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Solid tumors > Other: liver tumors, retinoblastoma, GCT, etc. > Flashcards

Other: liver tumors, retinoblastoma, GCT, etc. Flashcards

1
Q

Pathology of hepatoblastoma: what is the most frequent subtype?

A

Mixed fetal-embryonal

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2
Q

Pathology of hepatoblastoma: frequency and prognostic significance SCU?

A

Small cell undifferenciated: 5%, poor prognosis; overlap with low AFP

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3
Q

Pathology of hepatoblastoma: frequency and prognostic significance of pure fetal histology?

A

7%; very good prognostic factor

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4
Q

What is the most significant toxicity of SUPER-PLADO (Cisplatin/carboplatin-doxorubicin) acute? chronic?

A
  • Hematological toxicity (G-CSF needed)

- Ototoxicity (up to 50% with dose-dense regimen)

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5
Q

What is characteristic of fibrolamellar histology in HCC?

A

Surrounded by normal liver, rather than cirrhotic liver; increases chance of resectability

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6
Q

Name 5 prognostic factors of hepatoblastoma?

A
  1. Resectability/staging
  2. Histology
  3. AFP < 100
  4. Multifocality
  5. Age > 5 years old
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7
Q

What is the main difference between SIOP and COG approaches? Name 1 advantage for each.

A

SIOP: neoadjuvant for all cases; increase resectability and give more time for surgical planning
COG: upfront resection whenever possible; early identification of histology and potential reduction of therapy in pure fetal histology; no evidence of increased surgical morbidity

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8
Q

What is the outcome of hepatoblastoma:

  • Localized?
  • Metastatic?
A

Localized: 90%
Metastatic: 40-60%

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9
Q

What is the outcome of HCC?

What is the main prognostic factor?

A

5 year OS: 28%; 5 year: 17-19%

Resectability

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10
Q

Name 5 genetic syndromes associated with hepatoblastoma.

A
  • Beckwitt-Wiedemann
  • Hemihypertrophy, overgrowth syndromes
  • Gardner syndrome
  • Familial polyposis
  • Glycogen storage disorders
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11
Q

Name 3 environnemental causes associated with hepatoblastoma.

A
  • VLBW
  • Prematurity
  • Parental exposure to R-OH (controversial)
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12
Q

Name genetic causes of HCC.

A
  • Glycogen storage disorder
  • A1-antitrypsine deficiency
  • Tyrosinemia
  • Hemochromatosis
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13
Q

Name acquired/environnemental causes of HCC.

A
  • Biliary cirrhosis
  • Viral hepatitis
  • Alcool
  • Anabolic steroids
  • Aflatoxins, environmental carcinogens
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14
Q

PRE-TEXT staging: name the other “high-risk” features

A 
\+V: invasion of IVC and all hepatic veins
\+P: invasion of portal system
\+E: biopsy-proven extrahepatic disease
\+M: distant metastasis
\+H (controversial): hemorrhage/rupture
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15
Q

Describe SIOPEL risk stratification of HB

A

Standard risk: PRETEXT 1-2-3, no high risk features

High risk: PRE-TEXT 4, or high risk features; AFP<100, hemorrhage/rupture (controversial)

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16
Q

Describe COG risk stratification of HB

A 
VLR: Pure fetal histology, stage I
LR: Stage I or II, non-SCU histology
IR: Stage I or II, SCU histology; 
     Stage III
HR: Stage IV; AFP <100
17
Q

Describe COG staging of HB

A 
(Post-surgical)
I: fully resected
II: residual microscopic disease
III: gross residual disease
IV: metastatic disease
18
Q

Retinoblastoma: what is the “pathognomical” sign of histology?

A

Flexner-Wintersteiner rosettes (70%)

Rarely, fleurettes can be seen as well; show more photoreceptor differenciation

19
Q

Which immunostaining of small, round blue cell tumor would identify RB?

A

CRX (cone-rod homeobox)

20
Q

How many cases of RB are related to a germline mutation? How much of them are de novo?

A

25% germ line (multifocal or bilateral)

25% of them are inherited and 75% are de novo

21
Q

What is associated with a 13q14 deletion?

A

Syndrome including:

  • Heredity retinoblastoma
  • Dysmorphisms
  • Mental retardation
  • Subtle skeletal abnormalities
  • Motor impairment
22
Q

What is trilateral RB?

A

Association of bilateral RB and asynchronous midline intracranial tumor, generally a PNET (such as pineoblastoma)

23
Q

Retinoblastoma:

1) What is the metastatic work up?
2) In which patients can the work up be omitted?

