Diffuse hyperplout nephroblastoma 1 case

Children and boys, 11 months and 17 daysBecause of the discovery of painless abdominal swelling 1 week admission, no fever, blood urine and other discomfortPhysical examination: generally good condition, normal growth and development, abdominal bulgingThere is no similar family genetic historyAbdominal CT show: the volume of the double kidneys significantly increased, the size of about 12.6 cm x 10.8 cm x 6.9 cm (left), 12.1 cm x 8.5 cm x 7.4 cm (right), the contour is generally normal, the inside can be seen with multiple diffuse size of soft tissue knots and lumps, The boundary is unclear, the enhanced scanning part of the lesions are unevenly reinforced, the degree of reinforcement is significantly weaker than the kidney substance, the normal strengthening of the residual syllable smaller kidney substance between nodules and lumps and the surrounding areas, the residual normal kidney essence is obviously pressured thin, the kidney collection system is crushed and deformed, local visible mild expansion, which does not see the signs of filling defectsSwollen lymph nodes were not seen after the abdominal cavity and peritoneal (Figure 1, 2)Figure 1,2 reinforced CT shows double kidney diffuse size inequality nodules or lump shadow, double kidney contour is generally normal, part of the visible uneven reinforcement (white arrow), right kidney collection system pressure, water(Figure 1 is horizontal, and Figure 2 is the crown)right renal puncture pathology: diffuse hyperplial nephroblastoma disease, tumor cell size is more consistent, most of the chrysanthemum-like or small tube-like arrangement, part of the region interstitial rich, composed of loose shuttle cells, not mature renal tube and renal glomerular structure (Figure 3,4)3, 4 right renal pathology images show tumor cell size is more consistent, most of the chrysanthemum-shaped or small tube-like arrangement, part of the region melialist rich, composed of loose shuttle cells, no mature renal tube and kidney ball structurediscussionrenal cell tumor (nephrobsos, NS) clinically rare, is a benign lesions, but the existence of malignant tumor Diffuse hyperplallone nephroblassathroma disease (DHNS) refers to the fact that a single or double kidney is essentially filled with renal-derived tissue in whole or in partPathology: Renal residuals (nephrogenic rests, NR) are renal germs that are abnormally residual in the kidneys and represent the heterogeneous proliferism cells of the kidney development processNS refers to the multi-cooked, diffuse distribution of NR, divided into four types: leaf circumlo, intra-leaf, hybrid and full small leaf typeBoth NR and NS are known as WT pre-degeneration lesions, and the rate of adverse change is about 25% to 40% and 46%, respectivelyIt is reported that the ye-week NS severity rate is higher, especially the diffuse hyperploric leaf-week NS Clinical performance: NS can occur at any age, but most common in infants, generally in abdominal puffs and painless lumps, usually more, about 40% of cases are two sides, and WT two-sided incidence rate of about 6% to 7%, one-sided incidence of about 7% It is worth noting that when one side of the kidney seamount snr nS, the risk of the onset of THE onside kidney is obviously increased when the side kidney is generally combined with the NR under the mirror imaging performance: In ultrasound, NS is shown as an increase in kidney volume, compared with the renal cortex, the lesions are uniform and equal echo or low echo Perlman EJ and other reported 52 cases of leaf-shaped NS, in CT, its performance is an increase in kidney volume, but the contourofe of the kidney remains unchanged, the lesions are multiple, density is more homogeneous nodules, often located in the renal essential near cortical region, smaller lesions are easy to miss On MRI, compared with the renal cortex, the lesions Of T1WI showed equidistant or slightly lower signals, T2WI showed equal, low or high signal, enhanced scanning more than no reinforcement or mild reinforcement, the degree is significantly lower than the kidney substance the diagnosis of diagnosis : because Of the different biological behaviors of NS and WT biological behavior, their clinical treatment options are very different, so the diagnosis and differential diagnosis of the two, as well as to determine whether NS is combined with WT are very important In clinical work, it is mainly based on its imaging and pathological characteristics In general, NS kidney contours remain unchanged, multiple nodules, texture is homogeneous, no false envelope, enhanced scanning more than mild homogeneity reinforcement, while the latter is generally larger, enlarged, expansion, exogenous growth, and the existence of false envelope membrane, enhanced scanning is significantly uneven reinforcement, see Table 1 Table 1 NR, NS and WT clinical and major visual performance the imaging characteristics of this case are mainly characterized by the double kidney contours are generally normal, the internal coverage of varying sizes of nodules and lumps, most of the nodules are mildly reinforced, some lesions see uneven reinforcement Puncture pathology is shown as DHNS It is worth noting that the pathological examination is the diagnostic gold standard, but the site and quantity of its sampled tissue is limited, only local specimens for the diagnosis of the disease is limited, often lead to WT false negative diagnosis, therefore, intuitive and comprehensive imaging examination and image-guided biopsy is essential, for the larger size of the lesions, and the texture of the less homogeneous part, should be highly alert to the possibility of conversion or merging WT, should be concerned and biopsy sampling In addition to identifying nR and WT, NS also needs to be identified with diseases such as congenital mesoblastic nephroma, CMN, renal transparent cell cell cella of renal, CCSR, and renal transverse fibroids (rhabdoids of the renal, RTR) CMN mainly occurs in newborns less than 3 months old, and NS median age of about 16 months; CCSR is blood-rich tumor, necrosis and bleeding is common, enhanced scanning is "tiger spot signs" and prone to bone metastasis, while NS is characterized by texture homogenization, and enhanced scanning reinforcement is not obvious; RTR is unevenly characterized by mass, the edge visible fine line calcification, peripheral fascia is a new layer of conjuncital, and the kidneys are seen in a new layer of visible blood therapy: The main treatment for NS is chemotherapy, which can reduce the number of NS, reduce the volume, and delay the conversion of NS to WT In perlmanEJ et al., the average time between initial nS and WT in children with chemotherapy was 35 months, compared with about 6.5 months for children who did not receive chemotherapy If the patient develops WT during NS chemotherapy, it is also possible to retain the kidney unit for surgery in a word, NS has certain characteristics in imaging performance, can carry out diagnosis and differential diagnosis, has important value to the formulation of treatment plan, its final diagnosis still needs to be combined with pathological examination