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78 Cards in this Set
- Front
- Back
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What is the most common extracranial solid tumor in children?
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Neuroblastoma
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What is a favorable prognostic marker for neuroblastoma?
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Aneuploidy
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What is an unfavorable genetic marker in neuroblastoma?
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Amplification of N-myc oncogene
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What chromosomal abnormality is found in up to 80% of neuroblastom?
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Deletion of short arm of chromosome 1 (adverse)
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What pathologic classification is used in Neuroblastoma and what is good/bad prognosis?
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Shimada classification (stroma-poor is bad, stroma-rich is good)
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What % of patients with neuroblastoma present with metastasis?
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70%
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What is acute myoclonic encephalopathy and what is its association?
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Myoclonus, rapid multidirectional eye movement (opsoclonus), and ataxia. Good prognosis for neuroblastoma but the neurologic results are lasting
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How is acute myoclonic encephalopathy of neuroblastoma treated?
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ACTH, high dose IV gamma globulin, and cyclophosphamide
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What is Stage IVS for neuroblastoma?
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Localized primary tumor with dissemination limited to bone marrow, skin, and/or liver in infants <1 year
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How does the site of origin for neuroblastoma affect prognosis?
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Nonadrenal primary tumors have better survival
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Poor outcome in stage IV-S patients is associated with what biomarkers?
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Elevated enolase (>100), ferritin (>280), and urinary dopamine (>2500) and N-myc amplification
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What are the 5 prognostic factors for neuroblastoma?
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Age, stage, N-myc amplification, histologic grade, and ploidy (diploidy and tetraploidy bad, hyperdiploid good)
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How is low-risk neuroblastoma (stages I, II, IVS) treated?
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Surgery alone (chemo ONLY if N-myc amplification)
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How is high-risk neuroblastoma (stages III, IV) treated?
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Generally, chemo first to shrink tumor and then surgery
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What is the most common soft tissue sarcoma in children?
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Rhabdomyosarcoma
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What are the most common sites of GU rhabdo?
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Prostate, bladder, and paratesticular regions
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What are the key FH in Li-Fraumeni syndrome?
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Mother who had excess premenopausal breast cancer and siblings with increased risk for cancer
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What is the most common pathologic subtype of GU rhabdomyosarcoma and what/where are its variants?
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Embryonal (Botryoides in hollow organs like bladder/vagina) (Spindle cell or leiomyomatous in paratesticular region)- both excellent survival
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What are the three pathologic variants of GU rhabdomyosarcoma?
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Embryonal (good prognosis), alveolar (bad), undifferentiated (very bad)
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What mutation is associated with Li-Fraumeni syndrome?
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p53 mutation
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What chromosomal abnormalities are associated with alveolar rhabdo?
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Chromosomes 1 (PAX7), 2 (PAX3), or 13 (FKHR)
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What chromosomes are associated with embryonal rhabdo?
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LOH on chr 11p15 and IGF II overexpression
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Where does GU rhabdomyosarcoma usually spread?
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Lungs
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What is the best prognostic indicator for GU rhabdo?
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Stage at diagnosis
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Where does paratesticular rhabdo arise?
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Distal portion of spermatic cord
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Is paratesticular rhabdo a good or bad prognosis?
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Good- even the alveolar subtype is better than the others
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What is the treatment for paratesticular rhabdo?
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Unilateral radical orchiectomy
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Of those with paratesticular rhabdo, who should undergo RPLND and how?
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Children 10 years and older should undergo ipsilateral RPLND as part of routine staging before chemotherapy
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Where does vaginal rhabdo arise from?
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Anterior vaginal wall in area of embryonic vesicovaginal septum (UG sinus)
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How are vaginal and uterine rhabdo treated?
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Chemotherapy and delayed local therapy if possible
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What is the most common primary renal tumor of childhood?
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Wilms'
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Which three GU abnormalities are associated with Wilms' tumor?
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Hypospadias, fusion anomalies (horseshoe 7x more common) and cryptorchidism
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What is Denys-Drash syndrome?
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Male pseudohermaphroditism, renal mesangial sclerosis, and nephroblastoma
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Which mutation is the culprit in Denys-Drash and WAGR?
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Chromosome 11p13 Wilms' tumor gene WT1
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Aniridia and Wilms' tumor is most associated with…
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WAGR syndrome (Wilms', aniridia, genital anomalies, and mental Retardation)
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What gene is associated with WAGR?
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PAX6 gene (or WT1)
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What is Beckwith-Wiedemann syndrome?
