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04. RCC (937) > Renal Syndromes and Tidbits > Flashcards

Renal Syndromes and Tidbits Flashcards

1
Q

Periungual fibromas

A

Flesh-colored papules near the nail bed

Tuberous sclerosis

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2
Q

Fibrofolliculomas

A

Small white or flesh-colored papules on face, neck, back or upper trunk

Birt-Hogg-Dubé Syndrome

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3
Q

Trichilemmomas

A

Wart-like flesh colored papules typically on face or dorsum of hands and feet

PTEN Hamartoma (Cowden) Syndreom

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4
Q

Cutaneous Leiomyoma

A

Small pink0purple papules in same location as hair follicles
Often painful

HLRCC –> Hereditary leiomyomatous RCC

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5
Q

Papillomas

A

Hard, smooth flesh-colored papules usually on face, lips, mouth, hands and feet

PTEN Hamartoma (Cowden) Syndrome

(also has trichilemmomas, glans macules, and keratosis)

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6
Q

On workup of a renal mass…
If UA has proteinuria, get quantitative measure of urine protein

A
  • Assign a CKD stage urine proteinuria level and GFR
  • If <45 mL/min GFR before treatment, send to nephrology
  • If after treatment you expect GFR will be <30, send to nephrology
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7
Q

For renal masses, consider biopsy when:

A
  1. Suspicion that mass is infectious, inflammatory, AML, lymphoma, or a tumor metastasis to kidney
  2. Nephrectomy candidate who chooses surveillance, ablation or embolization
  3. To confirm diagnosis in metastatic patient who will not have cytoreductive nephrectomy before systemic chemotherapy
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8
Q

HMB-45 stain of renal tumor

A
  • Metastatic melanoma
  • AML
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9
Q

List of benign renal masses

A
  • Simple renal cyst (most common)
  • Papillary adenoma (most common SOLID benign)
  • Pseudotumor
  • AML
  • Oncocytoma
  • Juxtaglomerular tumor
  • Multilocular cystic nephroma
  • Mesoblastic nephroma
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10
Q

RCC TNM staging

A
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11
Q

Cancers that met to kidneys

A
  1. Lymphoma/leukemia
  2. Lung
  3. Breast
    Less common: stomach, colon, cervix, melanoma
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12
Q

Renal pseudotumors

A
  • Look like solid masses on some imaging, but are normal parenchyma

Examples: column of bertin, fetal lobulation, dromedary hump, hilar lip or uncus, nodular compensatory hypertrophy

CT/MRI with contrast or DMSA renal scan to tell apart
- On DMSA scan, pseudotumors will have normal uptake but true tumors will have decreased isotope uptake

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13
Q

Dromedary hump

A
  • A focal bulge at the id-lateral kidney thought to be from downward pressure from spleen or liver during development
  • More common on left
  • A pseudotumor
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14
Q

Genetic counseling in renal cancer recommended in the following patients:

A
  1. Dx less than or equal to 46yo
  2. Multifocal or bilateral renal masses
  3. Tumor pathology suggests genetic syndrome (ex: mix of RCC and oncocytoma –> BHG)
  4. Personal history suggests genetic syndrome
  5. Family history suggests genetic syndrome
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15
Q

von Hippel Lindau (gene and type)

A

VHL
Clear cell RCC

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16
Q

BAP1 Syndrome (gene and type)

A

BAP1
Clear cell RCC

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17
Q

CHEK2 Syndrome (gene and type)

A

CHEK2
Clear cell RCC

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18
Q

Chromosome 3 translocation syndrome (gene and type)

A

Chromosome 3
Clear cell RCC

19
Q

PTEN Hamartoma (Cowden) Syndrome (gene and type)

A

PTEN
Clear Cell RCC

20
Q

Hereditary Papillary RCC Type I (gene and type)

A

MET
Papillary RCC Type I

21
Q

Hereditary Leiomyomatosis RCC (gene and type)

A

FH
Papillary RCC Type 2

Aggressive –> surveillance not recommended even if tumors are small

22
Q

Birt-Hogg Dubé (BHD) (gene and type)

A

FLCN (on 17)

RCC mixed with oncocytoma, oncocytoma alone, chromophobe RCC alone, clear cell rarely

23
Q

Succinate dehydrogenase (SDH) deficiency RCC (gene and type)

A

SDH subunits B, C and D
Various types of RCC

Aggressive - no surveillance even for small tumors

24
Q

Tuberous sclerosis (gene and type)

A

TSC1, TSC2
Various RCC, AML

25
Q

Birt-Hogg-Dubé Syndrome
(presentation, cancer type, gene)

A

Skin fibrofolliculomas after age 20, air-filled pulmonary cysts, spontaneous pneumothorax, renal tumors

25% develop renal tumors –> most bilateral and multiple per kidney
- clear cell, chromophobe, oncocytoma, mix of oncocytoma and RCC

BHD is AD - FLCN on 17

26
Q

PTEN Hamartoma (Cowden) Syndrome

A

Mutation in PTEN gene
RCC develops in 35% of cases (usually ccRCC)

Associated with RCC, breast cancer, endometrial cancer, thyroid cancer, glans macula, skin lesions (papillomas, trichilemmomas, keratosis), macrocephaly and autism

