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Onc - Renal Flashcards

1
Q

Where does oncocytoma originate from?

How does it look grossly?

What syndrome is it associated with?

How do you distinguish from other renal neoplasms?

A

Epithelial tumor. Originates from intercalating cells in cortical collecting system

It’s well circumcscribed, non-encapsulated, central stellate scar

May occur as part of Birt-Hogg Dube syndrome

May use sestamibi SPECT/CT to distinguish from other tumors, need further study

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2
Q

What syndrome is AML associated with?

A

Tuberous Sclerosis

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3
Q

What medical treatment can you use to treat AML?

What class of medication is this?

What are the adverse events associated?

A

Everolimus, an mTOR inhibitor

Stomatitis, nasopharyngitis, acne like skin lesions

If stop medication, tumor may resume growth

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4
Q

What features are associated with AML hemorrhage?

Why is risk higher during pregnancy?

A

Tumors >4cm and intralesional vascular aneurysms >5mm

Estrogen-receptor-beta is present in all AMLs and ER-alpha in 30%, so increase in growth and risk of hemorrhage in pregnancy or if hormone therapy

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5
Q

What is pathognomotic finding on CT for AML?

A

Macroscopic fat on CT, ROI = -10U

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6
Q

What are mesenchymal tumors?

A 
AML
Leiomyoma
Hemangioma
Reninoma
Schwannoma
Lymphangioma
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7
Q

What is classic presentation of reninoma?

A

HTN, HYPOkalemia, elevated serum renin level

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8
Q

What are features of Von Hippel Lindau Syndrome?

What is the genetic mutation?

A

Clear cell RCC, often bilateral and multifocal

Retinal angiomas, endolymphatic sac tumors, benign CNS hemangioblastomas, pancreatic cysts and islet tumors, epididymal cystadenomas, and pheochromocytomas

Mutated VHL suppressor gene (3p25-26), unable to form E3 ubiquitous ligate complex which regulates degradation of regulatory proteins ie hypoxia inducible factor (HIF) -> overaccumulation of HIF results in upregulation of VEGF

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9
Q

What are the 4 well described hereditary clinical kidney cancer syndromes?

Are they autosomal dominant or recessive?

What are 2 more?

A
  1. VHL
  2. Hereditary Papillary Renal Cell Carcinoma (HPRCC)
  3. Hereditary Leiomyoma Renal Cell Carcinoma (HLRCC)
  4. Birt-Hogg-Dube Syndrome (BHD)

All are autosomal dominant

  1. BAP1 tumor predisposition syndrome
  2. Hereditary paraganglioma-pheochromocytoma syndromes
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10
Q

What are features of HPRCC?

What is the genetic mutation for this syndrome?

A

Hereditary papillary renal cell carcinoma

Bilateral multifocal type I papillary RCC, no extrarenal findings

Missense mutation of cMET proto-oncogene, 7q31

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11
Q

What are features of HLRCC?

What is the genetic mutation?

A

Papillary type II RCC or HLRCC-associated RCC, very aggressive tumor requiring early surgery with wide resection, often metastatic

Painful cutaneous and uterine leiomyomas

1q42-44, site of Fumarate Hydratase tumor suppressor gene

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12
Q

What are features of BHD?

What is genetic mutation?

A

Birt-Hogg-Dube syndrome

Bilateral, multifocal chromophobe RCC, oncocytoma or hybrid renal tumors

Fibrofolliculomas of head and neck, pulmonary cysts, spontaneous PTX

17p11.2, encodes tumor suppressor gene product Folliculin

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13
Q

What are features of BAP1 tumor predisposition syndrome?

What is genetic mutation?

A

Unifocal ccRCC (or other)

Other associated cancers include uveal and cutaneous melanomas and mesotheliomas

BRCA1 associated protein 1

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14
Q

What are features of HPPS?

What is mechanism/mutation?

A

Hereditary paraganglioma-pheochromocytoma syndromes

Unifocal tumor, vacillated cytoplasm and cytoplasmic inclusions with pale eosinophilia fluid

Associated tumors are paragangliomas, pheo, and gastrointestinal stromal tumors (GIST)

Mutation in succinct dehydrogenase complex

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15
Q

what are the 6 histologic classifications of RCC?

A
  1. Clear cell RCC - most common, from proximal convoluted tubule, loss of 3p
  2. Papillary RCC - Type I a/w polysome 7 and 17, loss of Y and MET mutations; Type II a/w mutation in fumarate hydra tase; think b/l even if not familial
  3. Chromophobe - histologically similar to oncocytoma, arises from collecting duct, mutation in TP53 and PTEN
  4. Collecting duct carcinoma - arises from collecting duct
  5. Renal medullary carcinoma - young AA adults with sickle cell trait
  6. Unclassified RCC - Xp11 translocation
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16
Q

What is first line therapy for favorable risk relapse or stage IV clear cell RCC?

A
  • axitinib (VEGF/C-KIT inhibitor) + pemrbolizumab (PDL1 inhibitor, aka Keytruda)
  • pazopanib (VEGF/C-KIT inhibitor)
  • sunitinib (VEGF/C-KIT inhibitor)
17
Q

What is first line therapy for intermediate/unfavorable risk relapse or stage IV clear cell RCC?

A
  • ipilimumab + nivolumab
  • axitinib + pemprolizumab
  • cabozantinib
18
Q

What renal tumor is associated with sickle cell trait?

A

Renal medullary carcinoma

19
Q

When do you use adjuvant therapy for RCC?

What is the treatment?

What is the trial that supported this?

A

Clear Cell RCC
Stage III disease
High risk for Relapse

Adjuvant sunitinib (TKI) x1 year

S-TRAC, showed higher DFS

20
Q

When do you repeat biopsy after ablative therapy for stage T1a renal cancer?

A

Per NCCN, if radiographic evidence of progressive increase in size with or without contrast enhancement, new modularity in or around treated zone, failure of the treated lesion to regress over time, or evidence of satellite or port site lesions

21
Q

When is cytoreductive nephrectomy before systemic therapy indicated?

A
  • if lung only mets
  • good prognostic features
  • good performance status
22
Q

What syndromes are associated with papillary RCC?

A

Hereditary papillary RCC (Type 1) aka HPRCC

Hereditary leiomyomitosis RCC (Type 2); associated with uterine and skin leiyomyomas`

23
Q

What features/anomalies do you see with VHL?

A 
Clear cell RCC
Renal cysts
Hemangioblastomas of the central nervous system
Retinal angiomas
Pheochromocytoma
24
Q

What is first line therapy for stage IV non-clear cell RCC?

A

sunitinib

Collecting duct: gem+cis or gem + carbo

25
Q

What is first line therapy for poor prognostic, stage IV non-clear cell RCC?

A

temsirolimus

26
Q

how do you distinguish AML from liposarcoma

A

AML doesn’t come from RP and doesn’t push on kidney

27
Q

What is a renal nephrometry score? What’s the max?

A

complexity of a mass. Likelihood of intervention vs. observation

Max score 12

28
Q

What diagnosis can be made:
Pulmonary cyst
AML
lymphangioleiomyotosis (LAM)

A

Tuberous sclerosis

29
Q

What is the indication for excision of AML?

A

Large size

Young, female

30
Q

What is subsequent treatment for metastatic clear cell?

A

cabozanitinib
nivolumab
axitinib
lenvatinib + everolimus

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