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13 Cards in this Set

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Pregnant woman with vaginal bleeding.

U/S shows "snowstorm" appearance. Serum hCG is markedly elevated.

Micro: circumferential trophoblastic hyperplasia

1. what are the 2 variants?
2. what are the genetic diferrences?
Molar pregnancy

1. complete vs. partial. Partial may have fetal parts, complete does not. partial has normal villi and elnarged villi.

2. Complete mole- diploid with 2 paternal chromosomes, partial- triploid (2 sperm)
Risk factors for chorioamnionitis?

1.
2.
3.
4.
5.
1. PPROM
2. infection with listeria
3. Gram B strep
4. E. coli
5. Candida

CMV does not cause neutrophilic chorioamnionitis, can cause histiocytic/plasmacytic villitis
young girl with isosexual precotious puberty. May have hemoperitoneum at presentation

Gross: Ovarian mass with areas of hemorrhage and necrosis

Micro: solid population of granulosa cells with dilater irregular immature follicles. Rare Call-Exner bodies

IHC: inhibin
Juvenile granulosa cell tumor
Young infant with an abdominal mass

Gross:?

Micro: SRBC tumor with homer-wright rosettes

1. Genetic abnormality?
Neuroblastoma

1. may have MYCN amplification
Young infant with an abdominal mass

Gross:

Micro: triphasic histology with epithelial, stromal, ebryonal components

1. prognosis factors?

IHC: membranous WT1

IHC:
Wilm's tumor

1. anaplasia
Young girl with a polypoid vaginal mass

Micro: Spindle cells that stain + for myoD1, desmin.

Do not have FKHR mutation
Vaginal embryonal rhabdomyosarcoma

FKHR mutations are seen in alveolar rhabdomyosarcomas
Pregnant woman with fetal distress resulting in ceasarian delivery

Gross: fleshy round necrotic mass within the placenta

Micro: small neoplastic capillary formation

1. risk to embryo/fetus?
Choroangioma

1. can cause high-cardiac output faliure, hydrops
Causes fetal hydrops through maternal transmission:

Micro: increased nucleated red cells with smudgy nuclei
Parvovirus
Infant (<2 years) with most common renal neoplasm.

Micro: uniform intersecting fascicles of tumor cells or highly cellular lesion with abundant mitoses. Extend into renal parenchyoma entrapping renal elements, may result in cysts for classic variant, well demarcated in cellular variant. Dysplastic change and cartilege may be seen.

1. Genetic alteration?

2. If cellular variant is NOT well demarcated?

3. If seen in child >2 years?
Mesoblastic nephroma

1: show t(12;15)- only in cellular variant

2. Infantile fibrosarcoma

3. Likely mentanephric stromal tumor- has "collarettes" around vessels and tubules
Most common primary mesenchymal liver in childhood. May present with high-output cardiac failure

Micro: Connection of small vascular spaces lined by bland endothelial cells

IHC: CD31. CD34, Factor VIII
Hemangioendothelioma
Cardiac abnormalities that result in cyanosis (righ-to-left shunting)

1.
2.
3.
4.
5.
All start with "T"

1. Tetralogy of Fallot
2. Transposition of Arteries
3. total anolymous pulmonary return
4. tricuspid atresia
5. truncus arteriosus
Infant born withfacial dysmorphism, cardiac abnormalities (tetralogy of Fallot), recurrent infections. Also have hypocalcemia due to parathyroid deficiency


1. Eponym?
2. Genetic cause?
1. Di George syndome- thymic hypoplasia causes T cell deficiency.
2. Deletion 22q
Infant born with macrocephaly, macroglossia, enlarged kidneys. May have hypoglycemia.

1. what tumors may develop?
2. genetic cause?
Beckwidth-weidermann syndrome

1. Wilms, hepatoblastoma, adrenal cortical carcinoma

2. Imprinting in 11p silences gene expression