Imaging patterns of fatty liver in pediatric patients

Fatty liver can present as focal, diffuse, heterogeneous, and multinodular forms. Being familiar with various patterns of steatosis can enable correct diagnosis. In patients with equivocal findings on ultrasonography, magnetic resonance imaging can be used as a problem solving tool. New techniques are promising for diagnosis and follow-up. We review imaging patterns of steatosis and new quantitative methods such as proton density fat fraction and magnetic resonance elastography for diagnosis of nonalcoholic fatty liver disease in children.Nonalcoholic fatty liver disease (NAFLD) is as widely encountered in children as in adults, with an estimated prevalence of 9.6% (1). It occurs due to accumulation of triglyceride in hepatocytes without alcohol ingestion. Nonalcoholic steatohepatitis (NASH) was first defined in children in 1983 (2). NAFLD includes a broad range of clinicopathologic features ranging from simple steatosis (fat with inflammation and/or fibrosis), steatohepatitis/NASH to cirrhosis. Some other diseases of liver can also cause hepatic steatosis including hepatitis B and C, Wilson’s disease, α-1-antitrypsin deficiency, autoimmune hepatitis, drug-induced liver injury (valproate, methotrexate, tetracycline, amiodarone, and prednisone), and total parenteral nutrition (3). Furthermore, fatty liver is a risk factor for cirrhosis, diabetes, and cardiovascular disease.In clinical practice, the diagnosis of NAFLD is made by increased serum ALT and/or presence of enlarged echogenic liver in ultrasonography. Being overweight or obese, and/or insulin resistance are highly indicative but not absolutely necessary for diagnosing NAFLD (4). The gold standard for diagnosis is liver biopsy, which additionally provides semi-quantitative analysis of NASH damage in children (5). It is an expensive, invasive procedure with a risk of morbidity (0.06%–0.35%) and mortality (0.01%–0.1%) (6).The evaluation of liver fat in children via noninvasive imaging modalities is needed to avoid complications of biopsy and for follow-up. Main imaging modalities for the assessment of pediatric NAFLD are ultrasonography (US) and magnetic resonance imaging (MRI). Computed tomography is the other imaging method for liver fat assessment, but ionizing radiation is a major drawback in children (7). Assessment of fat accumulation may cause diagnostic dilemmas and confusion due to manifestations with unusual structural patterns and imaging appearance of the liver. This article reviews the histopathology of pediatric NAFLD, radiologic evaluation and different structural patterns of childhood NAFLD/NASH on US and MRI. We also discuss diagnostic pitfalls and briefly review new imaging techniques.     

Calcifying fibrous pseudotumor of lung: a previously unreported entity

Calcifying fibrous pseudotumor (CFP) is one of the most unusual benign tumors of childhood and is located mostly in soft tissues, pleura, and peritoneum. The first case of pulmonary CFP in a 7-year-old boy is presented. The clinicopathologic features of this rare entity are discussed with special emphasis on histopathologic features and prognosis including a brief literature review. There are no presumptive clinical and laboratory findings, including tumor markers and imaging techniques, that distinguish CFP from other space-occupying lung lesions. The most important diagnostic aid is to bear this entity in mind when a child presents with an unexplained pulmonary mass. Total excision is mandatory for the appropriate diagnosis and treatment.     

Matrix metalloproteinase-9 expression correlated with tumor response in patients with locally advanced rectal cancer undergoing preoperative chemoradiotherapy

PURPOSE: To analyze whether the expression of matrix metalloproteinases (MMPs) and their tissue inhibitors are associated with tumor response to preoperative chemoradiotherapy in rectal cancer patients. METHODS AND MATERIALS: Forty-four patients who had undergone preoperative chemoradiotherapy were evaluated retrospectively. Treatment consisted of pelvic radiotherapy and two cycles of 5-fluorouracil plus leucovorin. Surgery was performed 6-8 weeks later. MMP-2, MMP-9, and tissue inhibitors of metalloproteinase-1 and -2 expression was analyzed by immunohistochemistry of the preradiation biopsy and surgical specimens. The intensity and extent of staining were evaluated separately, and a final score was calculated by multiplying the two scores. The primary endpoint was the correlation of expression with tumor response, with the secondary endpoint the effect of chemoradiotherapy on the expression. RESULTS: Preoperative treatment resulted in downstaging in 20 patients (45%) and no clinical response in 24 (55%). The pathologic tumor response was complete in 11 patients (25%), partial in 23 (52%), and none in 10 (23%). Positive MMP-9 staining was observed in 20 tumors (45%) and was associated with the clinical nodal stage (p = 0.035) and the pathologic and clinical response (p < 0.0001). The staining status of the other markers was associated with neither stage nor response. The overall pathologic response rate was 25% in MMP-9-positive patients vs. 52% in MMP-9-negative patients (p = 0.001). None of the 11 patients with pathologic complete remission was MMP-9 positive. CONCLUSIONS: Matrix metalloproteinase-9 expression correlated with a poor tumor response to preoperative chemoradiotherapy in rectal carcinoma patients.     

Relation between radionuclide imaging and pathologic findings of ureteropelvic junction obstruction in neonatal hydronephrosis

OBJECTIVE: An excessive amount of collagen fibers around the muscle cells in the ureteropelvic junction could be responsible for obstruction in patients with hydronephrosis. We aimed to elucidate the ultrastructure of the ureters and correlate this finding with the prognostic outcome and to correlate the histopathological findings with diuretic radionuclide renography findings. MATERIAL AND METHODS: Biopsy specimens of 20 children who underwent dismembered pyeloplasty for ureteropelvic junction obstruction were analyzed. The patients were grouped according to their age: infants (<12 months) and others (>12 months). Diuretic radionuclide imaging was performed using (99m)Tc mercaptylacetyltriglycine in the pre- and postoperative periods. Changes in differential renal function and excretion patterns on diuretic renography were evaluated in relation to the findings noted on histopathological examination of the biopsy specimens. Excretion patterns were classified as follows: A, normal; B, responsive to diuretic; C, minimal response to diuretic with some excretion after postural change; and D, very poor/no drainage despite diuretics. Biopsy materials were analyzed for the presence and extent of inflammation, fibrosis and changes in the smooth muscle layer using Masson's trichrome stain and immunohistochemical staining. Histopathological findings were graded from zero to three, depending on severity. RESULTS: In patients aged <12 months, preoperative differential renal function (DRF) was associated with fibrosis (F) and smooth muscle hypertrophy (SMH) [mean (SD) DRF for both F and SMH were Grade 0-1, 47.8% (6.4%); Grade 2-3, 36.2% (11.3%); p<0.05]; and change in DRF was associated with inflammation [Grade 0-1, -0.1% (4.0%); Grade 2-3, 5.8% (3.0%); p<0.05]. Excretion patterns or improvement in excretion were not associated with any of the histopathological features. Change in DRF was significantly associated with inflammation Grade 2-3 (beta coefficient, 5.8; 95% CI 1.4-10.3). CONCLUSIONS: Histopathological evaluation of renal parenchymal biopsy specimens obtained during pyeloplasty may be useful to provide an objective method for predicting the recovery of renal function. In addition, this will allow comparison of the types of histopathological alterations with the changes in differential renal function in order to predict the potential final improvement.