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Urology In-Service/Board Review > 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
Birt-Hogg-Dubé Syndrome (presentation, cancer type, gene)
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
PTEN Hamartoma (Cowden) Syndrome
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
Tuberous Sclerosis
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
von Hippel Lindau
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
Papillary Adenoma
Benign - Usually incidental finding during autopsy - <5mm, but microscopically looks the same as low grade papillary RCC - Adenomas arise from proximal tubule
30
Oncocytoma
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
Juxtaglomerular Tumor
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
Angiomyolipoma (AML)
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
Sarcomatoid elements in RCC
May be present in any subtype of RCC Indicated high-grade tumor and worse prognosis
34
Trichloroethylene
A chlorinated solvent Increases risk of RCC with exposure
35
Clear cell (conventional) RCC
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
Adenoma Sebaceum
Pink or red papules on cheek bones or nasolabial folds Tuberous sclerosis
37
Chromophobe RCC Classification
Arises from collecting duct Associated with multiple chromosomal losses Similar to oncocytoma under the microscope
38
Chromophil (papillary) RCC classification
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
Collecting Duct Carcinoma Classification
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
Renal medullary carcinoma
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
Paraneoplastic syndromes associated with RCC
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
Risk of RCC met based on size at presentation
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
RCC metastatic sites (most to least common)
Lung Bone (most common in spine) Regional LN Liver Adrenal Contralateral kidney Brain

Urology In-Service/Board Review (12 decks)

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