Maria Pia Bondioni

Hepatocellular Adenoma and Focal Nodular Hyperplasia: Value of Gadoxetic Acid–enhanced MR Imaging in Differential Diagnosis

PURPOSE To retrospectively evaluate the utility of gadoxetic acid-enhanced magnetic resonance (MR) imaging in the differential diagnosis of hepatocellular adenoma (HCA) and focal nodular hyperplasia (FNH). MATERIALS AND METHODS This study had institutional review board approval; the requirement for informed consent was waived. Eighty-two patients (58 patients with FNH and 24 patients with HCAs) with 111 lesions were included in the study. There were 74 female patients and eight male patients (mean age, 41.9 years±13.2 [standard deviation]; age range, 11-78 years). Two readers reviewed all images in terms of signal intensity (SI) features on unenhanced, dynamic, and hepatobiliary phase images. For quantitative analysis, contrast enhancement ratio (CER), lesion-to-liver contrast (LLC), and SI ratio on dynamic and hepatobiliary phase images were calculated. RESULTS The CER of FNH in the arterial phase (mean, 94.3%±33.2) was significantly higher than that of HCAs (mean, 59.3%±28.1) (P<.0001). During the hepatobiliary phase, the LLC of FNH showed minimally positive values (mean, 0.05±0.01) and that of HCAs demonstrated strong negative values (mean, -0.67±0.24) (P<.0001). The area under the receiver operating characteristic curve of the hepatobiliary phase SI ratio for differentiation of the two tumors was 0.97, and a sensitivity of 92% and specificity of 91% were found with a cutoff value of 0.87. Among six FNH lesions that showed atypical hypointensity during the hepatobiliary phase, four had a large central scar, one contained a substantial fat component, and one had abundant radiating fibrous septa. Three HCAs were isointense during the hepatobiliary phase owing to severe hepatic steatosis. CONCLUSION Gadoxetic acid-enhanced MR imaging facilitates the differentiation of FNH from HCA.

Solid hypervascular liver lesions: accurate identification of true benign lesions on enhanced dynamic and hepatobiliary phase magnetic resonance imaging after gadobenate dimeglumine administration.

Purpose:To evaluate hepatobiliary phase magnetic resonance imaging with gadobenate dimeglumine for differentiation of benign hypervascular liver lesions from malignant or high-risk lesions. Methods and Materials:Retrospective assessment was performed of 550 patients with 910 hypervascular lesions (302 focal nodular hyperplasia [FNH], 82 nodular regenerative hyperplasia [NRH], 59 hepatic adenoma or liver adenomatosis [HA/LA], 329 hepatocellular carcinomas [HCC], 12 fibrolamellar-HCC [FL-HCC], 21 peripheral cholangiocarcinomas [PCC], 105 metastases). Imaging was performed before and during the arterial, portal-venous, equilibrium, and hepatobiliary phases after gadobenate dimeglumine administration (0.05 mmol/kg). Histologic confirmation was available for ≥1 lesion per patient, except for patients with suspected FNH (diagnosis based on characteristic enhancement/follow-up). Lesion differentiation (benign/malignant) on the basis of contrast washout and lesion enhancement (hypo-/iso-/hyperintensity) was assessed (sensitivity, specificity, accuracy, PPV, and NPV) relative to histology or final diagnosis. Results:On portal-venous or equilibrium phase images, washout was not seen for 208 of 526 (39.5%) malignant (HCC, FL-HCC, PCC, metastases) and high-risk (HA/LA) lesions. Conversely, only 5 of 384 (1.3%) true benign lesions (FNH/NRH) showed washout. Taking washout as indicating malignancy, the sensitivity, specificity, and accuracy for malignant lesion identification during these phases was 61.8%, 98.7%, and 77.4%. On hepatobiliary phase images, 289 of 302 FNH, 82 of 82 NRH, 1 of 59 HA or LA, 62 of 341 HCC or FL-HCC, and 2 of 105 metastases were hyperintense or isointense. Taking iso- or hyperintensity as an indication for lesion benignity, the sensitivity, specificity, accuracy, PPV, and NPV for benign lesion identification was 96.6%, 87.6%, 91.4%, 85.1%, and 97.3%, respectively. Conclusions:Hepatobiliary phase imaging with gadobenate dimeglumine is accurate for distinguishing benign lesions from malignant or high-risk lesions. Biopsy should be considered for hypointense lesions on hepatobiliary phase images after gadobenate dimeglumine.

