Hi #MedTwitter
A recent case- discussion on #ASPNeph webinar #kidneymass in children intrigued me. Here’s what I learned.
#Nephtwitter #tweetorial
A recent case- discussion on #ASPNeph webinar #kidneymass in children intrigued me. Here’s what I learned.
#Nephtwitter #tweetorial
Do you know that most of the intra-abdominal masses originate from kidneys in children! (Potisek et al 2017)
Kidney masses
📌accounts for 60 % of abdominal masses
📌mostly benign and urologic in origin (Nelson 2018)
Kidney masses
📌accounts for 60 % of abdominal masses
📌mostly benign and urologic in origin (Nelson 2018)
What is the first step when you recognize a kidney mass in children?
Ans: B &C
💥It is important to exclude pseudo mass/ tumor
💥Most common causes-
📌Congenital anomalies: Prominent renal columns of Bertin, dromedary humps
📌Inflammatory: Focal pyelonephritis, abscess
📌Vascular: Renal artery aneurysm, AV fistula
📌Trauma/ Hematoma
💥It is important to exclude pseudo mass/ tumor
💥Most common causes-
📌Congenital anomalies: Prominent renal columns of Bertin, dromedary humps
📌Inflammatory: Focal pyelonephritis, abscess
📌Vascular: Renal artery aneurysm, AV fistula
📌Trauma/ Hematoma
💥Once pseudo masses are ruled out, differentiating benign from malignant is important
📌Cystic masses-usually benign
📌Imaging differentiates solid and cystic masses
📌Some cysts can be malignant
📌Bosniak Classification is helpful and is reliable in children
📌Cystic masses-usually benign
📌Imaging differentiates solid and cystic masses
📌Some cysts can be malignant
📌Bosniak Classification is helpful and is reliable in children
Which of the following has high malignant potential?
Ans: D. Bosniak 3
How to differentiate if a mass is originating from the kidney or not?
Ans: Claw sign
📌Determines if mass arises from solid structures
📌Refers to sharp angles on the either side of mass, formed by the surrounding normal parenchyma
📌Determines if mass arises from solid structures
📌Refers to sharp angles on the either side of mass, formed by the surrounding normal parenchyma
What is the most common benign kidney tumor in neonates?
Ans:💥 Mesoblastic Nephroma
📌Most common neonatal kidney mass
📌M>F
📌polyhydramnios, hydrops & preterm birth
📌US: the “ring” sign- concentric hyper & hypoechoic rims surrounding tumor
📌Path: interlacing fibro/myofibroblastic cell bundles interspersed with collagen & vessels
📌Most common neonatal kidney mass
📌M>F
📌polyhydramnios, hydrops & preterm birth
📌US: the “ring” sign- concentric hyper & hypoechoic rims surrounding tumor
📌Path: interlacing fibro/myofibroblastic cell bundles interspersed with collagen & vessels
What is the most common heritable benign tumor?
Ans: Angiomyolipoma
📌Rare Benign renal masses
📌80% with syndromes: TS, VHL, SW, NF, ADPKD
📌Macroscopic fat in CT/MRI
📌asymptomatic & <4cm➡️biannual/ annual follow-up with US/CT
📌Symptomatic or ≥4cm or B/L➡️surgery or arterial embolization
📌Rare Benign renal masses
📌80% with syndromes: TS, VHL, SW, NF, ADPKD
📌Macroscopic fat in CT/MRI
📌asymptomatic & <4cm➡️biannual/ annual follow-up with US/CT
📌Symptomatic or ≥4cm or B/L➡️surgery or arterial embolization
Other benign tumors are-
📌Metanephric stromal tumors
📌Metanephric adenoma
📌Cystic nephroma
📌Ossifying Renal Tumor of Infancy (ORTI)
📌Reninoma
📌Metanephric stromal tumors
📌Metanephric adenoma
📌Cystic nephroma
📌Ossifying Renal Tumor of Infancy (ORTI)
📌Reninoma
A 2.5 years old boy was brought to the pediatrician because mother noticed right sided abdominal distension. He was asymptomatic. What is the most common kidney tumor in the first decade?
Ans: Wilms tumor
📌90% of childhood kidney cancers
📌2-5 yrs of age
📌90% Sporadic
📌10% Hereditary: WAGR (WT1), Denys-Drash (WT1, 11p13), Beckwith Wiedemann (WT2, 11p15), Perlman, & Sotos
📌unilateral-75-95%
📌Symptoms: Abdominal pain, distention, HTN, malaise or hematuria
📌90% of childhood kidney cancers
📌2-5 yrs of age
📌90% Sporadic
📌10% Hereditary: WAGR (WT1), Denys-Drash (WT1, 11p13), Beckwith Wiedemann (WT2, 11p15), Perlman, & Sotos
📌unilateral-75-95%
📌Symptoms: Abdominal pain, distention, HTN, malaise or hematuria
💥 Imaging: Large, well defined intrarenal mass with uniform echogenicity in the US (often with necrosis, old hemorrhage, & Calcification ~15%
💥Path: Triphasic appearance -stromal, epithelial, & blastemal elements
💥Rx: Nephrectomy + adjuvant chemotherapy
💥Path: Triphasic appearance -stromal, epithelial, & blastemal elements
💥Rx: Nephrectomy + adjuvant chemotherapy
A 14 year old AA boy presents with painless gross hematuria. Which of the following associations should concern you?
Ans: D - s/o renal medullary carcinoma
📌Most common tumor of second decade
📌Sickle cell trait or heterozygous sickle cell disease
📌Painless hematuria, flank pain & palpable mass
📌Overexpression of transcription factor E3 (TFE3):Xp11.2 translocation involves gene fusion
📌Most common tumor of second decade
📌Sickle cell trait or heterozygous sickle cell disease
📌Painless hematuria, flank pain & palpable mass
📌Overexpression of transcription factor E3 (TFE3):Xp11.2 translocation involves gene fusion
Malignant tumors are rare in children (6-7% of all childhood tumors)
Other malignant kidney tumors are-
📌Clear cell sarcoma
📌Nephroblastomatosis
📌Rhabdoid tumor
Other malignant kidney tumors are-
📌Clear cell sarcoma
📌Nephroblastomatosis
📌Rhabdoid tumor
That’s All Folks…
For case-based discussion on this topic logon to @ASPNeph December radiology webinar
#FellowFOAMgroup @drM_sudha @RoshanPGeorgeMD @priti899 #pediatricnephrology
For case-based discussion on this topic logon to @ASPNeph December radiology webinar
#FellowFOAMgroup @drM_sudha @RoshanPGeorgeMD @priti899 #pediatricnephrology
جاري تحميل الاقتراحات...