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Core > Pediatrics: Genitourinary, Solid Organ > Flashcards

Pediatrics: Genitourinary, Solid Organ Flashcards

1
Q

Solid renal mass in a 0-3 y/o…

A
  • Nephroblastomatosis
  • Mesoblastic nephroma
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2
Q

Solid renal mass in a 4 y/o…

A
  • Wilms (very rare after age 10)
  • Wilms variants
  • Lymphoma
  • Multilocular cystic nephroma (if cystic, not solid)
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3
Q

Solid renal mass in a teenager…

A
  • Renal cell carcinoma
  • Lymphoma
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4
Q

Solid renal mass identified on day 1 of life…

A

Think mesoblastic nephroma

Note: AKA
“solid tumor of infancy”.

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5
Q

Management of nephroblastomatosis

A

Screening ultrasounds every 3 months until age 7-8 (due to risk of developing Wilms tumors)

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6
Q

Nephroblastomatosis

A

Persistent nephrogenic rests beyond 36 weeks gestation that present as solid renal masses

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7
Q

Bilateral Wilms tumors…

A

Nephroblastomatosis (persistence of fetal nephrogenic rests)

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8
Q

Can you be born with a Wilms tumor?

A

No, if born with a renal tumor think mesoblastic nephroma

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9
Q

What is the classic site of metastases for Wilms tumor?

A

Lungs

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10
Q

What is the classic site of metastases for a clear cell Wilms tumor?

A

Bone

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11
Q

What is the classic site of metastases for a rhabdoid Wilms tumor?

A

Brain

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12
Q

Next step: can’t differentiate between a multi-cystic nephroma and a cystic Wilms tumor

A

Surgical resection

Note: Never biopsy a possible Wilms tumor (you can seed tumor).

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13
Q

Adolescent renal cell carcinoma is associated with…

A
  • Prior chemotherapy (translocation subtype)
  • Sickle cell trait (medullary subtype)
  • Von Hippel-Lindau (clear cell subtype)
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14
Q

3 y/o with ultrasound demonstrating nephroblastomatosis and some necrotic regions…

A

Think Wilms tumor

Note: Uncomplicated nephroblastomatosis should not have any necrosis.

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15
Q

Imaging appearance of nephroblastomatosis

A

Focal homogenous renal mass

OR

Hypodense rind surrounding the kidney

Note: NO necrosis; any necrosis -> think Wilms.

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16
Q

Mesoblastic nephroma

A

A generally-benign fetal hamartoma of the kidney

Note: This is the most common neonatal renal tumor (80% diagnosed in the first month of life).

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17
Q

9 month old with a renal tumor that looks like Wilms…

A

Think mesoblastic nephroma (Wilms tumors are extremely rare before 1 year old)

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18
Q

Prenatal ultrasound demonstrates the left kidney to have numerous tiny cysts with a normal right kidney…

A

Think multi cystic dysplastic kidney (no actual functioning renal tissue)

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19
Q

Multicystic dysplastic kidney is associated with…

A

Abnormalities in the contralateral kidney (50%)

Note: Most commonly this is a UPJ obstruction.

20
Q

How can you differentiate between multi cystic dysplastic kidney and bad hydronephrosis?

A
  • In hydronephrosis, the cystic spaces communicate with the renal pelvis
  • Renal scintigraphy can be used in difficult cases (multi cystic dysplastic kidneys have no excretory function)
21
Q

Wilms tumors are associated with…

A
  • Beckwith-Wiedemann
  • Sotos
  • WAGR
  • Drash
22
Q

Beckwith-Wiedemann

A
  • Macroglossia
  • Wilms
  • Omphalocele
  • Hepatoblastoma
23
Q

Sotos

A
  • Macrocephaly
  • Mental retardation
  • Ugly face
24
Q

WAGR

A
  • Wilms
  • Aniridia
  • Genital anomalies
  • Growth Retardation
25
Q

Drash

A
  • Wilms
  • Pseudohermaphroditism
  • Progressive glomerulonephritis
26
Q

Wilms in a 1 y/o…

A

Look for other associated syndromes/anomalies:

  • Hemihypertrophy
  • Hypospadias
  • Cryptorchidism
27
Q

Wilms tumors are NEVER present prior to what age?

A

2 months (and extremely rare prior to 1 year)

28
Q

What are the common Wilms variants?

A
  • Clear cell (likes to cause lytic bone mets)
  • Rhabdoid (likes to cause aggressive rhabdoid brain tumors, horrible prognosis)
29
Q

What percentage of pts with a Wilms tumor will have bilateral Wilms tumors?

A

5-10%

30
Q

4 y/o with a cystic renal mass that protrudes into the renal pelvis…

A

Multilocular cystic nephroma

31
Q

Multilocular cystic nephromas are most common in what age groups?

A
  • Young boys (~4 y/o)
  • Middle aged women (~40s)

Note: Some people separate these into two different diseases: pediatric cystic nephromas and adult cystic nephromas.

32
Q

What is the most common renal malignancy of childhood?

A

Wilms tumor (followed by renal cell carcinoma)

33
Q

What is the most common subtype of renal cell carcinoma in children?

A

translocation carcinoma

Note: Clear cell carcinoma, which is the most common subtype in adults, is rare in kids.

34
Q

Kids with clear cell renal cell carcinoma should be screened for…

A

Von Hippel-Lindau

35
Q

Kids with translocation renal cell carcinoma often have a history of prior…

A

Cytotoxic chemotherapy

36
Q

Classic imaging appearance of translocation renal cell carcinoma

A

Solid, enhancing renal mass that is denser than renal cortex on non contrast CT and demonstrates expansile growth

37
Q

Multiple, bilateral, round, expansile renal masses in a 4 y/o…

A

Think lymphoma

38
Q

Lymphoma involving the kidneys in children is most often primary/secondary

A

Always secondary (hematogenous spread or direct invasion of lymphoma)

Note: Primary renal lymphoma is virtually nonexistent.

39
Q

Best initial imaging test for suspected bladder cancer in a kid

A

Bladder ultrasound (with a full bladder)

40
Q

What is the most common malignant bladder tumor in children?

A

Rhabdomyosarcoma

41
Q

Kid with bladder obstruction and bladder ultrasound demonstrates a large, nodular bladder mass…

A

Think rhabdomyosarcoma

Note: Specifically, the botryoid subtype has a nodular, bunch-of-grapes appearance.

42
Q

Most common location for pediatric rhabdomyosarcoma

A

Head/neck (specifically the orbit and nasopharynx)

43
Q

Which rhabdomyosarcoma subtype looks like a bunch-of-grapes?

A

Botryoid rhabdomyosarcoma

44
Q

What is the most common age rage for rhabdomyosarcoma

A

Bimodal: 2-4 and 15-17

45
Q

What percentage of rhabdomyosarcoma cases have metastatic disease at the time of presentation/diagnosis?

A

20%

46
Q

What is the most common atraumatic extra-testicular scrotal mass in a young male?

A

Paratesticular rhabdomyosarcoma

Note: If there was trauma, think hematoma.

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