MRI of focal nodular hyperplasia (FNH) with gadobenate dimeglumine (Gd-BOPTA) and SPIO (ferumoxides): An intra-individual comparison

To compare the efficacy of two different MR contrast agents for the detection and diagnosis of focal nodular hyperplasia (FNH).

Genetic Causes of Bronchiectasis: Primary Immune Deficiencies and the Lung

Primary immune deficiencies (PID) comprise a heterogeneous group of genetically determined disorders that affect development and/or function of innate or adaptive immunity. Consequently, patients with PID suffer from recurrent and/or severe infections that frequently involve the lung. While the nature of the immune defect often dictates the type of pathogens that may cause lung infection, there is substantial overlap of radiological findings, so that appropriate laboratory tests are mandatory to define the nature of the immune defect and to prompt appropriate treatment. At the same time, the recent identification of a large number of PID-causing genes now allows early, even presymptomatic diagnosis, thus representing an essential tool for prevention of lung damage. This review article describes the most common forms of PID, their cellular and molecular bases, and the associated lung abnormalities, and reports on available treatment.

Pulmonary and sinusal changes in 45 patients with primary immunodeficiencies: computed tomography evaluation.

Objective: The aims of the study were 1) to identify and quantify pulmonary changes in subjects affected by agammaglobulinemia (AG), and common variable immunodeficiency (CVID) and 2) to assess the incidence, type, and degree of chronic sinusitis and their relation to pulmonary changes. Methods: Forty-five patients affected by AG (18) and CVID (27) underwent computed tomography of lungs and paranasal sinuses. Results: Of 45 patients, 26 (57.7%) had pulmonary changes, more frequent among CVID than AG patients (P = 0.37). Bronchiectases were detected in 7 of 12 AG and in 9 of 14 CVID; the difference is not statistically significant (P = 0.53). Computed tomographic findings of chronic sinusitis were detected in 41 of 45 patients. There was no statistically significant difference between AG and CVID patients. Bronchial and sinusal abnormalities did not correlate in 11 patients followed longitudinally. Conclusions: On computed tomography, the type and severity of lung lesions do not correlate either with the type of immunodeficiency or with the severity of the sinusal involvement.

MR Imaging of Hepatocellular Adenomas and Differential Diagnosis Dilemma

Hepatocellular adenomas (HCAs) are currently categorized into distinct genetic and pathologic subtypes as follows: inflammatory hepatocellular adenoma, hepatocyte-nuclear-factor-1-alpha (HNF-1α-mutated) hepatocellular adenoma, and β-catenin-mutated hepatocellular adenomas; the fourth, defined as unclassified subtype, encompasses HCAs without any genetic abnormalities. This classification has accepted management implications due to different risks of haemorrhage and malignant transformation of the four subtypes. Imaging guided biopsy and/or surgical resection very important in obtaining definitive characterization; nevertheless, MRI with intra-extravascular and hepatobiliary (dual phase) agents, is an important tool not only in differential subtypes definition but even in surveillance with early identification of complications and discovery of some signs of HCA malignant degeneration. Inflammation, abnormal rich vascularisation, peliotic areas, and abundant fatty infiltration are pathologic findings differently present in the HCA subtypes and they may be detected by multiparametric MRI approach. Lesion enlargement and heterogeneity of signal intensity and of contrast enhancement are signs to be considered in malignant transformation. The purpose of this paper is to present the state of the art of MRI in the diagnosis of HCA and subtype characterization, with particular regard to morphologic and functional information available with dual phase contrast agents, and to discuss differential diagnosis with the most common benign and malignant lesions mimicking HCAs.