A

1) Bone scintigraphy; BMA/Bx; LP
(Also, ocular US and brain MRI should be done as part of the diagnostic work up, prior to staging)
2) Patients w/o HR features undergoing enucleation; patients with intra-ocular disease undergoing ocular salvage therapy

24
Q

Describe grossly the International Classification for Intraocular RB?

A

Group A: small tumors, away from foveola and disk
Group B: all remaining tumors confined to the retina
Group C: local subretinal fluid or seeding
Group D: diffuse subretinal fluid or seeding
Group E: presence of HR features (invasion of anterior segment, ciliary body, > 2/3 of globe, etc.)

25
Q

Retinoblastoma: which chemotherapy agents are active?

A
  • Microtubules inhibitors: VCR, paclitaxel
  • Platinum compounds (cisplatin, carboplatin)
  • Alkylating agents: cyclophosphamide, ifos
  • Anthracyclins: doxorubicin, idarubicin
  • Topoisomerase I inhibitor: topotecan
  • Topoisomerase II inhibitors: etoposide, teniposide
26
Q

Retinoblastoma: high risk features (5)

A

High risk pathology:
- Massive choroidal involvement
- Scleral invasion
- Invasion of optic nerve past the lamina cribosa
Transcleral involvement
Extension of disease to cut end of optic nerve

27
Q

Describe the surgical approach to ovarian GCT

A
  • En bloc resection of affected ovary and Fallopian tube
  • Resection of enlarged/suspicious LN
  • Peritoneal cytology necessary (collection of ascites or peritoneal washings)
    Not necessary: random omental or LN sampling, biopsy of contralateral ovary if appears normal
28
Q

Describe the surgical approach to testicular GCT

A
  • Inguinal approach: orchiectomy or testis-sparing enucleation (if focal lesion in pre-pubertal male)
  • If equivocal LN (2-4cm): LN biopsy (pre-pubertal) or RPLND (post-pubertal)
29
Q

Describe the surgical approach to sacrococcygeal GCT

A
  • En bloc resection of the tumor with coccygectomy

Consider biopsy followed by delayed resection (might increase survival by increasing rates of complete resection)

30
Q

Infantile choriocarcinoma

A
  • Very rare entity
  • Thought to be placental metastasis of maternal choriocarcinoma
  • Median age: 1 month old
  • Typical: FTT, anemia, hepatomegaly, seizures with very elevated b-HCG
  • Tx: multi-agent chemo (cisplatin, etposide +/- MTX) followed by delayed surgical resection
  • Screen mother after the diagnosis
31
Q

Terminology: what is the difference between germinoma, seminoma and dysgerminoma?

A

It is the location:
Germinoma: CNS
Seminoma: testicular
Dysgerminoma: ovary

Seminoma/dysgerminomas: usually occur in young adults, rare in children and teenagers
Very sensitive to radiation, but chemotherapy 1st line treatment to decrease long term morbidity
Surveillance only acceptable for stage I disease

32
Q

What pre-treatment tests are necessary in a patient with stage III gonadal tumor?

A
  • GFR
  • PFT, with DLCO measurements
  • Audiology testing
  • Fertility preservation: sperm banking, oocyte cryopreservation
33
Q

Cytogenetics in gonadal GCT

A

Pre-pubertal: del 6q, del 1p

Post-pubertal: i(12p), loss of 13, +21

34
Q

Growing teratoma syndrome

A

Situation when tumor enlarge during or after chemotherapy; repeat biopsy shows exclusively mature teratoma
Hypothesis: chemotherapy-induced differenciation of tumors
Treatment: surgery, when feasible; surveillance

35
Q

What proportion of patients with unilateral RB carry a germline mutation?

A

15%

36
Q

Which techniques are used to detect RB1 mutations?

What is the overall sensitivity?

A 
Multiples techniques (no hot spot, many point mutations): FISH, PCR, MLPA, sequencing, methylation
SE: 90-95%

**therefore, offsprings and sibling of survivors should be screened even if RB1 (-)ve re: possibility of false (-)ve

37
Q

Which second malignancies are commonly seen in retinoblastoma survivors?

A
  • Osteosarcomas (25-40%)
  • Soft tissue sarcomas (10-15%)
  • Melanomas and other skin cancers (15-20%)
  • Lung cancer and other common cancer of adulthood
38
Q

Cumulative incidence of malignancies in survivors of retinoblastoma:

  • treated with radiation?
  • not treated with radiation?
A

a) 35-40%

b) 20%

Solid tumors (5 decks)

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