Renal Cystic Disease, Part IV Flashcards
Simple Cysts and more
Simple Cysts
Thought to originate from distal convoluted tubule or collecting ducts
Increasing prevalence with increasing age
Male-to-female predominance: 2 to 3:1
Associated with smoking, HTN, reduced renal mass, increased body mass index, reduced kidney function
Similar to ADPKD, circulating levels of copeptin levels (validated surrogate for vasopressin) is associated with kidney length and function.
Simple Cysts
Generally asymptomatic, but may be associated with HTN, erythrocytosis
Infected cysts may develop thickened walls, calcifications.
Large cysts associated with severe HTN may be considered for drainage.
Kidney transplantation: Growth rate of simple cysts does not increase with kidney donation whether they are in the donor or recipient. Simple cysts do not adversely affect renal outcomes
Acquired Cystic Kidney Diseases
Conditions Associated with Acquired Cystic Kidney Diseases
Chronic hypokalemia: cysts typically localized to renal medulla; resolution possible after correction of hypokalemia (e.g., adrenalectomy for functioning tumors)
Acquired Cystic Kidney Diseases
Conditions Associated with Acquired Cystic Kidney Diseases
formacao de cistos em rins atroficos e assoc a drc
SEGUIMENTO RADIOLOGICO =RISCO 3X MAIOR DE DESENVOLVER NEOPLASIA
CKD-associated acquired cystic kidney diseases (ACKD)
Development of >3 to 5 macroscopic cysts in individuals without a hereditary cystic disease is required for the diagnosis of ACKD. ACKD typically involves >25% of renal parenchyma.
Acquired Cystic Kidney Diseases
Conditions Associated with Acquired Cystic Kidney Diseases
Increased risks with CKD vintage, 80% to 100% have ACKD by 10 years on dialysis.
May regress with successful kidney transplant
Acquired Cystic Kidney Diseases
Conditions Associated with Acquired Cystic Kidney Diseases
ACKD may transform into adenomas and RCC
- Unlike sporadic clear cell RCC, ACKD-associated RCC are typically multifocal and bilateral with lower proliferative activity and metastatic disease.
- ACKD-associated RCC are histologically distinct with eosinophilic cytoplasm with calcium oxalate crystal deposits in cribriform, tubulocystic, and papillary architecture.
Acquired Cystic Kidney Diseases
Conditions Associated with Acquired Cystic Kidney Diseases
Annual ultrasound screening (or at least 3 years after onset of dialysis with frequency increased to every 1 to 2 years if ACKD is present) is indicated in ESRD patients with good life expectancy. Interpretation is generally based on Bosniak classification
SEGUIMENTO RADIOLOGICO =RISCO 3X MAIOR DE DESENVOLVER NEOPLASIA.
Acquired Cystic Kidney Diseases
Bosniak classification of cysts (initially defined for CT, now often used for ultrasound):
bosniak I e II sao cistos confirmados como cistos simples-sem necessidade de seguimento
iif=follow up
bosniak 3 e 4 sao sugestivos de neoplasia
I: Benign simple cyst; hairline thin wall; no septation, calcification, or solid components; no enhancement with contrast CT
conteudo hipoecoico homogeneo, paredes finas, reforço acustico posterior- nao precisa complementar c tc
Acquired Cystic Kidney Diseases
Bosniak classification of cysts (initially defined for CT, now often used for ultrasound):
II: Benign cyst with a few hairline thin septations ± “perceived” enhancement may be present; fine calcification in cyst wall or septation(s). Uniformly high-attenuation lesions < 3 cm, well marginated; no enhancement with contrast CT
Acquired Cystic Kidney Diseases
Bosniak classification of cysts (initially defined for CT, now often used for ultrasound):
IIf (f is for follow-up): Cysts with multiple hairline thin septa or minimal smooth thickening of wall/septa; “perceived” enhancement of septa/wall; calcification of wall/septa that may be thick and nodular; no measurable contrast enhancement. >3cm
seguimento semestral no primeiro ano, anual por até 5 anos
Acquired Cystic Kidney Diseases
Bosniak classification of cysts (initially defined for CT, now often used for ultrasound):
III: “Indeterminate” cystic masses with thickened irregular or smooth wall/septa; measurable enhancement. Contrast enhancement implies vascularized mass, thus possible malignancy. Although possibly benign, surgery is generally indicated.
Acquired Cystic Kidney Diseases
Bosniak classification of cysts (initially defined for CT, now often used for ultrasound):
IV: Malignant cystic masses that also contain enhancing soft tissue components
Acquired Cystic Kidney Diseases
General management per Bosniak classification:
Class I and II are benign. Six- to twelve-month follow-up may be warranted in ESRD patients.
Class IIf requires follow-up within 3 to 6 months.
Class III and IV are considered malignant until proven otherwise. Surgical evaluation is required.
Management: bed rest, analgesics; if no resolution, consider renal embolization or laparoscopic nephrectomy.
Multilocular Cystic Nephroma, a.k.a. Papillary Cystadenoma, Benign Cystic Nephromas
Well-circumscribed, multiseptated, generally benign, cystic lesions typically classified as Bosniak III on CT that require surgery to exclude malignancy.
Multilocular Cystic Nephroma, a.k.a. Papillary Cystadenoma, Benign Cystic Nephromas
Thought to arise from hamartomatous origin, embryonic metanephric blastema, or variant of Wilms tumor.
