Upgrade to remove ads
Terms in this set (60)
In children, are benign or malignant tumors more common?
(can still cause problems due to location)
The most common tumor of infancy
Where are hemangiomas typically located?
on the skin of face and scalp
what are the 2 phases of growth of hemangiomas in infants?
rapid proliff: 0-3 months
involution: regress by age 7
Hemangiomas in infants may indicate _____ disease.
What is a common complication of large hemangiomas?
low platelets or cardiac stress
(hemangioma is taking all of the blood)
What are the 2 histological types of hemangiomas?
capillary (usually regresses on its own, flat red)
cavernous (may require surgery, large nodule)
What are 2 common lymphatic tumors seen in children?
Which pediatric lymphatic tumor in children is a result of hamartoma?
where are lymphangiomas often seen?
deep neck, mediastinum, retroperitoneum, axilla
A fibrous tumor in a 4 year old is removed and examined on histological slide. It shows low cellularity and normal cells. What type of tumor is it most likely? How should it be treated?
fibrous tumor -- fibromatosis
-- treated with removal with negative margins
This type of fibrous tumor in children is highly cellular. Where is it typically found?
typically @ extremities
What translocation is characteristically seen in highly cellular fibrous tumors seen in the extremities of children?
(condition is congenital-infantile fibrosarcoma)
What are the 2 main peaks of teratoma incidence?
1. < age 2
what are majority of teratomas considered?
mature (benign and well differentiated)
What is the most common site of a teratoma?
(also testes/ovary, midline structures)
Which gender is preferentially affected by teratomas?
females (4:1 ratio to males)
Which kind of teratomas are benign?
Which kind of teratomas are considered to have indeterminate malignant potential?
note: malignant teratomas are mixed with other cells or have changed germ cell types
What is the leading cause of death from disease in the 5-14 y.o. cohort?
What tumor account for more deaths in children less than 15 than all other tumors combined?
true or false: the incidence of congenital anomalies is higher in children with cancer?
(aniridia higher is people w/ Wilms' tumor)
Aniridia, or lack of an iris, is a condition commonly associated with what malignancy?
Wilms' Tumor (WAGR syndrome)
what malignancy is associated with Down Syndrome?
Most malignant pediatric tumors originate in what location?
bone marrow (think leukemias and lymphomas)
Most malignant adult tumors originate in what location?
What are 3 examples of "small round blue cell tumors"?
Ewing Sarcoma (PNET)
What is the most common intraocular childhood cancer? What is the origin?
what is the cause of 40% of retinoblastoma?
germ line mutation in RB gene
What histology is associated with retinoblastoma?
Tumors of sympathetic ganglia and adrenal medulla? What are they derived from?
derived from neural crest cells
The most common MALIGNANT tumor of infancy.
note: the most common tumor of infancy was hemangioma
what is the most common malignant extracranial solid tumor of childhood?
Most cases of neuroblastoma are caused by a sporadic mutation in which gene?
(gain of function mutation)
what are the most common locations of neuroblastoma?
adrenal medulla (40%)
A 1 y.o. child is seen under your care for a large abdominal mass, fever and weight loss. What is a potential cause?
(classic presentation for children < 2 y.o.
also "blueberry muffin baby")
How do children > 2 y.o. typically present with neuroblastoma?
metastatic disease-- bone pain, respiratory symptoms and GI complaints
A "blueberry muffin baby" describes:
children < 2 y.o. with neuroblastoma
-- large abdominal mass and have cutaneous metastasis that are discolored (the blueberries)
What can be tested in the serum as a marker of neuroblastoma?
catecholamines (in 90%)
-- logical b/c this is a tumor of sympathetic ganglia and the adrenal medulla
What are 3 ways that body fluids can be used to detect neuroblastoma?
serum for catecholamines
urine for VMA or HVA (catecholamine metabolites)
serum for neuron-specific enolase
what morphology is seen in neuroblastoma?
necrosis, cystic softening, and hemorrhage
small primitive cells
What rosettes are found in neuroblastoma histology?
-- contrast with Flexner-Wintersteiner rosettes seen in Rb
what immunohistochemistry stain is used to test for neuroblastoma?
FISH can be used to detect ______ amplification, which is commonly seen in neuroblastoma.
(N for neuroblastoma)
What characteristic change in neuroblastoma gives a positive prognosis?
-- e.g. matures into a ganglioneuroma (which lacks neuroblasts)
what does maturation of neuroblastoma require?
Children <18months with neuroblastoma have what prognosis? >18 months?
younger: better (even if stage 4S)
What is the most important prognostic indicator for neuroblastoma?
N-MYC oncogene amplification
(done via FISH study of chromosome2) --> high risk regardless of age & stage
how does ploidy in neuroblastoma determine prognosis?
in children <2 yrs old
What is the most common primary renal tumor of childhood?
There's an increased risk of Wilms' tumor in which 3 syndromes?
1. WAGR syndrome
2. Denys-Drash syndrome
3. Beckwith-Wiedemann syndrome
What are 2 mutations associated with Wilms' Tumor?
1. WT1 (tumor suppressor)
What is WAGR syndrome? What malignancy is it associated with?
Wilms' tumor (malignancy) (WT1 mutation) Aniridia, Genital anomalies and Retardation
What is aniridia sometimes associated with Wilms' Tumor?
PAX6 gene is on chromosome 11p13, which is the same location as the WT1 gene
A neonate is under your observation. You find that the child has aniridia (no irises) and hypospadias. What malignancy should you be concerned about?
Wilms' Tumor -- as a part of WAGR syndrome
Which Wilms' tumor associated syndrome has a dominant germline mutation in the WT1 gene?
Which Wilms' tumor associated syndrome is a result of failed imprinting?
-- result is increased expression of the WT2 gene
On histological specimens, a Wilms' tumor most closely mimics:
nephrogenesis (exhibits triphasic pattern -- blastema, stroma, epi cells)
What are nephrogenic rests? What are they associated with?
immature nephrogenic elements often found adjacent to Wilms' tumors
-- increased risk for a contralateral tumor so these patients will be followed
What is associated with a poor prognosis in Wilms' tumor patients?
-- these patients will likely not respond to chemotherapy bc correlated w p53 mutation
Sets found in the same folder
Path1 B3 - Neoplasia 1: Classification/N…
Path3 B3 - Neoplasia 3: Molecular Basis…
Principles of Lab Testing
Sets with similar terms
Cardiorenal IV Path
71. Pathology: Intestinal Neoplasia and Colonic Po…
Other sets by this creator
OBGYN Residency Study
Fam Med Aquifer
PDCI all + book
Other Quizlet sets
Arrangement of Electrons in Atoms
A&P II Exam 3
study guide 11