Term
| TGF-B effects related to invasion |
|
Definition
- increased angiogenesis
- increased deposit of ECM
- immunosuppression |
|
|
Term
Produced by regulatory T cells and
fibroblasts in the STROMA of invasive
carcinoma |
|
Definition
TGF-ß
Tumor Suppressor Gene |
|
|
Term
| - immunosuppressive immune cells: |
|
Definition
Tregs,
macrophages and PMNs |
|
|
Term
what are the three primary neoplasm of the heart and in what age group do they occur?
which one is malignant? |
|
Definition
Myxoma - adults
Rhabdomyoma - children
Angiosarcoma- adults (malignant one) |
|
|
Term
| Primary neoplasms of the heart Myxoma: clinical presentation |
|
Definition
Clinical presentation
- Ball valve obstruction-heart failure
- Fragmentation with systemic embolization
- Systemic inflammatory reaction-fever due to IL 6 from tumor |
|
|
Term
What is the most common neoplasm of the heart of infants?
benign or malignant? |
|
Definition
|
|
Term
| What aggressive primary malignancy occurs in the heart? |
|
Definition
|
|
Term
| • Believed to arise from totipotent germ cell rests |
|
Definition
| teratoma ( 75% mature benign benign) |
|
|
Term
| the most common location for a teratoma is sacrococcygeal what are some other locations? |
|
Definition
Midline- mediastinum; retroperitoneum;
head and neck
- Ovaries and testes |
|
|
Term
| word for mature to a benign type |
|
Definition
|
|
Term
| Histologic differences between adult and pediatric cancers |
|
Definition
Ped. cancers have
- Anaplasia less common
“-blastoma” : primitive (embryonal) appearance
with features of organogenesis is common
- small round blue cell histology |
|
|
Term
| most common solid non CNS tumor of children under 4 |
|
Definition
|
|
Term
| 5 most Common malignant tumors under age 4 |
|
Definition
1. Acute leukemia
2. Posterior fossa CNS tumors ( #1 solid)
3. Neuroblastoma - (#1 solid nonCNS)
4. Retinoblastoma
5. Wilm’s tumor ( nephroblastoma) |
|
|
Term
Characteristic histology of the most primitive
tumors of childhood |
|
Definition
| Small round blue cell tumors (SRBCT) |
|
|
Term
| Tumors with SRBCT histology: |
|
Definition
- Ewing’s sarcoma/primitive neuroectodermal tumor (PNET)
- Malignant lymphoma
- Poorly diff. neuroblastoma, rhabdomyosarcoma
- Occasional Wilm’s tumor |
|
|
Term
| neuroblastoma is a malignant cancer arising from |
|
Definition
| primordial neural crest cells of sym. ganglia |
|
|
Term
| most common location of neuroblastoma |
|
Definition
|
|
Term
mostly mature ganglion cells
- Schwann cells are present
- Favorable histology |
|
Definition
| Ganglioneuroma: most mature/Benign form of neuroblastoma |
|
|
Term
| nephroblastoma arises from where? |
|
Definition
|
|
Term
3 genetic conditions that in 10% predispose to get wilms tumor?
what gene involved? |
|
Definition
- WAGR : WT1 gene
- Denys-Drash : WT1 gene
- Beckwith-Wiedemann syndrome : WT2 |
|
|
Term
|
Definition
|
|
Term
Tumor suppressor gene
• Transcription factor for normal renal,
gonadal development |
|
Definition
|
|
Term
male pseudohermaphroditism and nephropathy, renal failure
you think? |
|
Definition
Denys-Drash:
Missense mutation in WT1 gene |
|
|
Term
aniridia (absent irises)
genital malformation
mental retardation
you think? |
|
Definition
WAGR
Chromosome 11 p deletion
Deleted region includes PAX6 gene for irises |
|
|
Term
Enlargement of organs; macroglossia
Hemihypertrophy
Adrenal cortical cytomegaly
you think? |
|
Definition
beckwith-wiedemann syndrome
WT-2 locus of multiple gene involved. |
|
|
Term
| What is the microscopic morphology of Wilm’s tumor? |
|
Definition
Triphasic microscopic morphology
- blastema; stroma and epithelial cell types |
|
|
Term
| Which disease in this section associated with HYPERTENSION!!! |
|
Definition
|
|
Term
| all retinoblastoma is associated with______ on chromosome ____ |
|
Definition
|
|
Term
Micro:
small round blue cells forming Flexner-
Wintersteiner rosettes (cells arranged in a circle) |
|
Definition
|
|
Term
| karyotype of retinoblastoma would see? |
|
Definition
| partial deletions in chromosome 13 |
|
|
Term
“Cat’s eye reflex” – leukocoria; whitish hue
Poor vision, strabismus may occur
lack red reflex
you think? |
|
Definition
|
|
