The Imaging Manifestations,Clinical Characteristics and Misdiagnosis of Primary Adrenocortical Carcinoma and Primary Adrenocortical Adenoma in Children
Objective The purpose of this study was to highlight the imaging findings of primary adrenocortical carcino-ma(PAC)and primary adrenocortical adenoma(PAA)on CT and MRI,to summarize the clinical characteristics and the factors causing misdiagnosis.Methods We retrospectively analyzed the Imaging characteristics of the patients with histo-logically confirmed PAC and PAA diagnosed.All the three patients with PAC were female,aged from 1 year and 1 months to 12 years and 11 months,with an average age of(5.06±5.56)years;among the five patients with PAA,there were 2 males and 3 females,aged from 9 months to 14 years,with an average age of(4.92±4.78)years.Preoperative plain and enhanced CT were performed in all 3 cases with PAC,Preoperative plain and enhanced MRI were performed in 1 case.Pre-operative plain and enhanced CT were performed in 3 cases with PAA.In addition,only CT plain scan in 1 case,and CT was not performed in the other case,however,preoperative plain and enhanced MRI were performed in these 2 cases.Re-sults 3 cases of PAC,1 was located in the left side and 2 in right side.5 cases of PAA,3 were located in the left side,2 was in right side.The lesions were circular in 5 cases,elliptical in 2 cases and lobulated in 1 case.The maximum diame-ter of PAC lesions ranged from 43 mm to 61 mm,with an average of(46.67±10.53)mm,The maximum diameter of PAA lesions ranged from 21 mm to 39 mm,with an average of(34.60±8.31)mm.Among the 3 patients with PAC,the reasons for the first visit were clitoris enlargement in 1 case,facial rash in 1 case,overeating in 1 case,and occasional occurrence in 1 case.CT examination mainly showed isodensity,cystic lesions/necrotic areas were seen,and the larger the volume,the more obvious.Calcification was found in 1 case,and no bleeding or bone destruction was observed.All lesions showed uneven enhancement on enhanced scan,and the enhancement degree of each stage was lower than that of renal parenchy-ma.Progressive enhancement in 1 case,and most obvious in the venous stage,decreased in the delayed stage in the other 2 cases.The small enhanced vascular shadows passing through the tumor,and no enhancement in cystic and necrotic areas.Left inferior lobe metastases with filling defect in inferior vena cava in 1 case.MRI examination showed equal or slightly lower signal on T1 WI and slightly higher signal on T2WI(compared with liver),scattered with irregular highly signal cystic degeneration/necrosis on T2WI,and obviously limited diffusion on DWI in 1 case.The inverse phase signal is slightly re-duced,and the signal strength index(SII)is about 9％.The enhanced scan showed uneven and obvious enhancement.Lesion occupying effect is obvious,adjacent to the liver parenchyma,right kidney is extremely obvious pressure.The infe-rior vena cava had a round filling defect.Retroperitoneal lymph node enlargement.Among 5 patients with PAA,the reasons for the first visit were progressive obesity in 1 case,breast enlargement in 1 case,abdominal pain and hematochezia in 1 case,and occasional occurrence in 2 cases.CT examination mainly showed that the isondensity image was dominant in 4 case,and cystic lesion/necrosis area was found inside,and the larger the volume was,the more obvious it was.However,calcification,hemorrhage and bone destruction were not observed.All lesions showed uneven enhancement on enhanced scan,and the enhancement degree of each stage was lower than that of renal parenchyma,and the enhancement was most obvious in the venous stage,and the delayed stage decreased somewhat.Small enhanced vascular shadows passing through the tumor,and no enhancement in cystic and necrotic areas.No distant metastasis was observed.MRI examination showed a slightly lower signal on T1 WI and a slightly higher signal on T2WI(compared with liver)in 2 cases.One had limited DWI diffusion and the other had no DWI.The reverse phase signals were all reduced,with SII of about 11％and 30％respec-tively.The enhanced scan showed mild uneven enhancement in 1 case and mild uniform enhancement in the other case.A-mong the 3 cases of PAC,2 cases were misdiagnosed as neurogenic tumor(NT),and 1 case was diagnosed correctly.Of the 5 PAA cases,2 were misdiagnosed as NT,1 was misdiagnosed as PAC,and the other 2 were diagnosed correctly.Conclusion The incidence of PAC and PAA in children is low,the clinical features,imaging and pathological findings are similar,and the lack of understanding of their signs are the main reasons for misdiagnosis.It is helpful for accurate di-agnosis to comprehensively understand and the imaging manifestations of PAC and PAA and analyze the causes of misdiag-nosis.
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