Isolated fallopian tube torsion in an adolescent: CT features

Sir, A 13-year-old girl presented to the emergency department with acute left lower quadrant pain of 12h duration, radiating to the left leg and associated with vomiting. She had been constipated for 3 days, but did not have any urinary symptoms. There was no past medical history of note. The initial working diagnosis was hip synovitis, and so she was referred to the orthopaedic department. She underwent US of both hips, which was normal, but pelvic US showed a well-delineated, nonhomogeneous hypoechoic mass, 5.0·4.0 cm in size, close to the left ovary. The uterus and ovaries were normal (Fig. 1). Laboratory data and tumour markers were normal on admission to hospital. Rectal examination identified a palpable mobile mass on the left side. As the US performed by the gynaecology department was considered non-diagnostic, the patient underwent CT of the pelvis. This showed a mass in the left iliac fossa (LIF) and left side of the pelvis, close to the uterus, which was not displaced. The mass was well-delineated with heterogeneous areas of differing density (Fig. 2a). There was variable patchy enhancement after IV contrast enhancement (Fig. 2b, c). Free pelvic fluid and inflammatory changes within the pelvic fat were seen (Fig. 2c). The left ovary was not seen clearly; the remaining pelvic viscera and colon were normal. Laparoscopy identified normal ovaries and uterus, but left fallopian tube torsion was evident. Vascularisation of the left ovary was normal. Left salpingectomy was performed and histological examination showed fallopian tube torsion with necrotic and hemorrhagic areas. Torsion of the fallopian tube is an uncommon cause of abdominal pain in premenarchal girls and postmenarchal teenagers. It can be related to intrinsic or extrinsic causes. Intrinsic factors predisposing to torsion include: (1) an abnormally long tube and mesosalpinx, (2) adnexal venous congestion related to premenarchal hormonal activity, or when the veins of the mesosalpinx are longer than the arteries, and (3) abnormal peristalsis of the tube. Extrinsic factors include: (1) pelvic masses, (2) trauma LETTER TO THE EDITOR

A Review of the Actual Knowledge of the Processes Governing Growth and Development of Long Bones

Autoptic samples of human bones (from 8 weeks of gestation to 12 years of age) and a second group of serial, skeletal x-rays (required for pathologies not related to bone dysplasia in children from 4 months to 17 years of age) provided the material for the analysis of the physes normal growth mechanism presented in this review. Before the appearance of the ossification centers epiphyseal growth rests exclusively on chondrocytes proliferation (interstitial growth), without any detectable differentiated cellular organization. When endochondral ossification starts a defined spatial disposition of chondrocytes and a corresponding organization of the intercellular matrix is set up, so that it is possible to identify a growth vector corresponding to the columns of piled chondrocytes with direction from hypertrophic toward the proliferative cell layers. The complexity of the tubular bones growth process is well represented by the spatial arrangement of the growth vectors. In the late epiphyseal growth another mechanism is active in addition to endochondral ossification, namely, articular cartilage interstitial growth and subchondral remodelling. The knowledge of the normal mode of organization of the physis and its temporal sequence can help to better understand of the deviaton from the normal development of metaphyseal and epiphyseal dysplasias.

Prenatal presentation and postnatal evolution of a patient with Jansen metaphyseal dysplasia with a novel missense mutation in PTH1R

Wave‐shaped ribs were detected at prenatal ultrasound in a 20+1 week female fetus. At birth, skeletal radiographs showed marked hypomineralization and suggested hypophosphatasia. However, elevated blood calcium and alkaline phosphatase excluded hypophosphatasia and raised the possibility of Jansen metaphyseal dysplasia. Molecular analysis of the PTH/PTHrP receptor gene (PTH1R) showed heterozygosity for a previously undescribed transversion variant (c.1373T>A), which predicts p.Ile458Lys. In vitro evaluation of wild type and mutant PTH/PTHrP receptors supported the pathogenic role of the p.Ile458Lys substitution, and confirmed the diagnosis of Jansen metaphyseal dysplasia. This disorder may present prenatally with wavy ribs and in the newborn with hypomineralization, and may therefore be confused with hypophosphatasia. The mottled metaphyseal lesions typically associated with this disease appear only in childhood.

Common variable immunodeficiency: computed tomography evaluation of bronchopulmonary changes including nodular lesions in 40 patients. Correlation with clinical and immunological data.

Background: Common variable immunodeficiency (CVID) is the most common symptomatic primary immunodeficiency characterized by recurrent respiratory tract infections, mainly sustained by encapsulated bacteria, that may cause irreversible changes in the lungs. Methods: Forty patients with CVID were evaluated by computed tomography of the lung; 20 of these underwent computed tomographic follow-up in a 5-year period, during which immunoglobulin replacement therapy was regularly performed. Results: Pulmonary changes were present in 65% of patients; bronchiectases were present in 65.38%. The incidence of pulmonary nodules was very high (38.46%) and correlated with splenomegaly (70%) and autoimmune phenomena (80%). Conclusions: Our study underscores the essential role of imaging, in particular computed tomography, in the identification and monitoring of pulmonary lesions in a large cohort of CVID patients, contributing at the same time to select patients more at risk to develop nodular lesions and potentially to use more appropriate therapeutic strategies.