state