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Excess growth at cellular, organ (macroglossia,nephromegaly, hepatomegaly), and body segment (hemihypertrophy)
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How should BWS patients be followed?
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Serial RUS every 3-4 months to detect tumors
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What chromosome and gene are associated with Beckwith-Wiedemann syndrome?
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WT2 on chromosome 11p15
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Which chromosomal abnormalities in Wilms' tumor is associated with increased relapse and death?
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LOH for chromosomes 1p and 16q
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What is the "classic" pathology for Wilms?
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TRIPHASIC: Islands of compact, undifferentiated blastema and presence of variable epithelial differentiation (embryonic tubules, rosettes, and glomeruloid structures) and stromal cells
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Blastemal vs. epithelial predominant: what is the prognosis for Wilms'?
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Blastemal predominant is highly aggressive but very responsive to chemo; Epithelial predominant is low aggressiveness, but more resistant to chemotherapy
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What are the two types of nephrogenic rests?
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Perilobar (PLNRS) and Intralobar (ILNRs)
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Which nephrogenic rests are associated with WAGR and which with BWS?
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PLNR associated with BWS. ILNR associated with DDS, aniridia
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Multiple nephrogenic rests in one kidney imply…
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NRs in other kidney and increased risk for development of contralateral disease for Wilms
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Nephroblastomatosis is…
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presence of multiple NRs
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What is the most common renal tumor in infants?
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Congenital mesoblastic nephroma
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What are two factors that increase relapse risk in Wilms' tumor patients?
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Spillage of tumor and positive lymph nodes
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What should be done before surgery for Wilms' tumor and why?
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Chest CT to check for lung mets (most common mets)- may need chemo
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Most important prognostic indicators for Wilms' tumor?
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Staging and histopathology
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Wilms Stage I or II FH and Stage I anaplastic are treated with…
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18 week pulse VCR, AMD (dactinomycin)
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Wilms Stage III FH or II/III focal anaplastic treated with…
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AMD, VCR, DOX, and 10.8 Gy abdominal irradiation
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Wilms Stage IV FH
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Abdominal irradiation and 12 Gy to both lungs
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How do you treat bilateral Wilms'?
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Biopsy first to confirm, the chemo to shrink the tumor to decrease risk of renal failure after eventual partial nephrectomy
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How long should you wait between nephrectomy for Wilms and renal transplant?
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Some say 2 years, some 1 year
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What are important predictors of survival for clear cell sarcoma of kidney?
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Younger age at diagnosis, lower stage, treatment with DOX, and absence of tumor necrosis
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How does CCSK differ from Wilms'?
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CCSK is associated with brain and bone mets (not lung) and CCSK stage I relapse and require irradiation
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What is the most lethal and aggressive childhood renal tumor?
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rhabdoid tumor of the kidney
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What are the three histologic subytpes of congenital mesoblastic nephroma?
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Classic, cellular and mixed
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How is congenital mesoblastic nephroma treated?
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Surgery only- must be a complete resection
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What histologic subtype is more common in RCC in children?
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Papillary
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What chromosomal abnormality is more common in children with RCC?
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Chromosome translocation involving common breakpoint in TFE gene located at Xp11.2
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AML develops in what % of patients with TSC?
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80%
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Which genes are associated with TSC?
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Chromosome 9 (TSC1) and 16 (TSC2)
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Which testicular tumor is the most common malignant testis tumor in infants and young boys?
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Yolk sac
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What is the pathognomonic pathologic sign of Yolk sac tumor?
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Schiller Duval bodies and staining for AFP
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What is the pathognomonic pathologic sign of Leydig cell tumor?
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Reinke crystals
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Normal AFP levels (<10) occur by what age?
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8 months
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What is the half life of beta-HCG?
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24 hours
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What is the half life of AFP?
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7 days
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What is the most common prepubertal testis tumor?
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Teratoma
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How are childhood testicular teratomas managed?
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Subtotal orchiectomy
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What is another name for a monodermal teratoma?
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Epidermoid cyst
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What is the most common metastatic site for YST in children?
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Lung
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How are Stage I YSTs followed?
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Monthly for 3 months with Cxray, CT or MRI, then 3 months, then 6 months for 36 months
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Which is the most common of the sex cord testicular tumors?
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Leydig cell (peak incidence 4-5 yo)
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What symptom may be found in conjunction with Sertoli cell tumors?
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Gynecomastia
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Which two syndromes are associated with large cell Sertoli cell tumors?
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Carney complex and Peutz-Jeghers syndrome
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