27
Q

Tuberous Sclerosis

A

Triad seen in 30%: mental retardation, seizures, adenoma sebaceum
AD causes by TSC1 (on 9) or TSC2 (on 16) mutation
Hamartomas - retina, brain, skin, kidney (AML), lung, heart (cardiac rhabdomyomas)

Uro manifestations: renal cysts, AML, RCC
- 60% with AML, most multiple
- 2% RCC of any type

Adenoma sebaceum, ash-leaf spots, shagreen patches, ungual fibromas

28
Q

von Hippel Lindau

A

AD, mutation of VHL gene on 3p

Non-uro: cerebellar hemangioblastoma, spinal HAB, retinal angioma
Uro: renal cysts (75%), ccRCC (50%), pheo (15%), epididymal cystadenoma (10%), epididymal cysts (7%)

VHL –> mutation 3p –> ccRCC is most common

RCC bilateral and multifocal, early (<40yo)
All renal cysts (even simple) have malignant potential (OK for surveillance until 3cm)

29
Q

Papillary Adenoma

A

Benign
- Usually incidental finding during autopsy
- <5mm, but microscopically looks the same as low grade papillary RCC
- Adenomas arise from proximal tubule

30
Q

Oncocytoma

A

Benign
Arises from collecting duct
Composed of oncocytes –> cells with eosinophilic granular cytoplasm
Most common in 40-60 yo M
Can’t be distinguished from RCC on imaging –> tx as RCC
“Spoke wheel” pattern on arteriogram, but nonspecific
Grossly: tan mass with a central scar and fibrous capsule
Micro: nests of polygonal cells with granular eosinophilic cytoplasm
RCC may coexist with oncocytoma

Tx of choice = surgical excision

31
Q

Juxtaglomerular Tumor

A

Rare but benign
Renin-secreting tumor, arises from JG apparatus
Usually <3cm in diameter
Presents young (<25yo), diastolic HTN, headaches, increased renin, hyperaldosteronism with hypokalemia

Treatment = nephron sparing excision

32
Q

Angiomyolipoma (AML)

A

Benign
Will stain positive for HMB-45
Occurs in 40-60yF, 60% of TS patients
Most AML patients do NOT have TS
On CT, it enhances: -20 to -80 HFU (fat), homogenous, not calcified or cystic

Everolimus = approved to shrink AML in TS patients not having surgery

When >4cm or sxs, can do embolization, ablation or excision

33
Q

Sarcomatoid elements in RCC

A

May be present in any subtype of RCC

Indicated high-grade tumor and worse prognosis

34
Q

Trichloroethylene

A

A chlorinated solvent

Increases risk of RCC with exposure

35
Q

Clear cell (conventional) RCC

A

Most common primary renal malignancy in adults

Arises from proximal tubule

Associated with loss of 3p, the short arm of chromosome 3

Most common type of RCC in patients with vHL

36
Q

Adenoma Sebaceum

A

Pink or red papules on cheek bones or nasolabial folds

Tuberous sclerosis

37
Q

Chromophobe RCC Classification

A

Arises from collecting duct
Associated with multiple chromosomal losses
Similar to oncocytoma under the microscope

38
Q

Chromophil (papillary) RCC classification

A

Classic pathologic feature = papillary architecture

Type I = basophilic cytoplasm, low grade
Type II = eosinophilic cytoplasm, high grade, worse prognosis

Arises from proximal tubule
Associated with polysomy (of 7 and 17), MET mutations, loss of Y chromosome

Papillary RCC = most common renal cancer in patients with chronic renal failure on dialysis

39
Q

Collecting Duct Carcinoma Classification

A

AKA Bellini duct carcinoma
Rare, highly malignant, arises from collecting duct usually in renal medulla or papilla
40% present with mets
Micro: hobnail cells lining tubular spaces
5-year survival = rare

40
Q

Renal medullary carcinoma

A

RARE form of collecting duct carcinoma

Young adults of African ancestry with sickle cell trait

Typical age = 10-39
Tumor rarely confined to kidney, rarely respond to chemo/rads
Poor prognosis; mean survival after nephrectomy = 15 weeks

Only tumor with racial predilection

41
Q

Paraneoplastic syndromes associated with RCC

A

10-20% will have paraneoplastic syndrome

  • Elevated ESR, alk phos
  • Weight loss, cachexia
  • Fever
  • Anemia
  • HTN - from renin produced by tumor
  • Hypercalcemia - from a PTH-like substance produced by the tumor
  • Stauffer’s syndrome = hepatic dysfunction; a reversible hepatitis associated with RCC that has NOT metastasized to the liver
  • Polycythemia - from erythropoietin produced by tumor
42
Q

Risk of RCC met based on size at presentation

A

Both lymphatic and hematogenous spread
A solitary met is present in 1% of cases –> if there are mets, they are multiple

<3cm - 4% risk
3-4 cm - 7% risk
4-7 cm - 16% risk
7-10 cm - 30%
10-15 cm - 41%
>15 cm - 51% risk

43
Q

RCC metastatic sites (most to least common)

A

Lung
Bone (most common in spine)
Regional LN
Liver
Adrenal
Contralateral kidney
Brain

04. RCC (937) (10 decks)

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